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3. Reuss CS, Wilansky S: Images in cardiovascular medicine. Eosinophilic heart disease in acute myeloproliferative disorder. Circulation; 2007 Jun 12;115(23):e614-6
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  • [Title] Images in cardiovascular medicine. Eosinophilic heart disease in acute myeloproliferative disorder.
  • [MeSH-major] Eosinophilia / complications. Heart Diseases / etiology. Myeloproliferative Disorders / complications


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4. Saint-Martin C, Leroy G, Delhommeau F, Panelatti G, Dupont S, James C, Plo I, Bordessoule D, Chomienne C, Delannoy A, Devidas A, Gardembas-Pain M, Isnard F, Plumelle Y, Bernard O, Vainchenker W, Najman A, Bellanné-Chantelot C, French Group of Familial Myeloproliferative Disorders: Analysis of the ten-eleven translocation 2 (TET2) gene in familial myeloproliferative neoplasms. Blood; 2009 Aug 20;114(8):1628-32
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  • [Title] Analysis of the ten-eleven translocation 2 (TET2) gene in familial myeloproliferative neoplasms.
  • The JAK2(V617F) mutation does not elucidate the phenotypic variability observed in myeloproliferative neoplasm (MPN) families.
  • In a patient with 2 TET2 mutations, the analysis of 5 blood samples at different phases of her disease showed the sequential occurrence of JAK2(V617F) and TET2 mutations concomitantly to the disease evolution.
  • TET2 mutations were mainly observed (10 of 12) in patients with primary myelofibrosis or patients with polycythemia vera or essential thrombocythemia who secondarily evolved toward myelofibrosis or acute myeloid leukemia.
  • [MeSH-major] Bone Marrow Neoplasms / genetics. DNA-Binding Proteins / genetics. Myeloproliferative Disorders / genetics. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adult. Aged. Cells, Cultured. DNA Mutational Analysis. Family. Female. Genetic Predisposition to Disease. Humans. Male. Middle Aged. Pedigree. Phenotype


5. Nussenzveig RH, Cortes J, Sever M, Quintás-Cardama A, Ault P, Manshouri T, Bueso-Ramos C, Prchal JT, Kantarjian H, Verstovsek S: Imatinib mesylate therapy for polycythemia vera: final result of a phase II study initiated in 2001. Int J Hematol; 2009 Jul;90(1):58-63
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  • Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN) characterized by excessive production of red blood cells.
  • Patients with PV are at a risk of thrombosis, bleeding, and transformation to myelofibrosis or acute myeloid leukemia.
  • We conducted an open-label phase II clinical trial of imatinib at the standard dose of 400 mg daily in 24 patients with PV.
  • Our data indicate that imatinib has minimal clinical activity in PV.

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  • (PMID = 19484334.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA016672
  • [Publication-type] Clinical Trial, Phase II; Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Benzamides; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptors, Platelet-Derived Growth Factor; EC 2.7.10.2 / JAK2 protein, human; EC 2.7.10.2 / Janus Kinase 2; EC 2.7.10.2 / Proto-Oncogene Proteins c-abl
  • [Other-IDs] NLM/ NIHMS672549; NLM/ PMC4378576
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6. Kiladjian JJ, Rain JD, Bernard JF, Briere J, Chomienne C, Fenaux P: Long-term incidence of hematological evolution in three French prospective studies of hydroxyurea and pipobroman in polycythemia vera and essential thrombocythemia. Semin Thromb Hemost; 2006 Jun;32(4 Pt 2):417-21
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  • Despite recent discoveries made in myeloproliferative disorders other than chronic myelogenous leukemia, which it is hoped will result in earlier diagnosis, and better evaluation and management of patients, hematological evolution to myelofibrosis, acute leukemia, and myelodysplastic syndromes (AL/MDS) remain major causes of long-term mortality in polycythemia vera (PV) and essential thrombocythemia (ET) patients.
  • We report updated results of three French prospective trials of hydroxyurea and pipobroman in PV and ET patients with a median follow-up longer than 10 years.
  • Although hydroxyurea currently remains the first choice in the treatment of high-risk PV and ET patients, the use of nonleukemogenic drugs, such as interferon alpha (IFN-alpha) or anagrelide, should be assessed more widely in randomized trials using accurate diagnostic criteria and taking into account the presence of the JAK2 mutation, given that they may have an impact on disease evolution.

