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3. 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.
  • Six patients had Down syndrome and AMKL, 6 had secondary AMKL, and 29 had de novo AMKL.
  • Patients with Down syndrome had a significantly higher 2-year event-free survival (EFS) estimate (83%) than did other patients with de novo AMKL (14%) or with secondary AMKL (20%; P < or =.038).
  • 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.
  • 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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4. 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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5. Henderson R, Spence L: Down syndrome with myelodysplasia of megakaryoblastic lineage. Clin Lab Sci; 2006;19(3):161-4
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  • [Title] Down syndrome with myelodysplasia of megakaryoblastic lineage.
  • The association between Down syndrome and acute myelogenous leukemia (AML) has been well documented.
  • AML in Down syndrome is usually a specific type of megakaryoblastic leukemia (M7, AMKL).
  • A myelodysplastic syndrome generally precedes this malignancy.
  • Down syndrome patients with AMKL have a much better prognosis than other children with AML.
  • A case study of a 22-month-old female with Down syndrome and myelodysplastic syndrome of a megakaryoblastic lineage is presented here.
  • The patient was placed on a national research group study and began chemotherapy treatment.
  • To date she has received two courses of cytarabine (ara-c) and daunorubicin therapy, which were tolerated well, and is awaiting her third course.
  • Her blood counts stabilize for a while after treatments and her prognosis is good.
  • [MeSH-major] Blood Platelets / pathology. Down Syndrome / complications. Megakaryocytes / pathology. Myelodysplastic Syndromes / complications
  • [MeSH-minor] Blood Cell Count. Drug Therapy. Female. Humans. Infant. Prognosis

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  • (PMID = 16910232.001).
  • [ISSN] 0894-959X
  • [Journal-full-title] Clinical laboratory science : journal of the American Society for Medical Technology
  • [ISO-abbreviation] Clin Lab Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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6. Ge Y, Jensen TL, Matherly LH, Taub JW: Transcriptional regulation of the cystathionine-beta -synthase gene in Down syndrome and non-Down syndrome megakaryocytic leukemia cell lines. Blood; 2003 Feb 15;101(4):1551-7
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  • [Title] Transcriptional regulation of the cystathionine-beta -synthase gene in Down syndrome and non-Down syndrome megakaryocytic leukemia cell lines.
  • Children with Down syndrome (DS) with acute myeloid leukemia (AML) have significantly higher event-free survival rates compared to those with non-DS AML, linked to greater cytosine arabinoside (ara-C) sensitivity and higher transcript levels of the chromosome 21-localized gene, cystathionine-beta-synthase (CBS), in DS myeloblasts.
  • In this study, we examined the transcriptional regulation of the CBS gene in the DS megakaryocytic leukemia (AMkL) cell line, CMK, characterized by significantly higher CBS transcripts compared with the non-DS AMkL cell line, CMS.
  • Decreased binding of Sp1/Sp3 in CMK nuclear extracts following treatment with calf alkaline phosphatase suggested a role for phosphorylation of Sp1/Sp3 in regulating CBS promoter activity and in the differential CBS expression between CMK and CMS cells.
  • The results of this study with clinically relevant cell line models suggest potential mechanisms for disparate patterns of CBS gene expression in DS and non-DS myeloblasts and may, in part, explain the greater sensitivity to chemotherapy shown by patients with DS AML.
  • [MeSH-major] Cystathionine beta-Synthase / genetics. Down Syndrome / enzymology. Gene Expression Regulation, Enzymologic. Leukemia, Megakaryoblastic, Acute / enzymology

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  • (PMID = 12393509.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA92308
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / SP3 protein, human; 0 / Sp1 Transcription Factor; 0 / Transcription Factors; 04079A1RDZ / Cytarabine; 148710-94-5 / Sp3 Transcription Factor; 9007-49-2 / DNA; EC 1.13.12.- / Luciferases; EC 4.2.1.22 / Cystathionine beta-Synthase
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7. Ge Y, Dombkowski AA, LaFiura KM, Tatman D, Yedidi RS, Stout ML, Buck SA, Massey G, Becton DL, Weinstein HJ, Ravindranath Y, Matherly LH, Taub JW: Differential gene expression, GATA1 target genes, and the chemotherapy sensitivity of Down syndrome megakaryocytic leukemia. Blood; 2006 Feb 15;107(4):1570-81
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  • [Title] Differential gene expression, GATA1 target genes, and the chemotherapy sensitivity of Down syndrome megakaryocytic leukemia.
  • Children with Down syndrome (DS) with acute megakaryocytic leukemia (AMkL) have very high survival rates compared with non-DS AMkL patients.
  • Transcripts for the bone marrow stromal-cell antigen 2 (BST2) gene, encoding a transmembrane glycoprotein potentially involved in interactions between leukemia cells and bone marrow stromal cells, were 7.3-fold higher (validated by real-time polymerase chain reaction) in the non-DS compared with the DS group.

