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1. Willmann M, Müllauer L, Schwendenwein I, Wolfesberger B, Kleiter M, Pagitz M, Hadzijusufovic E, Shibly S, Reifinger M, Thalhammer JG, Valent P: Chemotherapy in canine acute megakaryoblastic leukemia: a case report and review of the literature. In Vivo; 2009 Nov-Dec;23(6):911-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chemotherapy in canine acute megakaryoblastic leukemia: a case report and review of the literature.
  • Acute myeloid leukemia (AML) in dogs is a rare disease with poor prognosis.
  • In most subjects, palliative treatment or euthanasia is performed.
  • Treatment was well tolerated and complete remission was achieved.
  • Postinduction chemotherapy consisted of ARA-C, daunorubicin and prednisolone.
  • Again, no severe side-effects occurred and the disease was controlled, with 37 chemotherapy-cycles (ARA-C, 3 x 1,000 mg/m(2)/cycle), for 24 months.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Dog Diseases / drug therapy. Leukemia, Megakaryoblastic, Acute / veterinary
  • [MeSH-minor] Animals. Bone Marrow Cells / drug effects. Bone Marrow Cells / pathology. Cytarabine / administration & dosage. Daunorubicin / administration & dosage. Dogs. Etoposide / administration & dosage. Male. Prednisolone / administration & dosage. Remission Induction. Secondary Prevention. Vincristine / administration & dosage

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  • (PMID = 20023232.001).
  • [ISSN] 0258-851X
  • [Journal-full-title] In vivo (Athens, Greece)
  • [ISO-abbreviation] In Vivo
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 04079A1RDZ / Cytarabine; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 9PHQ9Y1OLM / Prednisolone; ZS7284E0ZP / Daunorubicin
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2. Athale UH, Razzouk BI, Raimondi SC, Tong X, Behm FG, Head DR, Srivastava DK, Rubnitz JE, Bowman L, Pui CH, Ribeiro RC: Biology and outcome of childhood acute megakaryoblastic leukemia: a single institution's experience. Blood; 2001 Jun 15;97(12):3727-32
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  • [Title] Biology and outcome of childhood acute megakaryoblastic leukemia: a single institution's experience.
  • To describe the clinical and biologic features of pediatric acute megakaryoblastic leukemia (AMKL) and to identify prognostic factors, experience at St Jude Children's Research Hospital was reviewed.
  • Of 281 patients with acute myeloid leukemia treated over a 14-year period, 41 (14.6%) had a diagnosis of AMKL.
  • The rate of remission induction was 60.5%, with a 48% rate of subsequent relapse.
  • Among patients who had de novo AMKL without Down syndrome, 2-year EFS was significantly higher after allogeneic bone marrow transplantation (26%) than after chemotherapy alone (0%; P =.019) and significantly higher when performed during remission (46%) than when performed during persistent disease (0%; P =.019).
  • Treatment outcome is very poor for patients with AMKL in the absence of Down syndrome.
  • Remission induction is the most important prognostic factor.
  • Allogeneic transplantation during remission offers the best chance of cure; in the absence of remission, transplantation offers no advantage over chemotherapy alone. (Blood.
  • [MeSH-major] Leukemia, Megakaryoblastic, Acute / diagnosis
  • [MeSH-minor] Bone Marrow Transplantation. Disease-Free Survival. Down Syndrome / complications. Female. Humans. Male. Neoplasms, Second Primary. Prognosis. Prospective Studies. Remission Induction. Retrospective Studies. Survival Rate. Transplantation, Homologous. Treatment Outcome

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  • (PMID = 11389009.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01 CA-20180; United States / NCI NIH HHS / CA / P30 CA-21765
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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3. Pagano L, Pulsoni A, Vignetti M, Mele L, Fianchi L, Petti MC, Mirto S, Falcucci P, Fazi P, Broccia G, Specchia G, Di Raimondo F, Pacilli L, Leoni P, Ladogana S, Gallo E, Venditti A, Avanzi G, Camera A, Liso V, Leone G, Mandelli F, GIMEMA: Acute megakaryoblastic leukemia: experience of GIMEMA trials. Leukemia; 2002 Sep;16(9):1622-6
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  • [Title] Acute megakaryoblastic leukemia: experience of GIMEMA trials.
  • The objective of the study was to evaluate the incidence, characteristics, treatment and outcome of acute megakaryoblastic leukemia (AMeL) in patients enrolled in GIMEMA trials.
  • Diagnosis was based on morphological criteria.
  • Four patients died in CR due to chemotherapy-related complications.
  • On the basis of these data, a specific therapeutic approach, possibly with innovative treatments, should be evaluated.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Transplantation. Leukemia, Megakaryoblastic, Acute / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Combined Modality Therapy. Cytogenetic Analysis. Female. Humans. Immunophenotyping. Middle Aged. Remission Induction. Survival Rate. Treatment Outcome


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4. Bastida Vilá P, Olivé Oliveras T, Díaz de Heredia Rubio C, Ortega Aramburu JJ: [Transient neonatal myeloproliferative disorder in the absence of Down syndrome]. An Pediatr (Barc); 2004 Dec;61(6):546-50
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  • [Title] [Transient neonatal myeloproliferative disorder in the absence of Down syndrome].
  • [Transliterated title] Síndrome mieloproliferativo transitorio neonatal en ausencia de síndrome de Down.
  • Transient neonatal leukemia or transient neonatal myeloproliferative disorder is commonly associated with Down syndrome.
  • However, 25 % of patients will subsequently develop acute megakaryoblastic leukemia or myelodysplastic syndrome.
  • We present three phenotypically normal patients with this disorder.
  • Diagnosis was guided by pathological examination of the skin lesions.
  • They received support treatment only without chemotherapy.
  • Caution must be exercised before initiating chemotherapy in these patients.
  • We discuss the differential diagnosis with congenital leukemia and the prognostic and therapeutic implications that this entails.
  • [MeSH-minor] Diagnosis, Differential. Female. Humans. Infant, Newborn. Leukemia / congenital. Leukemia / diagnosis. Prognosis. Remission, Spontaneous