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  • (PMID = 16810617.001).
  • [ISSN] 0094-6176
  • [Journal-full-title] Seminars in thrombosis and hemostasis
  • [ISO-abbreviation] Semin. Thromb. Hemost.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Interferon-alpha; 0 / Proto-Oncogene Proteins; 6Q99RDT97R / Pipobroman; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.2 / JAK2 protein, human; EC 2.7.10.2 / Janus Kinase 2; X6Q56QN5QC / Hydroxyurea
  • [Number-of-references] 42
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7. Naoe T: Developing target therapy against oncogenic tyrosine kinase in myeloid maliganacies. Curr Pharm Biotechnol; 2006 Oct;7(5):331-7
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  • Fusion genes involving ABL, ARG, PDGFRs, JAK2, SYK, TRKC, and FGFRs, and gain-of-function mutations of FLT3, KIT and JAK2 have been detected at various rates in myeloproliferative disease and acute myeloid leukemia.
  • Since the fusion or mutation of tyrosine kinase is a primary and central event in chronic myeloproliferative diseases, targeting the kinase activity has been thought to be an ideal intervention to treat these diseases.
  • The clinical success of imatinib for chronic myeloid leukemia has made this idea a reality, and has accelerated the development of new tyrosine kinase inhibitors (TKIs).
  • Challenging studies with TKIs have also been reported for acute myeloid leukemia.

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  • (PMID = 17076649.001).
  • [ISSN] 1873-4316
  • [Journal-full-title] Current pharmaceutical biotechnology
  • [ISO-abbreviation] Curr Pharm Biotechnol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Neoplasm Proteins; 0 / Protein Kinase Inhibitors; EC 2.7.10.1 / Protein-Tyrosine Kinases
  • [Number-of-references] 111
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8. Petrides PE: [Primary thrombocythemia: diagnosis and therapy]. Med Klin (Munich); 2006 Aug 15;101(8):624-34
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  • [Title] [Primary thrombocythemia: diagnosis and therapy].
  • BACKGROUND: Primary thrombocythemia is a rare acquired chronic disorder of the bone marrow which can occur at any age.
  • Diagnosis is based upon elevated platelet counts, morphologically and functionally altered platelets and characteristic bone marrow alterations as well as the exclusion of related myeloproliferative disorders which can also be accompanied by increased platelet counts.
  • Possible disease complications are thromboembolic and hemorrhagic events as well as a transformation into myelofibrosis or acute leukemia.
  • Inhibition of platelet aggregation with aspirin for the prevention of thromboembolic complications is first-line therapy; cytoreductive therapy with drugs such as hydroxyurea or interferon-alpha or thromboreductive therapy with the platelet-reducing agent anagrelide is required when thromboembolic complications are already present at diagnosis (secondary prevention) or when platelet counts are steadily increasing or various additional risk factors are present (primary prevention).
  • In up to 50% of the patients the recently discovered V617F mutation in the JAK2 gene can be identified which is supposed to be involved in the pathogenesis of this disease.
  • CLINICAL STUDIES: Because of the rarity of primary thrombocythemia thus far only two prospectively randomized studies have been carried out to compare cyto- and thromboreductive therapies.
  • CONCLUSION: Due to the existence of randomized clinical studies expert opinion will, in the future, be increasingly replaced by evidence-based therapy guidelines.
  • The improved knowledge of the molecular basis of the disease because of the discovery of the V617F mutation in the JAK2 gene has improved the molecular diagnosis and opened new avenues to molecular-targeted therapies.
  • [MeSH-minor] Adult. Aged. Aspirin / administration & dosage. Aspirin / therapeutic use. Child. Diagnosis, Differential. Drug Therapy, Combination. Enzyme Inhibitors / administration & dosage. Enzyme Inhibitors / therapeutic use. Female. Follow-Up Studies. Humans. Hydroxyurea / administration & dosage. Hydroxyurea / therapeutic use. Immunologic Factors / administration & dosage. Immunologic Factors / therapeutic use. Interferon-alpha / administration & dosage. Interferon-alpha / therapeutic use. Male. Mutation. Platelet Aggregation Inhibitors / administration & dosage. Platelet Aggregation Inhibitors / therapeutic use. Platelet Count. Pregnancy. Pregnancy Complications, Hematologic. Primary Prevention. Prospective Studies. Quinazolines / administration & dosage. Quinazolines / therapeutic use. Randomized Controlled Trials as Topic. Risk Assessment. Risk Factors. Time Factors

