“Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature

Karrati Ilham; Jahdaoui Adil; Ghita El Ghouat; Yahyaoui Hicham; Ait ameur Mustapha; Chakour Mohamed

doi:doi:10.11648/j.wjmcr.20210201.12

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“Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature

Karrati Ilham, Jahdaoui Adil, Ghita El Ghouat, Yahyaoui Hicham, Ait ameur Mustapha, Chakour Mohamed

Published in World Journal of Medical Case Reports (Volume 2, Issue 1)

Received: 16 January 2021 Accepted: 28 January 2021 Published: 9 February 2021

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Abstract

Polycythemia vera (PV), with essential thrombocythemia (ET) and primary myelofibrosis (PMF), belongs to the group of Philadelphia negative myeloproliferative neoplasms (MPN). PV is characterized in virtually all cases by the somatic JAK2 V617F mutation or another functionally similar JAK2 mutation that results in panmyelosis. Two phases of PV are recognized which include an initial polycythemic phase associated with elevated haemoglobin level, elevated haematocrit and increased red blood cells mass, and a later spent phase or post- polycythemic myelofibrosis phase, characterised by cytopenias including anaemia, ineffective haematopoiesis, bone marrow fibrosis, extramedullary haematopoiesis, and hypersplenism. The natural progression of PV includes a low incidence of evolution to a myelodysplastic or blast phase. Leukemic transformation in PV is described as a rare and late event, less common than primary myelofibrosis. The interval between diagnosis and leukemic evolution is highly variable, from a few years to >20 years; which implies a long-lasting exposure to myelosuppressive agents. Among the hematological transformations; evolution to secondary acute myeloid leukemia (AML) is associated with a poor prognosis; here we report a case of an 80 year old woman who progressed unusually to blast phase within two years of diagnosis of PV. The interest of this work lies in the fact that this transformation into AML occurred after a short period of evolution, which is not frequent enough in the literature.

Published in	World Journal of Medical Case Reports (Volume 2, Issue 1)
DOI	10.11648/j.wjmcr.20210201.12
Page(s)	4-6
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2021. Published by Science Publishing Group

Keywords

Polycythemia Vera, Acute Leukemia, Prognosis

References

[1]	Leukemic evolution of polycythemia vera and essential thrombocythemia: genomic profiles predict time to transformation LUQUE PAZ et al blood advances; 13 OCTOBER 2020 x VOLUME 4, NUMBER 19.
[2]	An unusual presentation of blast phase in JAK 2 mutated polycythemia vera L. Abraham and M. Paul Human Pathology: Case Reports 23 (2021) 200460.
[3]	Polycythemia vera and essential thrombocythemia: 2013 update on diagnosis, risk-stratification and management; Ayalew Tefferi, Tiziano Barbui American journal of Hematology. 2013 Jun; 88 (6): 507-16.
[4]	Critical review on polycythaemia vera; Ozokono P. C1; International Journal of Basic, Applied and Innovative Research, IJBAIR, 2019, 8 (2): 38–46.
[5]	Godon A, Dib M, Geneviève F, Ifrah N, Zandecki M. Découverte de blastes sanguins au décours de la polyglobulie de Vaquez. Ann Biol Clin. 2002; 60: 701-706.
[6]	Leukemic Transformation of Polycythemia Vera: A Single Center Study of 23 Patients Francesco Passamonti et al. 2005 American Cancer Society.
[7]	Management of polycythemia vera and essential thrombo¬cythemia J. J. Kiladjian; Correspondances en Onco-hématologie - Vol. III - n° 3 - juillet-août-septembre 2008.
[8]	New Perspectives on Polycythemia Vera: From Diagnosis to Therapy Alessandra Iurlo; International journal of molecular sciences. 2020 Aug 13; 21 (16): 5805.
[9]	G. Finazzi, V. Caruso, R. Marchioli, et al., Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study, Blood 105 (2005) 2664–2670.
[10]	Tefferi A, Solberg LA, Silverstein MN. A clinical update in polycythemia vera and essential thrombocythemia. Am J Med 2000, 109: 141-149.
[11]	Means RT. Polycythemia Vera. In: Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Wintrobe’s Clinical Hematology, 10th edition.Volume 2. Baltimore: Williams et Wilkins, 1999: 2374-2389.
[12]	Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia; Fransesco Passamonti et al, The American Journal of Medicine November 2004, 17 (10): 755-61.
[13]	The Impact of Clinicopathological Studies on Staging and Survival in Essential Thrombocythemia, Chronic Idiopathic Myelofibrosis, and Polycythemia Rubra Vera Hans Michael Kvasnicka1 et al Thieme E-Journals, seminars of thrombosis and hemostasis 2006.
[14]	Austrian recommendations for the management of polycythemia vera Sonja Burgstaller et al; Wien Klin Wochenschr. 2018 Sep; 130 (17-18): 535-542.
[15]	Risk of leukemic transformation in PV and ET patients Christine Chomienne et al; Pathologie Biologie 52 (2004) 289–293.