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  • (PMID = 16249385.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NIEHS NIH HHS / ES / P30 ES06639; United States / NCI NIH HHS / CA / R01 CA92308
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA Primers; 0 / GATA1 Transcription Factor; 0 / GATA1 protein, human; 04079A1RDZ / Cytarabine; EC 1.13.12.- / Luciferases
  • [Other-IDs] NLM/ PMC1895418
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8. Dastugue N, Lafage-Pochitaloff M, Pagès MP, Radford I, Bastard C, Talmant P, Mozziconacci MJ, Léonard C, Bilhou-Nabéra C, Cabrol C, Capodano AM, Cornillet-Lefebvre P, Lessard M, Mugneret F, Pérot C, Taviaux S, Fenneteaux O, Duchayne E, Berger R, Groupe Français d'Hematologie Cellulaire: Cytogenetic profile of childhood and adult megakaryoblastic leukemia (M7): a study of the Groupe Français de Cytogénétique Hématologique (GFCH). Blood; 2002 Jul 15;100(2):618-26
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  • [Title] Cytogenetic profile of childhood and adult megakaryoblastic leukemia (M7): a study of the Groupe Français de Cytogénétique Hématologique (GFCH).
  • To draw the cytogenetic profile of childhood and adult acute megakaryoblastic leukemia (M7), the Groupe Français de Cytogénétique Hématologique collected 53 cases of M7 (30 children and 23 adults).
  • Nine cytogenetic groups were identified: normal karyotypes (group 1), patients with Down syndrome (group 2), numerical abnormalities only (group 3), t(1;22)(p13;q13) or OTT-MAL transcript (group 4), t(9;22)(q34;q11) (group 5), 3q21q26 (group 6), -5/del(5q) or -7/del(7q) or both (group 7), i(12)(p10) (group 8), and other structural changes (group 9).
  • Although 90.5% of cases had no documented antecedent hematologic disorder or exposure to chemotherapy or radiotherapy, the morphologic and the cytogenetic findings indicated that M7 might be a secondary leukemia more often than suggested by preceding history, particularly among adults.
  • [MeSH-major] Cytogenetic Analysis. Leukemia, Megakaryoblastic, Acute / genetics

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  • [CommentIn] Blood. 2002 Nov 15;100(10):3838; author reply 3838-9 [12411327.001]
  • (PMID = 12091356.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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9. Al-Ahmari A, Shah N, Sung L, Zipursky A, Hitzler J: Long-term results of an ultra low-dose cytarabine-based regimen for the treatment of acute megakaryoblastic leukaemia in children with Down syndrome. Br J Haematol; 2006 Jun;133(6):646-8
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  • [Title] Long-term results of an ultra low-dose cytarabine-based regimen for the treatment of acute megakaryoblastic leukaemia in children with Down syndrome.
  • Children with acute megakaryoblastic leukaemia (AMKL) and Down syndrome (DS) show a favourable response to chemotherapy, probably due to increased sensitivity of the leukaemic blasts to cytarabine.
  • In contrast, dose-intensive approaches have resulted in disproportionate treatment-related mortality in this group.
  • The survival of children with AMKL and DS was retrospectively compared following treatment with a low-dose chemotherapy protocol, consisting of cytarabine (10 mg/m2/dose), retinylpalmitate and vincristine or standard chemotherapy.
  • Further reduction of treatment intensity in AMKL of children with DS, therefore, appears feasible.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Down Syndrome / drug therapy. Leukemia, Megakaryoblastic, Acute / drug therapy
  • [MeSH-minor] Child. Cytarabine / administration & dosage. Drug Administration Schedule. Epidemiologic Methods. Humans. Treatment Outcome. Vincristine / administration & dosage. Vitamin A / administration & dosage. Vitamin A / analogs & derivatives

