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A Rare Case of Polycythemia Vera Following Acute Undifferentiated Leukemia Remission

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초록

Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) characterized by the abnormal proliferation of red blood cells, and it is associated with leukocytosis and thrombocytosis [1]. A Janus kinase 2 ( JAK2 ) V617F mutation is found in 95% of PV patients and influences its pathogenesis and diagnosis [1-4]. Generally, AML development is a late event in PV patients [1, 5], and leukemic transformation occurs in 5-15% of patients [4]. We report a case of PV after complete remission (CR) of acute undifferentiated leukemia.

Abstract

Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) characterized by the abnormal proliferation of red blood cells, and it is associated with leukocytosis and thrombocytosis [1]. A Janus kinase 2 ( JAK2 ) V617F mutation is found in 95% of PV patients and influences its pathogenesis and diagnosis [1-4]. Generally, AML development is a late event in PV patients [1, 5], and leukemic transformation occurs in 5-15% of patients [4]. We report a case of PV after complete remission (CR) of acute undifferentiated leukemia.

참고문헌 (10)

  1. Passamonti F, "A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications", Leukemia, 24, pp.1574~1579 (2010)
  2. Antonioli E, "Polycythemia vera following autologous transplantation for AML: insights on the kinetics of JAK2V617F clonal dominance", Blood, 110, pp.4620~4621 (2007)
  3. Tefferi A, "Polycythemia vera and essential thrombocythemia: 2013 update on diagnosis, risk-stratification, and management", Am J Hematol, 88, pp.507~516 (2013)
  4. Lee HJ, "The role of JAK pathway dysregulation in the pathogenesis and treatment of acute myeloid leukemia", Clin Cancer Res, 19, pp.327~335 (2013)
  5. Gangat N, "Leucocytosis in polycythaemia vera predicts both inferior survival and leukaemic transformation", Br J Haematol, 138, pp.354~358 (2007)
  6. Dong Wook Jekarl, "JAK2 V617F mutation in myelodysplastic syndrome, myelodysplastic syndrome", myeloproliferative neoplasm, unclassifiable, refractory anemia with ring sideroblasts with thrombocytosis, and acute myeloid leukemia, 2010, pp.45(1), 46~50[DOI]
  7. Swaminathan S, "Novel immunophenotypic and morphologic presentation in acute myeloid leukemia (AML) with JAK2 V617F mutation", Eur J Haematol, 84, pp.180~182 (2010)
  8. Belotti A, "Development of polycythemia vera after chemotherapy-induced remission of acute myeloid leukemia: a case report", Acta Haematol, 126, pp.52~53 (2011)
  9. Portell CA, "De novo polycythaemia vera arising 5 years following acute myeloid leukemia remission: suggestion of a chemotherapy resistant JAK2 clone", Br J Haematol, 157, pp.266~267 (2012)
  10. Chabannon C, "A case of polycythemia vera occurring in a patient with acute non-lymphoblastic leukemia (ANLL) in long-term first complete remission", Leukemia, 8, pp.1243~1244 (1994)

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