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  • (PMID = 15574256.001).
  • [ISSN] 1695-4033
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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5. Lee JH, Choi SJ, Lee JH, Park JH, Kim H, Joo YD, Lee WS, Zang DY, Kim HJ, Lee KH, Cooperative Study Group A for Hematology: Standard induction chemotherapy followed by attenuated consolidation in elderly patients with acute myeloid leukemia. Ann Hematol; 2006 Jun;85(6):357-65
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  • [Title] Standard induction chemotherapy followed by attenuated consolidation in elderly patients with acute myeloid leukemia.
  • The benefits of intensive post-remission chemotherapy have not been verified in elderly patients with acute myeloid leukemia (AML).
  • To reduce fatal complications caused by intensive post-remission therapy, we performed a prospective phase II multicenter trial of standard induction chemotherapy ('7+3' of cytarabine plus daunorubicin), followed by two cycles of attenuated consolidation therapy ('5+1' of cytarabine plus daunorubicin) for elderly patients with AML, excluding those with M3.
  • Of these 24, 17 (70.8%) completed both planned cycles of consolidation therapy.
  • There were no fatal complications during consolidation therapy.
  • These findings suggest that, when compared with previous findings using more intensive consolidation therapy, attenuated consolidation therapy does not compromise outcomes in elderly AML patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Leukemia, Myeloid / drug therapy
  • [MeSH-minor] Acute Disease. Aged. Disease-Free Survival. Female. Flow Cytometry. Humans. Immunophenotyping / methods. Leukemia, Erythroblastic, Acute / drug therapy. Leukemia, Erythroblastic, Acute / immunology. Leukemia, Erythroblastic, Acute / pathology. Leukemia, Megakaryoblastic, Acute / drug therapy. Leukemia, Megakaryoblastic, Acute / immunology. Leukemia, Megakaryoblastic, Acute / pathology. Leukemia, Monocytic, Acute / drug therapy. Leukemia, Monocytic, Acute / immunology. Leukemia, Monocytic, Acute / pathology. Leukemia, Myeloid, Acute / drug therapy. Leukemia, Myeloid, Acute / immunology. Leukemia, Myeloid, Acute / pathology. Leukemia, Myelomonocytic, Acute / drug therapy. Leukemia, Myelomonocytic, Acute / immunology. Leukemia, Myelomonocytic, Acute / pathology. Male. Middle Aged. Prognosis. Prospective Studies. Treatment Outcome

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  • (PMID = 16575580.001).
  • [ISSN] 0939-5555
  • [Journal-full-title] Annals of hematology
  • [ISO-abbreviation] Ann. Hematol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] Germany
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6. Kreft A, Burg J, Fischer T, Kirkpatrick CJ: Essential thrombocythemia terminating in pure erythroleukemia. Am J Hematol; 2004 Oct;77(2):140-3
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  • [Title] Essential thrombocythemia terminating in pure erythroleukemia.
  • Transformation into acute leukemia is a rare event in essential thrombocythemia (ET).
  • The blasts are usually of myeloid, rarely of megakaryoblastic differentiation.
  • After 10 years of therapy with hydroxyurea, the patient developed acute leukemia of solely erythroid differentiation.
  • Chemotherapy with cytarabine and daunorubicin resulted in incomplete remission.
  • The patient died 2 months after diagnosis of acute erythroleukemia.
  • Transformation of ET into erythroleukemia may demonstrate the pluripotent potential of the neoplastic hemopoietic stem cell, with the ability to cause acute leukemia not only of myeloid or megakaryoblastic but also of erythroid lineage.
  • [MeSH-major] Bone Marrow / pathology. Leukemia, Erythroblastic, Acute / pathology. Thrombocytosis / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cell Transformation, Neoplastic. Fatal Outcome. Humans. Male. Middle Aged. Time Factors

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15389906.001).
  • [ISSN] 0361-8609
  • [Journal-full-title] American journal of hematology
  • [ISO-abbreviation] Am. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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7. Sakai C, Matsubayashi K, Saotome T, Ishii A, Kumagai K: Therapy-related myelodysplastic syndrome with trisomy 1q due to der(1;7) and megakaryoblastic proliferation developing during complete remission of therapy-related acute myeloid leukemia with t(8;21). Intern Med; 2004 Jul;43(7):582-6
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  • [Title] Therapy-related myelodysplastic syndrome with trisomy 1q due to der(1;7) and megakaryoblastic proliferation developing during complete remission of therapy-related acute myeloid leukemia with t(8;21).
  • Therapy-related acute myeloid leukemia (t-AML) with t(8;21) and therapy-related myelodysplastic syndrome (t-MDS) with trisomy 1q due to der(1;7) developed in the same patient with T-cell lymphoma at intervals of six years.
  • After the development of t-MDS with trisomy 1q, during complete remission of t-AML, the number of megakaryoblasts increased to maximally 74% of leukocytes in the blood.
  • This is a very rare case of two separate therapy-related myeloid malignancies (early t-AML and late t-MDS) and is also a notable case of t-MDS with trisomy 1q due to der(1;7) accompanied by megakaryoblastic proliferation.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Chromosomes, Human, Pair 1. Leukemia, Myeloid, Acute / chemically induced. Lymphoma, T-Cell / drug therapy. Myelodysplastic Syndromes / chemically induced. Trisomy / genetics


8. Oki Y, Kantarjian HM, Zhou X, Cortes J, Faderl S, Verstovsek S, O'Brien S, Koller C, Beran M, Bekele BN, Pierce S, Thomas D, Ravandi F, Wierda WG, Giles F, Ferrajoli A, Jabbour E, Keating MJ, Bueso-Ramos CE, Estey E, Garcia-Manero G: Adult acute megakaryocytic leukemia: an analysis of 37 patients treated at M.D. Anderson Cancer Center. Blood; 2006 Feb 1;107(3):880-4
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  • [Title] Adult acute megakaryocytic leukemia: an analysis of 37 patients treated at M.D. Anderson Cancer Center.
  • To characterize acute megakaryocytic leukemia (FAB M7 AML), we identified 37 patients with M7 AML treated at M.D.
  • The median age of the M7 AML group was 56 years (range, 21-78 years); 22 patients (59%) had an antecedent hematologic disorder or myelodysplastic syndrome or both, and 7 patients (19%) had previously received chemotherapy for other malignancies.
  • Complete remission rates were 43% with M7 AML and 57% with non-M7 AML (P = .089).
  • [MeSH-major] Chromosome Aberrations. Chromosomes, Human. Leukemia, Megakaryoblastic, Acute / mortality. Leukemia, Megakaryoblastic, Acute / pathology
  • [MeSH-minor] Adult. Aged. Disease-Free Survival. Female. Humans. Male. Middle Aged. Multivariate Analysis. Myelodysplastic Syndromes / mortality. Myelodysplastic Syndromes / pathology. Myelodysplastic Syndromes / therapy. Neoplasms. Neoplasms, Second Primary / mortality. Neoplasms, Second Primary / pathology. Neoplasms, Second Primary / therapy. Primary Myelofibrosis / mortality. Primary Myelofibrosis / pathology. Primary Myelofibrosis / therapy. Retrospective Studies