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  • (PMID = 16704441.001).
  • [ISSN] 0007-1048
  • [Journal-full-title] British journal of haematology
  • [ISO-abbreviation] Br. J. Haematol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 04079A1RDZ / Cytarabine; 11103-57-4 / Vitamin A; 1D1K0N0VVC / retinol palmitate; 5J49Q6B70F / Vincristine
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10. Ariffin H, Garcia JC, Daud SS, Ibrahim K, Aizah N, Ong GB, Chong LA, Mohamad Z: GATA1 mutations in patients with down syndrome and acute megakaryoblastic leukaemia do not always confer a good prognosis. Pediatr Blood Cancer; 2009 Jul;53(1):108-11
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  • [Title] GATA1 mutations in patients with down syndrome and acute megakaryoblastic leukaemia do not always confer a good prognosis.
  • Children with Down syndrome and acute megakaryoblastic leukemia (DS-AMKL) have been shown to have increased sensitivity to cytarabine based chemotherapy.
  • [MeSH-major] Cytidine Deaminase / metabolism. Down Syndrome / genetics. GATA1 Transcription Factor / genetics. Leukemia, Megakaryoblastic, Acute / genetics. Mutation
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow / pathology. Child, Preschool. Female. Humans. Infant. Prognosis

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • (PMID = 19260099.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / GATA1 Transcription Factor; 0 / GATA1 protein, human; EC 3.5.4.5 / Cytidine Deaminase
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11. Savaşan S, Buck S, Raimondi SC, Becton DL, Weinstein H, Chang M, Ravindranath Y: CD36 (thrombospondin receptor) expression in childhood acute megakaryoblastic leukemia: in vitro drug sensitivity and outcome. Leuk Lymphoma; 2006 Oct;47(10):2076-83
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  • [Title] CD36 (thrombospondin receptor) expression in childhood acute megakaryoblastic leukemia: in vitro drug sensitivity and outcome.
  • The outcome for children with acute megakaryoblastic leukemia (AMKL) remains poor, except for cases associated with Down syndrome (DS).
  • This study compared immunophenotypic and drug sensitivity patterns of childhood AMKL cases with or without DS.
  • In children without DS, high expression of CD36 on AMKL blasts identified a sub-group with immunophenotypic and drug sensitivity patterns similar to that of DS AMKL.
  • CD36 expression in acute myeloid leukemia cases other than AMKL was not associated with increased in vitro drug sensitivity.
  • CD36 expression in AMKL may be an indicator of megakaryoblast maturation and chemotherapy sensitivity.
  • [MeSH-major] Antigens, CD36 / biosynthesis. Gene Expression Regulation, Neoplastic. Leukemia, Megakaryoblastic, Acute / complications. Leukemia, Megakaryoblastic, Acute / metabolism
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor. Cell Membrane / metabolism. Child. Child, Preschool. Cytarabine / pharmacology. Daunorubicin / pharmacology. Down Syndrome / complications. Humans. Immunophenotyping. Infant. Infant, Newborn. Sensitivity and Specificity. Treatment Outcome

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  • [CommentIn] Leuk Lymphoma. 2006 Oct;47(10):2004-5 [17071466.001]
  • (PMID = 17071479.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-21765; United States / NCI NIH HHS / CA / U10 CA 30969
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD36; 0 / Biomarkers, Tumor; 04079A1RDZ / Cytarabine; ZS7284E0ZP / Daunorubicin
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12. Kudo K, Hama A, Kojima S, Ishii R, Morimoto A, Bessho F, Sunami S, Kobayashi N, Kinoshita A, Okimoto Y, Tawa A, Tsukimoto I: Mosaic Down syndrome-associated acute myeloid leukemia does not require high-dose cytarabine treatment for induction and consolidation therapy. Int J Hematol; 2010 May;91(4):630-5
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  • [Title] Mosaic Down syndrome-associated acute myeloid leukemia does not require high-dose cytarabine treatment for induction and consolidation therapy.
  • The present study aimed to identify optimal treatment intensity in children with mosaic Down syndrome (DS) and acute megakaryoblastic leukemia (AMKL).
  • UPN5 with GATA1 mutation suffered from acute pneumonia and pancreatitis and discontinued chemotherapy.
  • UPN7 relapsed after cessation of chemotherapy and was rescued with allo-PBSCT.
  • [MeSH-major] Antimetabolites, Antineoplastic / administration & dosage. Cytarabine / administration & dosage. Down Syndrome / genetics. Down Syndrome / mortality. Leukemia, Megakaryoblastic, Acute. Mosaicism
  • [MeSH-minor] Child, Preschool. Disease-Free Survival. Dose-Response Relationship, Drug. Female. GATA1 Transcription Factor / genetics. Humans. Infant. Male. Prevalence. Retrospective Studies. Survival Rate