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Karrati Ilham, Jahdaoui Adil, Ghita El Ghouat, Yahyaoui Hicham, Ait ameur Mustapha, et al. (2021). “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature. World Journal of Medical Case Reports, 2(1), 4-6. https://doi.org/10.11648/j.wjmcr.20210201.12

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ACS Style

Karrati Ilham; Jahdaoui Adil; Ghita El Ghouat; Yahyaoui Hicham; Ait ameur Mustapha, et al. “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature. World J. Med. Case Rep. 2021, 2(1), 4-6. doi: 10.11648/j.wjmcr.20210201.12

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AMA Style

Karrati Ilham, Jahdaoui Adil, Ghita El Ghouat, Yahyaoui Hicham, Ait ameur Mustapha, et al. “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature. World J Med Case Rep. 2021;2(1):4-6. doi: 10.11648/j.wjmcr.20210201.12

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@article{10.11648/j.wjmcr.20210201.12,
  author = {Karrati Ilham and Jahdaoui Adil and Ghita El Ghouat and Yahyaoui Hicham and Ait ameur Mustapha and Chakour Mohamed},
  title = {“Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature},
  journal = {World Journal of Medical Case Reports},
  volume = {2},
  number = {1},
  pages = {4-6},
  doi = {10.11648/j.wjmcr.20210201.12},
  url = {https://doi.org/10.11648/j.wjmcr.20210201.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.wjmcr.20210201.12},
  abstract = {Polycythemia vera (PV), with essential thrombocythemia (ET) and primary myelofibrosis (PMF), belongs to the group of Philadelphia negative myeloproliferative neoplasms (MPN). PV is characterized in virtually all cases by the somatic JAK2 V617F mutation or another functionally similar JAK2 mutation that results in panmyelosis. Two phases of PV are recognized which include an initial polycythemic phase associated with elevated haemoglobin level, elevated haematocrit and increased red blood cells mass, and a later spent phase or post- polycythemic myelofibrosis phase, characterised by cytopenias including anaemia, ineffective haematopoiesis, bone marrow fibrosis, extramedullary haematopoiesis, and hypersplenism. The natural progression of PV includes a low incidence of evolution to a myelodysplastic or blast phase. Leukemic transformation in PV is described as a rare and late event, less common than primary myelofibrosis. The interval between diagnosis and leukemic evolution is highly variable, from a few years to >20 years; which implies a long-lasting exposure to myelosuppressive agents. Among the hematological transformations; evolution to secondary acute myeloid leukemia (AML) is associated with a poor prognosis; here we report a case of an 80 year old woman who progressed unusually to blast phase within two years of diagnosis of PV. The interest of this work lies in the fact that this transformation into AML occurred after a short period of evolution, which is not frequent enough in the literature.},
 year = {2021}
}