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  • (PMID = 16123215.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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9. Wang D, Ke XY, Wang J, Xu F, Hu YF: [Correlation between MDR1 genetic polymorphism and prognosis in acute myeloid leukemia]. Zhonghua Yi Xue Za Zhi; 2007 May 29;87(20):1384-8
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  • [Title] [Correlation between MDR1 genetic polymorphism and prognosis in acute myeloid leukemia].
  • OBJECTIVE: To assess the correlation of the multidrug resistance-1 (MDR1) gene single nucleotide polymorphisms (SNP) C1236T, G2677T/A and C3435T with the outcome of induction chemotherapy in patients with de novo acute myeloid leukemia (AML).
  • Bone marrow smear was made at the end of the first induction chemotherapy to estimate whether complete remission (CR) has been achieved with the clinical characteristics.
  • CONCLUSION: With important prognostic significance, MDR1 genetic polymorphisms, such as G2677T/A can predict whether complete remission can be achieved after the first course of induction chemotherapy.
  • [MeSH-major] Leukemia, Myeloid / genetics. P-Glycoprotein / genetics. Polymorphism, Single Nucleotide
  • [MeSH-minor] Acute Disease. Adolescent. Adult. Aged. Aged, 80 and over. Base Sequence. DNA Mutational Analysis. Female. Gene Frequency. Genotype. Humans. Leukemia, Erythroblastic, Acute / drug therapy. Leukemia, Erythroblastic, Acute / genetics. Leukemia, Erythroblastic, Acute / pathology. Leukemia, Megakaryoblastic, Acute / drug therapy. Leukemia, Megakaryoblastic, Acute / genetics. Leukemia, Megakaryoblastic, Acute / pathology. Leukemia, Monocytic, Acute / drug therapy. Leukemia, Monocytic, Acute / genetics. Leukemia, Monocytic, Acute / pathology. Leukemia, Myeloid, Acute / drug therapy. Leukemia, Myeloid, Acute / genetics. Leukemia, Myeloid, Acute / pathology. Leukemia, Myelomonocytic, Acute / drug therapy. Leukemia, Myelomonocytic, Acute / genetics. Leukemia, Myelomonocytic, Acute / pathology. Leukemia, Promyelocytic, Acute / drug therapy. Leukemia, Promyelocytic, Acute / genetics. Leukemia, Promyelocytic, Acute / pathology. Male. Middle Aged. P-Glycoproteins. Prognosis. Remission Induction

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  • (PMID = 17785057.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / ABCB1 protein, human; 0 / P-Glycoprotein; 0 / P-Glycoproteins
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10. Hirose Y, Kiyoi H, Iwai M, Yokozawa T, Ito M, Naoe T: Successful treatment with imatinib mesylate of a CML patient in megakaryoblastic crisis with severe fibrosis. Int J Hematol; 2002 Nov;76(4):349-53
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  • [Title] Successful treatment with imatinib mesylate of a CML patient in megakaryoblastic crisis with severe fibrosis.
  • The prognosis of patients with chronic myeloid leukemia in blastic crisis (CML-BC) remains extremely poor, and multiagent chemotherapy regimens commonly used to treat acute leukemia offer only short-term benefits.
  • Therefore, the advent of the novel molecularly targeted anticancer agent imatinib mesylate is a breakthrough in CML therapy.
  • We present a CML patient in megakaryoblastic crisis with severe myelofibrosis, who was treated with imatinib at a dosage of 400 mg/day and achieved complete remission together with a marked regression of myelofibrosis after 1 month.
  • The effect of imatinib on the long-term prognosis remains unclear, although the agent is clearly a promising drug for treating CML-BC even in cases of myelofibrosis.
  • [MeSH-major] Blast Crisis / drug therapy. Leukemia, Myelogenous, Chronic, BCR-ABL Positive / drug therapy. Piperazines / administration & dosage. Primary Myelofibrosis / drug therapy. Pyrimidines / administration & dosage

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  • (PMID = 12463599.001).
  • [ISSN] 0925-5710
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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11. Chitlur MB, Bhambhani K, Mohamed AN, Ravindranath Y, Savaşan S: Acute megakaryoblastic leukemia with t(17;22)(q21;q13) and liver dysfunction. Cancer Genet Cytogenet; 2004 Oct 15;154(2):167-8
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  • [Title] Acute megakaryoblastic leukemia with t(17;22)(q21;q13) and liver dysfunction.
  • The t(1;22)(p13;q13) is associated with acute megakaryoblastic leukemia (AMKL) seen mostly in young infants and known to have a poor prognosis.
  • A 5-year-old child had prolonged prothrombin and partial thromboplastin times, low albumin, and decreased vitamin K-dependent coagulation factors and factor V activities at the time of AMKL diagnosis.
  • All of these factors normalized following chemotherapy when remission was achieved.
  • Here, we present an AMKL pediatric case with a novel translocation and significant hepatocellular dysfunction that resolved with chemotherapy.
  • [MeSH-major] Chromosomes, Human, Pair 17. Chromosomes, Human, Pair 22. Leukemia, Megakaryoblastic, Acute / genetics. Liver Diseases / complications. Translocation, Genetic
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Female. Humans. Karyotyping