13. Brink DS: Transient leukemia (transient myeloproliferative disorder, transient abnormal myelopoiesis) of Down syndrome. Adv Anat Pathol; 2006 Sep;13(5):256-62
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  • [Title] Transient leukemia (transient myeloproliferative disorder, transient abnormal myelopoiesis) of Down syndrome.
  • Transient leukemia of Down syndrome (DS-TL), also known as transient myeloproliferative disorder of Down syndrome (DS) and transient abnormal myelopoiesis of DS, occurs in approximately 10% of DS neonates and in phenotypically normal neonates with trisomy 21 mosaicism.
  • Despite its typical transient nature, 20% to 30% of DS-TL patients develop overt (nontransient) acute leukemia, usually within 3 years and typically of the M7 phenotype (acute megakaryoblastic leukemia).
  • The pathogenesis of DS-TL (and of subsequent acute leukemia) involves mutation of GATA1 (on chromosome X), which normally encodes a transcription factor integral to normal development of erythroid, megakaryocytic, and basophilic/mast cell lines.
  • Though indications for chemotherapy in DS-TL have not been firmly established, the blasts of DS-TL are sensitive to low-dose cytosine arabinoside.
  • [MeSH-major] Down Syndrome / complications. Myeloproliferative Disorders / complications. Myeloproliferative Disorders / physiopathology
  • [MeSH-minor] GATA1 Transcription Factor / genetics. Humans. Hydrops Fetalis / etiology. Infant, Newborn. Leukemia / etiology. Liver Diseases / etiology. Mutation

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  • (PMID = 16998319.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / GATA1 Transcription Factor; 0 / GATA1 protein, human
  • [Number-of-references] 81
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14. Duchayne E, Fenneteau O, Pages MP, Sainty D, Arnoulet C, Dastugue N, Garand R, Flandrin G, Groupe Français d'Hématologie Cellulaire, Groupe Français de Cytogénétique Hématologique: Acute megakaryoblastic leukaemia: a national clinical and biological study of 53 adult and childhood cases by the Groupe Français d'Hématologie Cellulaire (GFHC). Leuk Lymphoma; 2003 Jan;44(1):49-58

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Acute megakaryoblastic leukaemia: a national clinical and biological study of 53 adult and childhood cases by the Groupe Français d'Hématologie Cellulaire (GFHC).
  • This study is based on accurate morphological and immunological study to select pure megakaryoblastic proliferations and to eliminate megakaryocytic participation in haemopathies.
  • Among children, besides the well-known Down syndrome M7, we in particular, studied ten t(1;22) M7 and one OTT-MAL transcript positive case with normal karyotype presenting specific features.
  • We were already aware of their younger age, female and tumoral presentation, but we also found a lower percentage of bone marrow blasts, sometimes without any megakaryoblastic bone marrow involvement, but always, with a dysmegakaryocytopoiesis associated with micromegakaryocytes.
  • They are generally good responders to intensive AML chemotherapy with very long disease-free survivals (DFS).
  • [MeSH-major] Leukemia, Megakaryoblastic, Acute / classification. Leukemia, Megakaryoblastic, Acute / pathology