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TY  - JOUR
T1  - “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature
AU  - Karrati Ilham
AU  - Jahdaoui Adil
AU  - Ghita El Ghouat
AU  - Yahyaoui Hicham
AU  - Ait ameur Mustapha
AU  - Chakour Mohamed
Y1  - 2021/02/09
PY  - 2021
N1  - https://doi.org/10.11648/j.wjmcr.20210201.12
DO  - 10.11648/j.wjmcr.20210201.12
T2  - World Journal of Medical Case Reports
JF  - World Journal of Medical Case Reports
JO  - World Journal of Medical Case Reports
SP  - 4
EP  - 6
PB  - Science Publishing Group
SN  - 2994-726X
UR  - https://doi.org/10.11648/j.wjmcr.20210201.12
AB  - Polycythemia vera (PV), with essential thrombocythemia (ET) and primary myelofibrosis (PMF), belongs to the group of Philadelphia negative myeloproliferative neoplasms (MPN). PV is characterized in virtually all cases by the somatic JAK2 V617F mutation or another functionally similar JAK2 mutation that results in panmyelosis. Two phases of PV are recognized which include an initial polycythemic phase associated with elevated haemoglobin level, elevated haematocrit and increased red blood cells mass, and a later spent phase or post- polycythemic myelofibrosis phase, characterised by cytopenias including anaemia, ineffective haematopoiesis, bone marrow fibrosis, extramedullary haematopoiesis, and hypersplenism. The natural progression of PV includes a low incidence of evolution to a myelodysplastic or blast phase. Leukemic transformation in PV is described as a rare and late event, less common than primary myelofibrosis. The interval between diagnosis and leukemic evolution is highly variable, from a few years to >20 years; which implies a long-lasting exposure to myelosuppressive agents. Among the hematological transformations; evolution to secondary acute myeloid leukemia (AML) is associated with a poor prognosis; here we report a case of an 80 year old woman who progressed unusually to blast phase within two years of diagnosis of PV. The interest of this work lies in the fact that this transformation into AML occurred after a short period of evolution, which is not frequent enough in the literature.
VL  - 2
IS  - 1
ER  -

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Author Information

Karrati Ilham

Hematology Laboratory, Avicenna Military Hospital, Marrakesh, Morocco
Jahdaoui Adil

Hematology Laboratory, Avicenna Military Hospital, Marrakesh, Morocco
Ghita El Ghouat

Hematology Laboratory, Avicenna Military Hospital, Marrakesh, Morocco
Yahyaoui Hicham

Hematology Laboratory, Avicenna Military Hospital, Marrakesh, Morocco
Ait ameur Mustapha

Hematology Laboratory, Avicenna Military Hospital, Marrakesh, Morocco
Chakour Mohamed

Hematology Laboratory, Avicenna Military Hospital, Marrakesh, Morocco

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Plain Text BibTeX RIS

APA Style

Karrati Ilham, Jahdaoui Adil, Ghita El Ghouat, Yahyaoui Hicham, Ait ameur Mustapha, et al. (2021). “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature. World Journal of Medical Case Reports, 2(1), 4-6. https://doi.org/10.11648/j.wjmcr.20210201.12

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ACS Style

Karrati Ilham; Jahdaoui Adil; Ghita El Ghouat; Yahyaoui Hicham; Ait ameur Mustapha, et al. “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature. World J. Med. Case Rep. 2021, 2(1), 4-6. doi: 10.11648/j.wjmcr.20210201.12

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AMA Style

Karrati Ilham, Jahdaoui Adil, Ghita El Ghouat, Yahyaoui Hicham, Ait ameur Mustapha, et al. “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature. World J Med Case Rep. 2021;2(1):4-6. doi: 10.11648/j.wjmcr.20210201.12