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  • (PMID = 15474155.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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12. Olson JL, May MJ, Stork L, Kadan N, Bateman JB: Acute megakaryoblastic leukemia in Down syndrome: orbital infiltration. Am J Ophthalmol; 2000 Jul;130(1):128-30
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  • [Title] Acute megakaryoblastic leukemia in Down syndrome: orbital infiltration.
  • PURPOSE: To describe an uncommon ocular presentation of acute megakaryoblastic leukemia in a child with Down syndrome.
  • RESULTS: Bone marrow biopsy and immunophenotyping established the diagnosis of acute megakaryoblastic leukemia (FAB-M7).
  • The leukemia was treated successfully with chemotherapy, with resolution of proptosis.
  • The patient remained in remission more than 1 year after cessation of treatment.
  • CONCLUSIONS: Bilateral proptosis can be a presenting sign of acute megakaryoblastic leukemia, a malignancy associated with Down syndrome.
  • [MeSH-major] Down Syndrome / complications. Leukemia, Megakaryoblastic, Acute / complications. Orbital Neoplasms / complications
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Exophthalmos / diagnosis. Exophthalmos / etiology. Female. Humans. Immunophenotyping. Infant. Keratitis / diagnosis. Keratitis / etiology. Tomography, X-Ray Computed

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  • (PMID = 11004278.001).
  • [ISSN] 0002-9394
  • [Journal-full-title] American journal of ophthalmology
  • [ISO-abbreviation] Am. J. Ophthalmol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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13. Webb DK: Optimizing therapy for myeloid disorders of Down syndrome. Br J Haematol; 2005 Oct;131(1):3-7
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  • [Title] Optimizing therapy for myeloid disorders of Down syndrome.
  • Children with Down syndrome (DS) are at increased risk of leukaemia.
  • Myeloid disorders include transient abnormal myelopoiesis (TAM), myelodysplasia (MDS) and acute myeloid leukaemia (AML).
  • Mutations in the GATA-1 gene, which encodes for a transcription factor central to the normal development of the erythroid and megakaryocytic lineages, are found in cases of TAM, MDS and AML in DS children.
  • DS children with MDS/AML mostly present between the ages of 1 and 4 years, and have a large predominance of megakaryoblastic disease (French-American-British type M7).
  • The MDS and AML are part of a single disease entity (myeloid leukaemia of Down syndrome) that is extremely sensitive to chemotherapy.
  • Resistant disease and relapse are rare, but treatment-related toxicity is high, and deaths in remission have exceeded those due to disease in most series.
  • Accordingly, controlled dosage reduction is the focus of contemporary treatment studies.
  • [MeSH-major] Down Syndrome / complications. Down Syndrome / therapy. Leukemia, Myeloid / complications. Leukemia, Myeloid / therapy
  • [MeSH-minor] Acute Disease. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Drug Administration Schedule. Humans. Infant. Megakaryocytes / immunology. Neural Tube Defects / complications. Neural Tube Defects / therapy

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  • (PMID = 16173956.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 42
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14. Hiçsönmez G, Cetin M, Okur H, Erdemli E, Gürgey A: The potential effect of short-course high-dose steroid on the maturation and apoptosis of leukemic cells in a child with acute megakaryoblastic leukemia. Leuk Lymphoma; 2003 Jun;44(6):1037-42
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  • [Title] The potential effect of short-course high-dose steroid on the maturation and apoptosis of leukemic cells in a child with acute megakaryoblastic leukemia.
  • High-dose methylprednisolone (HDMP) treatment has been shown to induce differentiation of myeloid leukemic cells in children with acute promyelocytic leukemia and other subtypes (FAB AML M1-M2-M4) of acute myeloblastic leukemia.
  • In the present study, a child with acute megakaryoblastic leukemia (AMKL) was given HDMP (30 mg/kg/day) orally in a single dose for the first 4 days of induction therapy.
  • A marked decrease in peripheral blood blast cells and an increase in platelet count associated with a striking change in bone marrow (BM) morphology was observed following a short-course of HDMP treatment alone.
  • BM cells developed distinct morphology characterized by cytoplasmic blebbing and some appeared as platelet producing micromegakaryocytes.
  • Flow cytometric analysis of the BM cells 4 days after HDMP treatment demonstrated a decrease in the percentage of cells co-expressing CD34 and CD117 antigens and a marked increase in CD42a antigen.
  • These changes in BM morphology and immunophenotype may suggest maturation effect of HDMP on megakaryocytic leukemic cells.
  • These results suggest that HDMP treatment may induce differentiation and apoptosis of leukemic cells in a child with AMKL and it could be a promising agent for remission induction of patients with AMKL.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Apoptosis / drug effects. Leukemia, Megakaryoblastic, Acute / drug therapy. Leukemia, Megakaryoblastic, Acute / pathology. Methylprednisolone / therapeutic use
  • [MeSH-minor] Bone Marrow Cells / drug effects. Bone Marrow Cells / pathology. Child, Preschool. Cytarabine / administration & dosage. Flow Cytometry. Humans. Male. Mitoxantrone / administration & dosage. Time Factors. Treatment Outcome

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  • (PMID = 12854906.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 04079A1RDZ / Cytarabine; BZ114NVM5P / Mitoxantrone; X4W7ZR7023 / Methylprednisolone
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15. Yakoub-Agha I, de La Salmonière P, Ribaud P, Sutton L, Wattel E, Kuentz M, Jouet JP, Marit G, Milpied N, Deconinck E, Gratecos N, Leporrier M, Chabbert I, Caillot D, Damaj G, Dauriac C, Dreyfus F, François S, Molina L, Tanguy ML, Chevret S, Gluckman E: Allogeneic bone marrow transplantation for therapy-related myelodysplastic syndrome and acute myeloid leukemia: a long-term study of 70 patients-report of the French society of bone marrow transplantation. J Clin Oncol; 2000 Mar;18(5):963-71
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  • [Title] Allogeneic bone marrow transplantation for therapy-related myelodysplastic syndrome and acute myeloid leukemia: a long-term study of 70 patients-report of the French society of bone marrow transplantation.
  • PURPOSE: To identify predictive factors of survival, relapse, and transplantation-related mortality (TRM) among patients with therapy-related myelodysplastic syndrome (t-MDS) or acute leukemia (t-AML) who underwent allogeneic bone marrow transplantation (BMT).
  • Thirty-three patients had received induction-type chemotherapy before BMT.
  • At the time of transplantation, there were 24 patients in complete remission (CR) and 46 with active disease.
  • CONCLUSION: BMT is an effective treatment for patients with t-MDS or t-AML who have responsive disease and, in particular, who have no poor-risk cytogenetic features.
  • [MeSH-major] Bone Marrow Transplantation. Leukemia, Megakaryoblastic, Acute / therapy. Myelodysplastic Syndromes / therapy. Neoplasms, Second Primary / therapy. Transplantation, Homologous


16. Li-Thiao-Te V, Bourges-Petit E, Capiod JC, Horle B, Micheli J, Morin G, Maingourd Y, Pautard B: [Congenital transient leukemia: a case report]. Arch Pediatr; 2008 Jan;15(1):33-6
MedlinePlus Health Information. consumer health - Down Syndrome.