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  • (PMID = 12691142.001).
  • [ISSN] 1042-8194
  • [Journal-full-title] Leukemia & lymphoma
  • [ISO-abbreviation] Leuk. Lymphoma
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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15. Webb DK: Optimizing therapy for myeloid disorders of Down syndrome. Br J Haematol; 2005 Oct;131(1):3-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optimizing therapy for myeloid disorders of Down syndrome.
  • Children with Down syndrome (DS) are at increased risk of leukaemia.
  • 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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16. Ge Y, Stout ML, Tatman DA, Jensen TL, Buck S, Thomas RL, Ravindranath Y, Matherly LH, Taub JW: GATA1, cytidine deaminase, and the high cure rate of Down syndrome children with acute megakaryocytic leukemia. J Natl Cancer Inst; 2005 Feb 2;97(3):226-31
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  • [Title] GATA1, cytidine deaminase, and the high cure rate of Down syndrome children with acute megakaryocytic leukemia.
  • Down syndrome children with acute megakaryocytic leukemia (AMkL) have higher cure rates than non-Down syndrome acute myeloid leukemia (AML) patients treated with cytosine arabinoside (ara-C).
  • Megakaryoblasts from Down syndrome AML patients are more sensitive in vitro to ara-C than cells from non-Down syndrome AML patients.
  • Somatic mutations in the GATA1 transcription factor have been detected exclusively and almost uniformly in Down syndrome AMkL patients, suggesting a potential linkage to the chemotherapy sensitivity of Down syndrome megakaryoblasts.
  • Stable transfection of wild-type GATA1 cDNA into the Down syndrome AMkL cell line CMK resulted in decreased (8- to 17-fold) ara-C sensitivity and a threefold-lower generation of the active ara-C metabolite ara-CTP compared with that for mock-transfected CMK cells.
  • Cytidine deaminase transcripts were a median 5.1-fold (P = .002) lower in Down syndrome megakaryoblasts (n = 16) than in blast cells from non-Down syndrome patients (n = 56).
  • These results suggest that GATA1 transcriptionally upregulates cytidine deaminase and that the presence or absence of GATA1 mutations in AML blasts likely confers differences in ara-C sensitivities due to effects on cytidine deaminase gene expression, which, in turn, contributes to the high cure rate of Down syndrome AMkL patients.
  • [MeSH-major] Cytidine Deaminase / metabolism. DNA-Binding Proteins / metabolism. Down Syndrome / complications. Down Syndrome / metabolism. Leukemia, Megakaryoblastic, Acute / metabolism. Transcription Factors / metabolism
  • [MeSH-minor] Antimetabolites, Antineoplastic / metabolism. Arabinofuranosylcytosine Triphosphate / metabolism. Arabinofuranosyluracil / metabolism. Blotting, Western. Child. Cytarabine / metabolism. Erythroid-Specific DNA-Binding Factors. GATA1 Transcription Factor. Humans. Polymerase Chain Reaction. Time Factors. Transcription, Genetic. Transcriptional Activation. Up-Regulation

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  • (PMID = 15687366.001).
  • [ISSN] 1460-2105
  • [Journal-full-title] Journal of the National Cancer Institute
  • [ISO-abbreviation] J. Natl. Cancer Inst.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA92308
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / DNA-Binding Proteins; 0 / Erythroid-Specific DNA-Binding Factors; 0 / GATA1 Transcription Factor; 0 / GATA1 protein, human; 0 / Transcription Factors; 04079A1RDZ / Cytarabine; 13191-15-6 / Arabinofuranosylcytosine Triphosphate; 3083-77-0 / Arabinofuranosyluracil; EC 3.5.4.5 / Cytidine Deaminase
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17. 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.
  • [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


18. Edwards H, Xie C, LaFiura KM, Dombkowski AA, Buck SA, Boerner JL, Taub JW, Matherly LH, Ge Y: RUNX1 regulates phosphoinositide 3-kinase/AKT pathway: role in chemotherapy sensitivity in acute megakaryocytic leukemia. Blood; 2009 Sep 24;114(13):2744-52
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  • [Title] RUNX1 regulates phosphoinositide 3-kinase/AKT pathway: role in chemotherapy sensitivity in acute megakaryocytic leukemia.
  • Translocations or down-regulation of RUNX1 have been linked to favorable clinical outcomes in acute leukemias, suggesting that RUNX1 may also play critical roles in chemotherapy responses in acute leukemias; however, the molecular mechanisms remain unclear.
  • The median level of RUNX1b transcripts in Down syndrome (DS) children with acute megakaryocytic leukemia (AMkL) were 4.4-fold (P < .001) lower than that in non-DS AMkL cases.
  • Our results suggest that RUNX1 may play a critical role in chemotherapy response in AMkL by regulating the PI3-kinase/Akt pathway.
  • Thus, the treatment of AMkL may be improved by integrating PI3-kinase or Akt inhibitors into the chemotherapy of this disease.