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  • (PMID = 16896569.001).
  • [ISSN] 0723-5003
  • [Journal-full-title] Medizinische Klinik (Munich, Germany : 1983)
  • [ISO-abbreviation] Med. Klin. (Munich)
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 0 / Immunologic Factors; 0 / Interferon-alpha; 0 / Platelet Aggregation Inhibitors; 0 / Quinazolines; 0 / anagrelide; R16CO5Y76E / Aspirin; X6Q56QN5QC / Hydroxyurea
  • [Number-of-references] 45
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23. Vassilopoulos G, Palassopoulou M, Zisaki K, Befani M, Bouronikou E, Giannakoulas N, Stathopoulou E, Matsouka P: Successful control of acute myelofibrosis with lenalidomide. Case Rep Med; 2010;2010:421239
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  • [Title] Successful control of acute myelofibrosis with lenalidomide.
  • Acute panmyelosis with myelofibrosis (APMF) is a rare, fatal hematological neoplasm that is characterized by the acute onset of cytopenias and fibrosis in the bone marrow in the absence of splenomegaly or fibrosis-related morphological changes in the RBCs.
  • We present the case of a 59-year-old female who presented with a two-month history of anemia, leucopenia and a normal platelet count.
  • At 4 months after diagnosis, the patient was started on Lenalidomide, 10 mg/day for a 21-d-course along with growth factor support.
  • To our knowledge, this is the first case for a medication that could reverse the fatal outcome of APMF.

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  • [Cites] Blood. 2007 Aug 1;110(3):986-93 [17473062.001]
  • [Cites] Blood. 2002 Nov 15;100(10):3495-503 [12393681.001]
  • [Cites] Br Med J. 1963 Aug 24;2(5355):472-7 [14043710.001]
  • [Cites] N Engl J Med. 2005 Feb 10;352(6):549-57 [15703420.001]
  • [Cites] Mod Pathol. 2005 May;18(5):603-14 [15578075.001]
  • [Cites] Blood. 2006 Aug 15;108(4):1158-64 [16609064.001]
  • [Cites] Ann Hematol. 2004 Aug;83(8):513-21 [15173958.001]
  • (PMID = 21274282.001).
  • [ISSN] 1687-9635
  • [Journal-full-title] Case reports in medicine
  • [ISO-abbreviation] Case Rep Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3026984
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24. Hoffman R, Prchal JT, Samuelson S, Ciurea SO, Rondelli D: Philadelphia chromosome-negative myeloproliferative disorders: biology and treatment. Biol Blood Marrow Transplant; 2007 Jan;13(1 Suppl 1):64-72
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  • [Title] Philadelphia chromosome-negative myeloproliferative disorders: biology and treatment.
  • The Philadelphia chromosome (Ph)-negative myeloproliferative disorders (MPDs) include essential thrombocythemia (ET), idiopathic myelofibrosis (IMF), and polycythemia vera (PV).
  • Both mutations activate JAK-STAT signaling pathways and likely play a role in disease progression.
  • Both ET and PV are associated with prolonged clinical courses associated with frequent thrombotic and hemorrhagic events, and progression to myelofibrosis and acute leukemia.
  • Intermediate/high-risk IMF or myelofibrosis after ET or PV is associated with a sufficiently poor prognosis to justify the use of allogeneic stem cell transplantation, which is capable of curing such patients.
  • [MeSH-major] Janus Kinase 2 / genetics. Myeloproliferative Disorders / genetics. Myeloproliferative Disorders / therapy