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  • [Title] [Congenital transient leukemia: a case report].
  • Neonates with Down's syndrome have an increased risk for congenital leukaemia, particularly acute megakaryoblastic leukaemia (FAB, M7) which most often resolves spontaneously and is called transient leukaemia.
  • OBSERVATION: We report a transient leukaemia with an isolated pericardial effusion in a phenotypically normal neonate.
  • Complete remission remains after 32 months.
  • DISCUSSION: Congenital leukaemias, with trisomy 21 on blasts cells have a good prognosis that justifies observation before using chemotherapy.
  • [MeSH-major] Down Syndrome / complications. Leukemia, Megakaryoblastic, Acute / congenital
  • [MeSH-minor] Antigens, CD / analysis. Humans. Infant. Male. Remission, Spontaneous

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  • (PMID = 18162385.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antigens, CD
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17. Hatae Y, Yagyu K, Yanazume N, Chou Y, Iizuka S, Takeda T, Shikano T, Eguchi M: [Lineage switch on recurrence from minimally differentiated acute leukemia (M0) to acute megakaryocytic leukemia (M7)]. Rinsho Ketsueki; 2002 Jul;43(7):543-7
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  • [Title] [Lineage switch on recurrence from minimally differentiated acute leukemia (M0) to acute megakaryocytic leukemia (M7)].
  • Phenotypic switch in acute leukemia is a rare phenomenon.
  • We report on a female infant with minimally differentiated acute leukemia (M 0) which underwent a lineage switch on relapse.
  • She was treated according to the Children's Cancer and Leukemia Study Group schedule and a complete remission was achieved 1.5 months after starting induction therapy.
  • However, she relapsed in spite of continued chemotherapy in July 1997, when the cytomorphological pattern changed and the patient was diagnosed both morphologically and immunologically as having M 7.
  • We attempted aggressive reinduction therapy, but without effect.
  • The patient simultaneously developed severe pneumonia and died in February, 1998.
  • A lineage switch on relapse and resistance to chemotherapy may be associated with the occurrence of genetic aberration.
  • [MeSH-major] Leukemia / pathology. Leukemia, Megakaryoblastic, Acute / pathology
  • [MeSH-minor] Acute Disease. Cell Lineage. Female. Humans. Infant. Recurrence

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  • (PMID = 12229123.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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18. Law L, Tuscano J, Wun T, Ahlberg K, Richman C: Filgrastim treatment of acute myelogenous leukemia (M7) relapse after allogeneic peripheral stem cell transplantation resulting in both graft-versus-leukemia effect with cytogenetic remission and chronic graft-versus-host disease manifesting as polyserositis and subsequent bronchiolitis obliterans with organizing pneumonia. Int J Hematol; 2002 Nov;76(4):360-4
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  • [Title] Filgrastim treatment of acute myelogenous leukemia (M7) relapse after allogeneic peripheral stem cell transplantation resulting in both graft-versus-leukemia effect with cytogenetic remission and chronic graft-versus-host disease manifesting as polyserositis and subsequent bronchiolitis obliterans with organizing pneumonia.
  • However, the majority of the patients who responded also developed graft-versus-host disease (GVHD).
  • We report the first case of polyserositis following the use of filgrastim to treat a patient with acute myelogenous leukemia (M7), who had relapsed after an initially successful allogeneic PSCT.
  • The polyserositis manifested with effusions and was initially controlled with high doses of steroids and pericardial stripping; however, after a quiescent period the patient eventually developed bronchiolitis obliterans with organizing pneumonia that required additional immunosuppressive therapy.
  • [MeSH-major] Cryptogenic Organizing Pneumonia / etiology. Granulocyte Colony-Stimulating Factor / administration & dosage. Leukemia, Megakaryoblastic, Acute / complications. Leukemia, Megakaryoblastic, Acute / drug therapy. Peripheral Blood Stem Cell Transplantation / adverse effects. Serositis / etiology
  • [MeSH-minor] Adolescent. Chronic Disease. Cytogenetic Analysis. Female. Filgrastim. Graft vs Host Disease / chemically induced. Graft vs Host Disease / etiology. Graft vs Host Disease / pathology. Graft vs Leukemia Effect. Humans. Recombinant Proteins. Remission Induction / methods. Transplantation, Homologous


19. Al-Shehri A, Al-Seraihy A, Owaidah TM, Belgaumi AF: Megakaryocytic blast crisis at presentation in a pediatric patient with chronic myeloid leukemia. Hematol Oncol Stem Cell Ther; 2010;3(1):42-6
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  • [Title] Megakaryocytic blast crisis at presentation in a pediatric patient with chronic myeloid leukemia.
  • Patients with chronic myeloid leukemia (CML) infrequently present in blast crisis (BC).
  • While most BC are of myeloid origin, megakaryocytic BC is rare, especially at the time of CML diagnosis.
  • We describe the first pediatric patient presenting with megakaryocytic leukemia and having BCR-ABL1 translocation as the single chromosomal abnormality.
  • Clinical features were more suggestive of CML in megakaryocytic blast crisis than Philadelphia chromosome positive de novo AML.
  • The patient was treated with AML-directed chemotherapy and imatinib mesylate followed by umbilical cord blood stem cell transplantation.
  • [MeSH-major] Blast Crisis / pathology. Leukemia, Megakaryoblastic, Acute / pathology. Leukemia, Myelogenous, Chronic, BCR-ABL Positive / pathology. Megakaryocytes / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Benzamides. Child. Cord Blood Stem Cell Transplantation. Diagnosis, Differential. Female. Humans. Imatinib Mesylate. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Remission Induction. Treatment Outcome