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  • (PMID = 19638627.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NIEHS NIH HHS / ES / P30 ES006639; United States / NCI NIH HHS / CA / R01 CA120772; United States / NCI NIH HHS / CA / CA120772; United States / NIEHS NIH HHS / ES / P30 ES06639
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Core Binding Factor Alpha 2 Subunit; 0 / RNA, Small Interfering; 0 / RUNX1 protein, human; 04079A1RDZ / Cytarabine; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.1.137 / Class I Phosphatidylinositol 3-Kinases; EC 2.7.1.137 / PIK3CD protein, human; EC 2.7.11.1 / Oncogene Protein v-akt
  • [Other-IDs] NLM/ PMC2756129
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19. Taub JW, Ge Y: Down syndrome, drug metabolism and chromosome 21. Pediatr Blood Cancer; 2005 Jan;44(1):33-9
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  • [Title] Down syndrome, drug metabolism and chromosome 21.
  • It has been recognized that chromosomal abnormalities in childhood leukemia, are linked to both leukemogenesis and segregate patients into prognostic treatment groups.
  • This is best exemplified in cases of children with Down syndrome (DS), who have significantly higher risks of developing leukemia compared to non-DS children and distinctive treatment outcomes, particularly in cases of acute myeloid leukemia (AML).
  • The high event-free survival (EFS) rates of DS AML patients and in particular, patients with megakaryocytic leukemia (AMkL), at least in part reflects an increased sensitivity to cytosine arabinoside (ara-C) secondary to increased expression of the chromosome 21-localized gene, cystathionine-beta-synthase, and potentially global mechanisms which increase the susceptibility of cells to undergo apoptosis.
  • Somatic mutations of the X-linked transcription factor gene, GATA1, have been detected uniformly and exclusively in DS AMkL cases, which may lead to altered expression of GATA1 target genes and alter the metabolism of drugs including ara-C.
  • Hyperdiploid acute lymphoblastic leukemia (ALL) cells with extra copies of chromosome 21, generate higher levels of the active methotrexate (MTX) metabolite, MTX polyglutamates.
  • Microarray technology should lead to the identification of additional gene targets linked to the treatment response of specific cytogenetic leukemia subgroups.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / metabolism. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chromosomes, Human, Pair 21. Down Syndrome / complications. Leukemia, Megakaryoblastic, Acute / drug therapy. Leukemia, Myeloid / drug therapy
  • [MeSH-minor] Acute Disease. Antineoplastic Agents, Alkylating / adverse effects. Antineoplastic Agents, Alkylating / metabolism. Antineoplastic Agents, Alkylating / therapeutic use. Child. Disease-Free Survival. Humans. Methotrexate / adverse effects. Methotrexate / metabolism. Methotrexate / therapeutic use. Prognosis

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  • [Copyright] (c) 2004 Wiley-Liss, Inc.
  • (PMID = 15390307.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA92308
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; YL5FZ2Y5U1 / Methotrexate
  • [Number-of-references] 45
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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).
  • 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. 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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  • [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.
  • 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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22. Creutzig U, Reinhardt D, Diekamp S, Dworzak M, Stary J, Zimmermann M: AML patients with Down syndrome have a high cure rate with AML-BFM therapy with reduced dose intensity. Leukemia; 2005 Aug;19(8):1355-60
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  • [Title] AML patients with Down syndrome have a high cure rate with AML-BFM therapy with reduced dose intensity.
  • Despite improved prognosis in acute myelogenous leukaemia (AML) children with Down syndrome (DS), therapy-related toxicity remained a problem.
  • AML-DS patients were in median 1.8 years old, and 102/118 (86%) showed the typical morphology of acute megakaryoblastic leukaemia.
  • In study 93, seven DS patients did not receive AML-specific chemotherapy, and treatment modifications were more common.
  • Therapy-related toxicity was generally lower in DS patients treated according to study 98.
  • We conclude that a standardised and dose-reduced treatment schedule including the main components of AML treatment is advisable for AML children with DS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Down Syndrome / complications. Leukemia, Myeloid, Acute / drug therapy
  • [MeSH-minor] Adolescent. Age Factors. Child. Child, Preschool. Cytarabine / administration & dosage. Disease-Free Survival. Etoposide / administration & dosage. Female. Humans. Idarubicin / administration & dosage. Infant. Leukemia, Megakaryoblastic, Acute / pathology. Male. Survival Rate. Treatment Outcome