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@article{10.11648/j.wjmcr.20210201.12,
  author = {Karrati Ilham and Jahdaoui Adil and Ghita El Ghouat and Yahyaoui Hicham and Ait ameur Mustapha and Chakour Mohamed},
  title = {“Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature},
  journal = {World Journal of Medical Case Reports},
  volume = {2},
  number = {1},
  pages = {4-6},
  doi = {10.11648/j.wjmcr.20210201.12},
  url = {https://doi.org/10.11648/j.wjmcr.20210201.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.wjmcr.20210201.12},
  abstract = {Polycythemia vera (PV), with essential thrombocythemia (ET) and primary myelofibrosis (PMF), belongs to the group of Philadelphia negative myeloproliferative neoplasms (MPN). PV is characterized in virtually all cases by the somatic JAK2 V617F mutation or another functionally similar JAK2 mutation that results in panmyelosis. Two phases of PV are recognized which include an initial polycythemic phase associated with elevated haemoglobin level, elevated haematocrit and increased red blood cells mass, and a later spent phase or post- polycythemic myelofibrosis phase, characterised by cytopenias including anaemia, ineffective haematopoiesis, bone marrow fibrosis, extramedullary haematopoiesis, and hypersplenism. The natural progression of PV includes a low incidence of evolution to a myelodysplastic or blast phase. Leukemic transformation in PV is described as a rare and late event, less common than primary myelofibrosis. The interval between diagnosis and leukemic evolution is highly variable, from a few years to >20 years; which implies a long-lasting exposure to myelosuppressive agents. Among the hematological transformations; evolution to secondary acute myeloid leukemia (AML) is associated with a poor prognosis; here we report a case of an 80 year old woman who progressed unusually to blast phase within two years of diagnosis of PV. The interest of this work lies in the fact that this transformation into AML occurred after a short period of evolution, which is not frequent enough in the literature.},
 year = {2021}
}

Copy | Download

TY  - JOUR
T1  - “Transformation of Polycythemia Vera into Acute Myeloid Leukemia”: A Case Report with Review of the Literature
AU  - Karrati Ilham
AU  - Jahdaoui Adil
AU  - Ghita El Ghouat
AU  - Yahyaoui Hicham
AU  - Ait ameur Mustapha
AU  - Chakour Mohamed
Y1  - 2021/02/09
PY  - 2021
N1  - https://doi.org/10.11648/j.wjmcr.20210201.12
DO  - 10.11648/j.wjmcr.20210201.12
T2  - World Journal of Medical Case Reports
JF  - World Journal of Medical Case Reports
JO  - World Journal of Medical Case Reports
SP  - 4
EP  - 6
PB  - Science Publishing Group
SN  - 2994-726X
UR  - https://doi.org/10.11648/j.wjmcr.20210201.12
AB  - Polycythemia vera (PV), with essential thrombocythemia (ET) and primary myelofibrosis (PMF), belongs to the group of Philadelphia negative myeloproliferative neoplasms (MPN). PV is characterized in virtually all cases by the somatic JAK2 V617F mutation or another functionally similar JAK2 mutation that results in panmyelosis. Two phases of PV are recognized which include an initial polycythemic phase associated with elevated haemoglobin level, elevated haematocrit and increased red blood cells mass, and a later spent phase or post- polycythemic myelofibrosis phase, characterised by cytopenias including anaemia, ineffective haematopoiesis, bone marrow fibrosis, extramedullary haematopoiesis, and hypersplenism. The natural progression of PV includes a low incidence of evolution to a myelodysplastic or blast phase. Leukemic transformation in PV is described as a rare and late event, less common than primary myelofibrosis. The interval between diagnosis and leukemic evolution is highly variable, from a few years to >20 years; which implies a long-lasting exposure to myelosuppressive agents. Among the hematological transformations; evolution to secondary acute myeloid leukemia (AML) is associated with a poor prognosis; here we report a case of an 80 year old woman who progressed unusually to blast phase within two years of diagnosis of PV. The interest of this work lies in the fact that this transformation into AML occurred after a short period of evolution, which is not frequent enough in the literature.
VL  - 2
IS  - 1
ER  -

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