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  • (PMID = 20231813.001).
  • [ISSN] 1658-3876
  • [Journal-full-title] Hematology/oncology and stem cell therapy
  • [ISO-abbreviation] Hematol Oncol Stem Cell Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Saudi Arabia
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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20. Kudo K, Kojima S, Tabuchi K, Yabe H, Tawa A, Imaizumi M, Hanada R, Hamamoto K, Kobayashi R, Morimoto A, Nakayama H, Tsuchida M, Horibe K, Kigasawa H, Tsukimoto I, Japanese Childhood AML Cooperative Study Group: Prospective study of a pirarubicin, intermediate-dose cytarabine, and etoposide regimen in children with Down syndrome and acute myeloid leukemia: the Japanese Childhood AML Cooperative Study Group. J Clin Oncol; 2007 Dec 1;25(34):5442-7
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  • [Title] Prospective study of a pirarubicin, intermediate-dose cytarabine, and etoposide regimen in children with Down syndrome and acute myeloid leukemia: the Japanese Childhood AML Cooperative Study Group.
  • PURPOSE: To evaluate a less intensive chemotherapeutic regimen specifically designed for patients with Down syndrome (DS) and acute myeloid leukemia (AML), and to determine the prognostic factors for event-free survival.
  • PATIENTS AND METHODS: Seventy-two patients with AML-DS were treated with remission induction chemotherapy consisting of pirarubicin (25 mg/m2/d for 2 days), cytarabine (100 mg/m2/d for 7 days), and etoposide (150 mg/m2/d for 3 days).
  • Patients received four courses of intensification therapy of the same regimen.
  • Prophylaxis for CNS leukemia was not included.
  • RESULTS: All but two patients were younger than 4 years, and 67 of the 72 patients (93%) were diagnosed as acute megakaryoblastic leukemia (AMKL).
  • Seventy of the 72 patients (97.2%) achieved a complete remission (CR), and the estimated 4-year event-free survival (EFS) rate was 83% +/- 9%.
  • Risk-oriented therapy should be considered for future trials in AML-DS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Down Syndrome / complications. Leukemia, Megakaryoblastic, Acute / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Cytarabine / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Doxorubicin / analogs & derivatives. Etoposide / administration & dosage. Female. Humans. Infant. Male. Prospective Studies. Treatment Outcome


21. Chang HH, Lu MY, Jou ST, Lin KH, Tien HF, Wu ET, Lin DT: Neoplastic disorders of hematopoiesis in children with Down's syndrome--a single institution experience in Taiwan. J Formos Med Assoc; 2005 May;104(5):333-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • This study analyzed the clinical characteristics and treatment outcomes of 16 children with DS who developed neoplastic disorders.
  • METHODS: All DS patients aged < 18 years of age with a diagnosis of leukemia or myelodysplastic syndrome (MDS) from 1990 to 2002 were included in this retrospective study.
  • The clinical and laboratory characteristics of patients, including age at diagnosis, gender, initial hemogram, cytogenetic findings, immunophenotype, treatment regimen and outcomes were analyzed.
  • Among them, 9 patients (56%) had acute myeloid leukemia (AML), mainly of the megakaryoblastic subtype.
  • Three of these patients developed MDS prior to the onset of AML.
  • Of the 5 patients who underwent chemotherapy, 3 remained in remission with a survival time of 29, 59, and 109 months, and the remaining 2 died as a consequence of chemotherapy toxicity.
  • Among the 6 patients (38%) who developed transient myeloproliferative disorder, 2 were lost to follow-up, 2 died from DS-associated congenital heart abnormalities and 2 survived without any AML changes.
  • The remaining 1 patient (6%) who developed ALL was still in his first remission although this patient suffered profound chemotherapy complications during treatment.
  • CONCLUSIONS: This study found that AML is the most common hematologic neoplasm in Taiwanese children with DS, especially megakaryoblastic leukemia.
  • Long-term remission of AML in DS patients can be achieved with appropriate treatment.

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  • (PMID = 15959600.001).
  • [ISSN] 0929-6646
  • [Journal-full-title] Journal of the Formosan Medical Association = Taiwan yi zhi
  • [ISO-abbreviation] J. Formos. Med. Assoc.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] Singapore
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22. Razzouk BI, Raimondi SC, Srivastava DK, Pritchard M, Behm FG, Tong X, Sandlund JT, Rubnitz JE, Pui CH, Ribeiro RC: Impact of treatment on the outcome of acute myeloid leukemia with inversion 16: a single institution's experience. Leukemia; 2001 Sep;15(9):1326-30
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  • [Title] Impact of treatment on the outcome of acute myeloid leukemia with inversion 16: a single institution's experience.
  • To identify treatment factors that may affect the survival of children with inv(16)(p13.1q22), we compared the outcomes of 19 patients with this genetic feature treated at our institution during two treatment eras.
  • All entered complete remission (CR) with induction therapy.
  • Eight of the nine children treated in era 1 died, seven of relapsed leukemia.
  • Era 2 treatment protocols differed from those of era 1 in their use of higher doses of cytarabine and etoposide during induction and consolidation chemotherapy and in their use of 2-chlorodeoxyadenosine (2-CDA).
  • This study underscores the dependence of the prognostic impact of cytogenetic features on the efficacy of treatment.
  • [MeSH-major] Leukemia, Myeloid / therapy
  • [MeSH-minor] Acute Disease. Adolescent. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / therapeutic use. Bone Marrow Transplantation. Child. Child, Preschool. Chromosome Inversion. Chromosomes, Human, Pair 16. Cladribine / therapeutic use. Combined Modality Therapy. Cytarabine / administration & dosage. Cytarabine / therapeutic use. Drug Therapy, Combination. Etoposide / administration & dosage. Etoposide / therapeutic use. Female. Humans. Infant. Leukemia, Megakaryoblastic, Acute / genetics. Leukemia, Megakaryoblastic, Acute / therapy. Leukemia, Monocytic, Acute / genetics. Leukemia, Monocytic, Acute / therapy. Leukemia, Myeloid, Acute / genetics. Leukemia, Myeloid, Acute / therapy. Leukemia, Myelomonocytic, Acute / genetics. Leukemia, Myelomonocytic, Acute / therapy. Male. Prognosis. Treatment Outcome