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  • (PMID = 17222772.001).
  • [ISSN] 1083-8791
  • [Journal-full-title] Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation
  • [ISO-abbreviation] Biol. Blood Marrow Transplant.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P01 CA108671-01A2
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Review
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.2 / JAK2 protein, human; EC 2.7.10.2 / Janus Kinase 2
  • [Number-of-references] 68
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25. Ngirabacu MC, Ravoet C, Dargent JL, Meuleman N, Ahmad I, Ysebrant L, Bennani J, André M, Bron D: Long-term follow-up of autologous peripheral blood stem cell transplantation in the treatment of a patient with acute panmyelosis with myelofibrosis. Haematologica; 2006 Dec;91(12 Suppl):ECR53
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  • [Title] Long-term follow-up of autologous peripheral blood stem cell transplantation in the treatment of a patient with acute panmyelosis with myelofibrosis.
  • [MeSH-major] Leukemia, Myeloid / surgery. Peripheral Blood Stem Cell Transplantation. Primary Myelofibrosis / surgery
  • [MeSH-minor] Acute Disease. Anemia, Refractory / drug therapy. Anemia, Refractory / etiology. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Cytarabine / administration & dosage. Erythropoietin / therapeutic use. Etoposide / administration & dosage. Follow-Up Studies. Granulocyte Colony-Stimulating Factor / therapeutic use. Humans. Male. Methotrexate / administration & dosage. Middle Aged. Mitoxantrone / administration & dosage. Postoperative Complications / drug therapy. Postoperative Complications / etiology. Recombinant Proteins. Transplantation Conditioning / adverse effects. Transplantation, Autologous. Whole-Body Irradiation / adverse effects

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  • (PMID = 17194659.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Recombinant Proteins; 04079A1RDZ / Cytarabine; 11096-26-7 / Erythropoietin; 143011-72-7 / Granulocyte Colony-Stimulating Factor; 6PLQ3CP4P3 / Etoposide; BZ114NVM5P / Mitoxantrone; YL5FZ2Y5U1 / Methotrexate
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26. Takahashi N, Lee Y, Tsai DY, Ishii K, Kamio S: [Improvement of detection of early CT signs in hyperacute stroke using a novel noise reduction filter]. Nihon Hoshasen Gijutsu Gakkai Zasshi; 2008 Jul 20;64(7):881-2
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  • PURPOSE: In the diagnosis of hyperacute stroke, an early CT sign such as the loss of gray-white matter interface may be difficult to detect within the first hours of the onset of symptoms because of the presence of quantum noise on CT images.
  • METHODS AND MATERIALS: Our method provides an adaptive partial median filter (APMF), which can reduce local noise without blurring of anatomical structure using variable filter shape and size according to the pixel value distribution of object around a center pixel.
  • The APMF can enhance the loss of gray-white matter interface due to hyperacute stroke.
  • The CT images of 26 patients with acute (<5 hours) middle cerebral artery territory infarction were proved with follow-up CT.
  • The APMF was applied to all the CT images.
  • Four radiologists, without and with applying the APMF, indicated their confidence level regarding the presence (or absence) of the early CT signs on each CT images.
  • RESULTS: A 78% noise reduction with the APMF was obtained from simulation.
  • The average area under the ROC curve (Az) was improved from 0.868 to 0.924 for all radiologists by applying the APMF to the original images.
  • The difference in Az values with and without the APMF was statistically significant with a P value of .002 for all radiologists.
  • CONCLUSION: Our proposed APMF can improve the visibility of gray-white matter interface.
  • As a result, the APMF can help radiologists detect the early CT signs at emergency CT scan.
  • [MeSH-minor] Acute Disease. Chicago. Congresses as Topic. Humans. ROC Curve. Radiology. Societies, Medical