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  • [Copyright] Leukemia (2005) 19, 1355-1360.
  • (PMID = 15920490.001).
  • [ISSN] 0887-6924
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] eng
  • [Publication-type] Clinical Trial; 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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23. Qayed M, Ahmed I, Valentini RP, Cushing B, Rajpurkar M: Hypercalcemia in pediatric acute megakaryocytic leukemia: case report and review of the literature. J Pediatr Hematol Oncol; 2009 May;31(5):373-6
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  • [Title] Hypercalcemia in pediatric acute megakaryocytic leukemia: case report and review of the literature.
  • Here, we report an 8-month-old non-Down syndrome infant with acute megakaryocytic leukemia and severe hypercalcemia at presentation.
  • A review of the literature reveals that this is the first case of hypercalcemia complicating acute megakaryocytic leukemia reported in the pediatric literature.
  • Though the etiology of this complication remains unclear, our experience suggests that early institution of chemotherapy along with supportive care is the best treatment for control of hypercalcemia.
  • [MeSH-major] Hypercalcemia / etiology. Leukemia, Megakaryoblastic, Acute / complications
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Density Conservation Agents / administration & dosage. Bone Diseases, Metabolic / drug therapy. Bone Diseases, Metabolic / etiology. Bone Diseases, Metabolic / radiography. Diphosphonates / administration & dosage. Humans. Infant. Male

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  • (PMID = 19415024.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bone Density Conservation Agents; 0 / Diphosphonates; OYY3447OMC / pamidronate
  • [Number-of-references] 34
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24. Ge Y, LaFiura KM, Dombkowski AA, Chen Q, Payton SG, Buck SA, Salagrama S, Diakiw AE, Matherly LH, Taub JW: The role of the proto-oncogene ETS2 in acute megakaryocytic leukemia biology and therapy. Leukemia; 2008 Mar;22(3):521-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The role of the proto-oncogene ETS2 in acute megakaryocytic leukemia biology and therapy.
  • Acute myeloid leukemia (AML) in Down syndrome (DS) children has several unique features including a predominance of the acute megakaryocytic leukemia (AMkL) phenotype, higher event-free survivals compared to non-DS children using cytosine arabinoside (ara-C)/anthracycline-based protocols and a uniform presence of somatic mutations in the X-linked transcription factor gene, GATA1.
  • ETS2 transcripts measured by real-time RT-PCR were 1.8- and 4.1-fold, respectively, higher in DS and non-DS megakaryoblasts than those in non-DS myeloblasts.
  • These results suggest that ETS2 expression is linked to the biology of AMkL in both DS and non-DS children, and that ETS2 acts by regulating expression of hematopoietic lineage and transcription factor genes involved in erythropoiesis and megakaryopoiesis, and in chemotherapy sensitivities.

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  • (PMID = 18094719.001).
  • [ISSN] 1476-5551
  • [Journal-full-title] Leukemia
  • [ISO-abbreviation] Leukemia
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA092308; United States / NCI NIH HHS / CA / R01 CA92308; United States / NIEHS NIH HHS / ES / P30 ES06639; United States / NIEHS NIH HHS / ES / P30 ES006639; United States / NCI NIH HHS / CA / T32 CA009531; United States / NCI NIH HHS / CA / R01 CA120772
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / ETS2 protein, human; 0 / GATA1 Transcription Factor; 0 / GATA1 protein, human; 0 / Neoplasm Proteins; 0 / Proto-Oncogene Protein c-ets-2; 04079A1RDZ / Cytarabine; ZS7284E0ZP / Daunorubicin
  • [Other-IDs] NLM/ NIHMS505525; NLM/ PMC3809919
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25. 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
The Weizmann Institute of Science GeneCards and MalaCards databases. gene/protein/disease-specific - MalaCards for tetrasomy 21 .

  • [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.
  • 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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