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  • (PMID = 11516092.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA20180; United States / NCI NIH HHS / CA / CA21765
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 04079A1RDZ / Cytarabine; 47M74X9YT5 / Cladribine; 6PLQ3CP4P3 / Etoposide
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23. Taketani T, Taki T, Sako M, Ishii T, Yamaguchi S, Hayashi Y: MNX1-ETV6 fusion gene in an acute megakaryoblastic leukemia and expression of the MNX1 gene in leukemia and normal B cell lines. Cancer Genet Cytogenet; 2008 Oct 15;186(2):115-9
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  • [Title] MNX1-ETV6 fusion gene in an acute megakaryoblastic leukemia and expression of the MNX1 gene in leukemia and normal B cell lines.
  • Patients with infant acute myeloid leukemia (AML) who carry a t(7;12)(q36;p13) translocation have been reported to have a poor clinical outcome.
  • A 23-month-old girl with acute megakaryoblastic leukemia (AMKL) exhibited chromosome abnormalities, including add(7)(q22), and del(12)(p12p13).
  • The patient responded well to chemotherapy, achieved complete remission, and at writing had been in complete remission for 60 months.
  • [MeSH-major] B-Lymphocytes / metabolism. Gene Fusion. Homeodomain Proteins / genetics. Leukemia / genetics. Leukemia, Megakaryoblastic, Acute / genetics. Proto-Oncogene Proteins c-ets / genetics. Repressor Proteins / genetics. Transcription Factors / genetics

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  • (PMID = 18940475.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ETS translocation variant 6 protein; 0 / Homeodomain Proteins; 0 / MNX1 protein, human; 0 / Proto-Oncogene Proteins c-ets; 0 / Repressor Proteins; 0 / Transcription Factors
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24. Kojima S, Sako M, Kato K, Hosoi G, Sato T, Ohara A, Koike K, Okimoto Y, Nishimura S, Akiyama Y, Yoshikawa T, Ishii E, Okamura J, Yazaki M, Hayashi Y, Eguchi M, Tsukimoto I, Ueda K: An effective chemotherapeutic regimen for acute myeloid leukemia and myelodysplastic syndrome in children with Down's syndrome. Leukemia; 2000 May;14(5):786-91
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  • [Title] An effective chemotherapeutic regimen for acute myeloid leukemia and myelodysplastic syndrome in children with Down's syndrome.
  • In recent pediatric collaborative studies of acute myeloid leukemia (AML), patients with Down's syndrome (DS) have better outcome than other patients when they were treated according to their intensive AML protocols.
  • Remission induction chemotherapy consisted of daunorubicin (25 mg/m2/day for 2 days), cytosine arabinoside (100 mg/m2/day for 7 days), and etoposide (150 mg/m2/day for 3 days).
  • Patients received one to seven courses of consolidation therapy of the same regimen.
  • Of the 33 patients, all were younger than 4 years and diagnosed as having acute megakaryoblastic leukemia or myelodysplastic syndrome.
  • All patients achieved a complete remission and estimated 8 year event-free survival rate was 80+/-7%.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Down Syndrome / complications. Leukemia, Myeloid, Acute / complications. Leukemia, Myeloid, Acute / drug therapy. Myelodysplastic Syndromes / complications. Myelodysplastic Syndromes / drug therapy
  • [MeSH-minor] Child, Preschool. Cytarabine / administration & dosage. Daunorubicin / administration & dosage. Disease-Free Survival. Etoposide / administration & dosage. Female. Humans. Infant. Leukemia, Megakaryoblastic, Acute / complications. Leukemia, Megakaryoblastic, Acute / drug therapy. Male. Probability. Remission Induction. Survival Rate. Time Factors. Treatment Outcome


25. Mattei D, Mordini N, Lo Nigro C, Ghirardo D, Ferrua MT, Osenda M, Gallamini A, Bacigalupo A, Viscoli C: Voriconazole in the management of invasive aspergillosis in two patients with acute myeloid leukemia undergoing stem cell transplantation. Bone Marrow Transplant; 2002 Dec;30(12):967-70
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  • [Title] Voriconazole in the management of invasive aspergillosis in two patients with acute myeloid leukemia undergoing stem cell transplantation.
  • A major problem is how to manage patients who had invasive aspergillosis during remission induction and consolidation therapy and then undergo SCT.
  • We report two cases of patients who underwent remission induction for AML, developed invasive aspergillosis during antifungal prophylaxis with itraconazole, failed amphotericin B deoxycholate and liposomal amphotericin B treatment, were successfully treated with voriconazole and eventually underwent SCT with voriconazole prophylaxis without reactivation of invasive aspergillosis.
  • [MeSH-major] Aspergillosis / drug therapy. Bone Marrow Transplantation / adverse effects. Deoxycholic Acid / analogs & derivatives. Leukemia, Megakaryoblastic, Acute / therapy. Leukemia, Myelomonocytic, Acute / therapy. Lung Diseases, Fungal / drug therapy. Peripheral Blood Stem Cell Transplantation / adverse effects. Pyrimidines / therapeutic use. Triazoles / therapeutic use
  • [MeSH-minor] Adult. Amphotericin B / administration & dosage. Amphotericin B / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Cytarabine / administration & dosage. Cytarabine / adverse effects. Daunorubicin / administration & dosage. Daunorubicin / adverse effects. Drug Combinations. Drug Resistance, Fungal. Etoposide / administration & dosage. Etoposide / adverse effects. Fatal Outcome. Humans. Immunocompromised Host. Itraconazole / therapeutic use. Liposomes. Male. Middle Aged. Recurrence. Remission Induction. Salvage Therapy. Transplantation Conditioning / adverse effects. Transplantation, Autologous / adverse effects. Transplantation, Homologous / adverse effects. Voriconazole