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  • (PMID = 18719308.001).
  • [ISSN] 0369-4305
  • [Journal-full-title] Nihon Hōshasen Gijutsu Gakkai zasshi
  • [ISO-abbreviation] Nihon Hoshasen Gijutsu Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
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27. Barbui T, Finazzi G: Evidence-based management of polycythemia vera. Best Pract Res Clin Haematol; 2006;19(3):483-93
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  • The clinical course of polycythemia vera is marked by significant thrombotic complications and a variable risk of the disease turning either into myeloid metaplasia with myelofibrosis or into acute myeloid leukemia.
  • However, there is concern that certain myelosuppressive drugs accelerate the disease progression to acute leukemia.
  • This chapter provides updated estimates of the risk of thrombosis and disease progression and evaluates the various randomized and observational studies in polycythemia vera, according to an evidence-based approach.

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  • (PMID = 16781485.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interferon-alpha; 0 / Platelet Aggregation Inhibitors; X6Q56QN5QC / Hydroxyurea
  • [Number-of-references] 27
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28. Orazi A, O'Malley DP, Jiang J, Vance GH, Thomas J, Czader M, Fang W, An C, Banks PM: Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia. Mod Pathol; 2005 May;18(5):603-14
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  • [Title] Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia.
  • The WHO criteria for diagnosing acute panmyelosis with myelofibrosis are somewhat distinct from those for acute megakaryoblastic leukemia.
  • However, clinical and hematopathologic findings partially overlap.
  • To determine the potential importance of bone marrow biopsy supplemented by immunohistochemistry in distinguishing between these two conditions, we studied 17 bone marrow biopsies of well-characterized cases of acute panmyelosis with myelofibrosis (six cases) and acute megakaryoblastic leukemia (11 cases).
  • Acute panmyelosis with myelofibrosis is characterized by a multilineage myeloid proliferation with a less numerous population of blasts than acute megakaryoblastic leukemia (P<0.01).
  • In the former condition, blasts are always positive with CD34, while in acute megakaryoblastic leukemia they express CD34 in 60% of the cases.
  • The blasts in acute panmyelosis with myelofibrosis only rarely express megakaryocytic antigens.
  • By contrast, acute megakaryoblastic leukemia has a significantly higher proportion of blasts expressing megakaryocytic antigens (P<0.01 with CD42b).
  • [MeSH-major] Bone Marrow Cells / pathology. Leukemia, Megakaryoblastic, Acute / pathology. Primary Myelofibrosis / pathology
  • [MeSH-minor] Acute Disease. Adolescent. Adult. Aged. Aged, 80 and over. Antigens, CD / analysis. Child. Child, Preschool. Chromosome Aberrations. Diagnosis, Differential. Female. Flow Cytometry. Humans. Karyotyping. Male. Middle Aged

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  • (PMID = 15578075.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD
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29. Rain JD: [Polycythemia vera]. Rev Prat; 2005 Oct 15;55(15):1659-68
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Polycythemia vera (PV) is a chronic myeloproliferative disorder due to a haematopoietic stem cell's clonal proliferation.
  • This acquired disorder is often associated with thrombocytosis, leukocytosis and splenomegaly.
  • Generally, diagnosis remains easy, based on basic clinical and biological abnormalities.
  • Sometimes, positive diagnosis required more sophisticated tests as assay of endogenous erythroid colony, erythropoietin blood level and bone marrow biopsy.
  • Usually natural history of disease remains long with a good quality of life.
  • In some cases complications occur: mainly thrombosis and late myeloid metaplasia with myelofibrosis and acute leukemia.
  • Therapeutic approachs remain complex and difficult to optimize based up on age and disease severity.
  • [MeSH-major] Polycythemia Vera / diagnosis. Polycythemia Vera / drug therapy
  • [MeSH-minor] Biopsy. Bone Marrow / pathology. Diagnosis, Differential. Erythroid Cells. Erythropoietin / blood. Humans. Leukemia / chemically induced. Leukemia / prevention & control. Quality of Life. Severity of Illness Index

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  • (PMID = 16334202.001).
  • [ISSN] 0035-2640
  • [Journal-full-title] La Revue du praticien
  • [ISO-abbreviation] Rev Prat
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 11096-26-7 / Erythropoietin
  • [Number-of-references] 16
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30. Avcı Z, Malbora B, Gülşan M, Şahin FI, Celasun B, Özbek N: Acute massive myelofibrosis with acute lymphoblastic leukemia. Turk J Haematol; 2009 Dec 5;26(4):204-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Acute massive myelofibrosis with acute lymphoblastic leukemia.
  • Acute myelofibrosis is characterized by pancytopenia of sudden onset, megakaryocytic hyperplasia, extensive bone marrow fibrosis, and the absence of organomegaly.
  • Acute myelofibrosis in patients with acute lymphoblastic leukemia is extremely rare.
  • We report a 4-year-old boy who was diagnosed as having acute massive myelofibrosis and acute lymphoblastic leukemia.
  • Performing bone marrow aspiration in this patient was difficult (a "dry tap"), and the diagnosis was established by means of a bone marrow biopsy and immunohistopathologic analysis.
  • The prognostic significance of acute myelofibrosis in patients with acute lymphoblastic leukemia is not clear.

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  • (PMID = 27265634.001).
  • [ISSN] 1300-7777
  • [Journal-full-title] Turkish journal of haematology : official journal of Turkish Society of Haematology
  • [ISO-abbreviation] Turk J Haematol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Turkey
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31. Geron I, Abrahamsson AE, Barroga CF, Kavalerchik E, Gotlib J, Hood JD, Durocher J, Mak CC, Noronha G, Soll RM, Tefferi A, Kaushansky K, Jamieson CH: Selective inhibition of JAK2-driven erythroid differentiation of polycythemia vera progenitors. Cancer Cell; 2008 Apr;13(4):321-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Polycythemia Vera (PV) is a myeloproliferative disorder (MPD) that is commonly characterized by mutant JAK2 (JAK2V617F) signaling, erythrocyte overproduction, and a propensity for thrombosis, progression to myelofibrosis, or acute leukemia.
  • Thus, TG101348 may be an effective inhibitor of JAK2V617F+ MPDs in clinical trials.


32. Orazi A, Czader MB: Myelodysplastic syndromes. Am J Clin Pathol; 2009 Aug;132(2):290-305
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  • Submitted cases highlighted important issues and difficulties in relation to the diagnosis and classification of MDS.
  • Much of the discussion focused on the correlation, or lack of it, between morphologic examination and other diagnostic techniques, cytogenetics in particular.
  • The cases included examples of isolated del(5q) chromosomal abnormality, including the "classical" 5q- syndrome and other myeloid neoplasms.
  • Particularly challenging is the correct identification of fibrotic subtypes of MDSs and their separation from subsets of acute myeloid leukemia with myelofibrosis such as acute panmyelosis with myelofibrosis.
  • At least for the foreseeable future, the diagnosis of MDS requires integration of morphologic, immunophenotypic, and genetic features in the light of patient history and clinical manifestations.