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  • (PMID = 12476292.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Drug Combinations; 0 / Liposomes; 0 / Pyrimidines; 0 / Triazoles; 0 / liposomal amphotericin B; 005990WHZZ / Deoxycholic Acid; 04079A1RDZ / Cytarabine; 304NUG5GF4 / Itraconazole; 6PLQ3CP4P3 / Etoposide; 7XU7A7DROE / Amphotericin B; 87687-70-5 / amphotericin B, deoxycholate drug combination; JFU09I87TR / Voriconazole; ZS7284E0ZP / Daunorubicin
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26. Massey GV, Zipursky A, Chang MN, Doyle JJ, Nasim S, Taub JW, Ravindranath Y, Dahl G, Weinstein HJ, Children's Oncology Group (COG): A prospective study of the natural history of transient leukemia (TL) in neonates with Down syndrome (DS): Children's Oncology Group (COG) study POG-9481. Blood; 2006 Jun 15;107(12):4606-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A prospective study of the natural history of transient leukemia (TL) in neonates with Down syndrome (DS): Children's Oncology Group (COG) study POG-9481.
  • A unique transient leukemia (TL) has been described in newborns with Down syndrome (DS; or trisomy 21 mosaics).
  • This leukemia has a high incidence of spontaneous remission; however, early death and subsequent development of acute megakaryoblastic leukemia (AMKL) have been reported.
  • We prospectively evaluated 48 infants with DS and TL to determine the natural history and biologic characteristics of this disease, identify the clinical characteristics associated with early death or subsequent leukemia, and assess the incidence of subsequent leukemia.
  • Most children were able to spontaneously clear peripheral blasts (89%), normalize blood counts (74%), and maintain a complete remission (64%).
  • Early death occurred in 17% of infants and was significantly correlated with higher white blood cell count at diagnosis (P < .001), increased bilirubin and liver enzymes (P < .005), and a failure to normalize the blood count (P = .001).
  • Recurrence of leukemia occurred in 19% of infants at a mean of 20 months.
  • Development of leukemia was significantly correlated with karyotypic abnormalities in addition to trisomy 21 (P = .037).
  • Ongoing collaborative clinical studies are needed to determine the optimal role of chemotherapy for infants at risk for increased mortality or disease recurrence and to further the knowledge of the unique biologic features of this TL.
  • [MeSH-major] Chromosomes, Human, Pair 21. Down Syndrome. Leukemia, Megakaryoblastic, Acute. Mosaicism. Trisomy

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  • [CommentIn] Blood. 2006 Dec 1;108(12):3952-3; author reply 3953 [17114573.001]
  • (PMID = 16469874.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzymes; RFM9X3LJ49 / Bilirubin
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27. Sandoval C, Pine SR, Guo Q, Sastry S, Stewart J, Kronn D, Jayabose S: Tetrasomy 21 transient leukemia with a GATA1 mutation in a phenotypically normal trisomy 21 mosaic infant: case report and review of the literature. Pediatr Blood Cancer; 2005 Jan;44(1):85-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tetrasomy 21 transient leukemia with a GATA1 mutation in a phenotypically normal trisomy 21 mosaic infant: case report and review of the literature.
  • Infants with constitutional trisomy 21 are at increased risk of developing transient and acute megakaryoblastic leukemia (AMKL).
  • Mutations in GATA1 have been identified in trisomy 21 patients with AMKL, and this lesion is thought to be an initial event by virtue of its presence during transient leukemia.
  • Transient leukemia is also observed in phenotypically normal infants albeit much less commonly so.
  • Almost all these infants are mosaic for trisomy 21, and the clinical course of transient leukemia recapitulates that observed in constitutional trisomy 21.
  • We report a phenotypically normal infant with tetrasomy 21 transient leukemia, GATA1 mutation within exon 2, and trisomy 21 mosaicism restricted to the hematopoietic tissue.
  • Two years after diagnosis, low levels of trisomy 21 persisted in the peripheral blood, which resolved 2.5 years after diagnosis.
  • The literature review identified 32 phenotypically normal infants with transient leukemia.
  • Ninety-one percent (29 of 32) were observed and three received chemotherapy at diagnosis of transient leukemia.
  • Nineteen percent (6 of 32) developed acute leukemia, and four continued in remission (two died).
  • Transient leukemia in trisomy 21 mosaicism recapitulates the condition observed in constitutional trisomy 21 at the biological and clinical levels.
  • Infants should be followed for the development of acute leukemia.
  • [MeSH-major] DNA-Binding Proteins / genetics. Down Syndrome / genetics. Leukemia, Megakaryoblastic, Acute / genetics. Transcription Factors / genetics
  • [MeSH-minor] Aneuploidy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Erythroid-Specific DNA-Binding Factors. Female. GATA1 Transcription Factor. Humans. Infant, Newborn. Male. Mutation. Phenotype. Prognosis. Risk Factors

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  • [Copyright] (c) 2004 Wiley-Liss, Inc.
  • (PMID = 15390279.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / Erythroid-Specific DNA-Binding Factors; 0 / GATA1 Transcription Factor; 0 / GATA1 protein, human; 0 / Transcription Factors
  • [Number-of-references] 46
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28. Papageorgiou SG, Pappa V, Economopoulou C, Tsirigotis P, Konsioti F, Ionnidou ED, Chondropoulos S, Vasilatou D, Papageorgiou E, Economopoulos T, Dervenoulas J: Dasatinib induces long-term remission in imatinib-resistant Philadelphia chromosome-positive acute megakaryoblastic leukemia but fails to prevent development of central nervous system progression. Leuk Res; 2010 Sep;34(9):e254-6
Hazardous Substances Data Bank. IMATINIB MESYLATE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dasatinib induces long-term remission in imatinib-resistant Philadelphia chromosome-positive acute megakaryoblastic leukemia but fails to prevent development of central nervous system progression.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Central Nervous System Neoplasms / secondary. Leukemia, Megakaryoblastic, Acute / drug therapy. Philadelphia Chromosome. Piperazines / therapeutic use. Pyrimidines / therapeutic use. Thiazoles / therapeutic use
  • [MeSH-minor] Benzamides. Dasatinib. Drug Resistance, Neoplasm. Humans. Imatinib Mesylate. Karyotyping. Male. Middle Aged. Remission Induction

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  • (PMID = 20392492.001).
  • [ISSN] 1873-5835
  • [Journal-full-title] Leukemia research
  • [ISO-abbreviation] Leuk. Res.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 0 / Thiazoles; 8A1O1M485B / Imatinib Mesylate; RBZ1571X5H / Dasatinib
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29. Reinhardt D, Diekamp S, Langebrake C, Ritter J, Stary J, Dworzak M, Schrauder A, Zimmermann M, Fleischhack G, Ludwig WD, Harbott J, Creutzig U: Acute megakaryoblastic leukemia in children and adolescents, excluding Down's syndrome: improved outcome with intensified induction treatment. Leukemia; 2005 Aug;19(8):1495-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Acute megakaryoblastic leukemia in children and adolescents, excluding Down's syndrome: improved outcome with intensified induction treatment.
  • [MeSH-major] Leukemia, Megakaryoblastic, Acute / drug therapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Clinical Trials as Topic. Cytarabine / therapeutic use. Etoposide / therapeutic use. Female. Humans. Idarubicin / therapeutic use. Infant. Male. Remission Induction / methods. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 15920489.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 04079A1RDZ / Cytarabine; 6PLQ3CP4P3 / Etoposide; ZRP63D75JW / Idarubicin; ICE protocol 4
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