33. Takahashi N, Lee Y, Tsai DY, Ishii K, Kinoshita T, Tamura H, Kimura M: Improvement of detection of hypoattenuation in acute ischemic stroke in unenhanced computed tomography using an adaptive smoothing filter. Acta Radiol; 2008 Sep;49(7):816-26
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  • [Title] Improvement of detection of hypoattenuation in acute ischemic stroke in unenhanced computed tomography using an adaptive smoothing filter.
  • PURPOSE: To evaluate the effect of a previously proposed adaptive smoothing filter for improving detection of parenchymal hypoattenuation of acute ischemic stroke on unenhanced CT images.
  • The adaptive partial median filter (APMF) designed for improving detectability of hypoattenuation areas on unenhanced CT images was applied.
  • Seven radiologists, including four certified radiologists and three radiology residents, indicated their confidence level regarding the presence (or absence) of hypoattenuation on CT images, first without and then with the APMF processed images.
  • Their performances without and with the APMF processed images were evaluated by receiver operating characteristic (ROC) analysis.
  • RESULTS: The mean areas under the ROC curves (AUC) for all observers increased from 0.875 to 0.929 (P = 0.002) when the radiologists observed with the APMF processed images.
  • The mean sensitivity in the detection of hypoattenuation significantly improved, from 69% (126 of 182 observations) to 89% (151 of 182 observations), when employing the APMF (P = 0.012).
  • The specificity, however, was unaffected by the APMF (P = 0.41).
  • CONCLUSION: The APMF has the potential to improve the detection of parenchymal hypoattenuation of acute ischemic stroke on unenhanced CT images.


34. Kawagoe H, Grosveld GC: Conditional MN1-TEL knock-in mice develop acute myeloid leukemia in conjunction with overexpression of HOXA9. Blood; 2005 Dec 15;106(13):4269-77
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  • [Title] Conditional MN1-TEL knock-in mice develop acute myeloid leukemia in conjunction with overexpression of HOXA9.
  • Coexpression of MN1-TEL and IL-3, but not SCF, rapidly caused a fatal myeloproliferative disease rather than acute myeloid leukemia (AML).
  • Thus, the leukemogenic effect of MN1-TEL in our knock-in mice is pleiotropic, and the type of secondary mutation determines disease outcome.

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  • (PMID = 16105979.001).
  • [ISSN] 0006-4971
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA217G; United States / NCI NIH HHS / CA / CA72999-07
  • [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 / Cytokines; 0 / ETS translocation variant 6 protein; 0 / Homeodomain Proteins; 0 / Mn1 protein, mouse; 0 / Oncogene Proteins; 0 / Proto-Oncogene Proteins c-ets; 0 / Proto-Oncogene Proteins c-myc; 0 / Repressor Proteins; 0 / homeobox protein HOXA9
  • [Other-IDs] NLM/ PMC1895240
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35. Jones CM, Dickinson TM, Salvado A: Phase II open label trial of imatinib in polycythemia rubra vera. Int J Hematol; 2008 Dec;88(5):489-94
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Polycythemia rubra vera is a chronic myeloproliferative disorder characterized by panmyelosis with the resultant potential for thrombosis, myelofibrosis, and acute leukemia.
  • Patients meeting the Polycythemia Vera Study group criteria for the diagnosis of polycythemia vera, either naïve or intolerant to prior treatment were allowed to enroll.

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  • (PMID = 19009241.001).
  • [ISSN] 1865-3774
  • [Journal-full-title] International journal of hematology
  • [ISO-abbreviation] Int. J. Hematol.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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36. Finazzi G, Barbui T: Expertise-based management in essential thrombocythemia and polycythemia vera. Cancer J; 2007 Nov-Dec;13(6):372-6
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  • The clinical courses of polycythemia vera (PV) and essential thrombocythemia (ET) are characterized by an increased incidence of thrombotic and hemorrhagic complications and an inherent tendency to progress into myelofibrosis or acute myeloid leukemia.
  • Interferon-alpha (IFN-alpha) or anagrelide could be considered in selected young patients or as second-line therapy in those intolerant of hydroxyurea or with refractory disease.






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