Abstract
Introduction
Polycythemia vera (PV) is one of the most common forms of myeloproliferative neoplasms. Acute myeloid leukemia secondary to PV is well reported, and the mechanism has been clarified to some extent. Only a limited number of cases have been reported about the development of acute lymphoblastic leukemia (ALL) in the course of PV, and the possible underlying mechanism has not been explored well.
Case presentation
A 75-year-old patient who developed ALL 3 years after he was diagnosed with PV. The presence of remarkable splenomegaly, typical immunophenotyping of the peripheral blood and increased expression of serum fibrosis markers indicated the existence of extramedullary hematopoiesis which may ascribe to myelofibrosis. After the treatment of dosage-modulated chemotherapy, the patient got complete remission.
Conclusion
The JAK2 mutation may the underlying factor that contributes to the development of ALL, and the existence of MF may indicate the progression to post- polycythemic MF, which may be a risk factor for the accelerated transformation.
References
Anastasi J (2009) The myeloproliferative neoplasms: insights into molecular pathogenesis and changes in WHO classification and criteria for diagnosis. Hematol Oncol Clin N Am 23(4):693–708
Najean Y, Rain JD (1997) Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood 90(9):3370–3377
Rampal R, Mascarenhas J (2014) Pathogenesis and management of acute myeloid leukemia that has evolved from a myeloproliferative neoplasm. Curr Opin Hematol 21(2):65–71
Camós M, Cervantes F, Montoto S, Hernández-Boluda JC, Villamor N, Montserrat E (1999) Acute lymphoid leukemia following polycythemia vera. Leuk Lymphoma 32(3–4):395–398
Rege-Cambrin G, Mecucci C, Tricot G, Michaux JL, Louwagie A, Van Hove W, Francart H, Van den Berghe H (1987) A chromosomal profile of polycythemia vera. Cancer Genet Cytogenet 25(2):233–245
Roth AD, Oral A, Przepiorka D, Gollin SM, Chervenick PA (1993) Chronic myelogenous leukemia and acute lymphoblastic leukemia occurring in the course of polycythemia vera. Am J Hematol 43(2):123–128
Neilson JR, Patton WN, Williams MD, Mayne EE, Boughton BJ (1994) Polycythaemia rubra vera transforming to acute lymphoblastic leukaemia with a common immunophenotype. J Clin Pathol 47(5):471–472
Anastasi J, Pettenati MJ, Le Beau MM, Kwaan HC, Weil SC (1988) Acute lymphoblastic leukemia in a patient with longstanding polycythemia vera: cytogenetic analysis reveals two distinct abnormal clones. Am J Hematol 29(1):33–37
Tefferi A, Vardiman JW (2008) Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia 22(1):14–22
Lee HJ, Daver N, Kantarjian HM, Verstovsek S, Ravandi F (2013) The role of JAK pathway dysregulation in the pathogenesis and treatment of acute myeloid leukemia. Clin Cancer Res 19(2):327–335
Xu W, Chen B, Tong X (2014) Chronic myeloid leukemia patient with co-occurrence of BCR-ABL junction and JAK2 V617F mutation. Int J Hematol 99(1):87–90
Ohyashiki K, Aota Y, Akahane D, Gotoh A, Miyazawa K, Kimura Y, Ohyashiki JH (2005) The JAK2 V617F tyrosine kinase mutation in myelodysplastic syndromes (MDS) developing myelofibrosis indicates the myeloproliferative nature in a subset of MDS patients. Leukemia 19(12):2359–2360
Yang YN, Qin YW, Wang C (2014) JAK2 V617F detected in two B-cell chronic lymphocytic leukemia patients without coexisting Philadelphia chromosome-negative myeloproliferative neoplasms: a report of two cases. Oncol Lett. 8(2):841–844
dos Santos NR, Ghysdael J (2006) A transgenic mouse model for TEL-JAK2-induced B-cell lymphoma/leukemia. Leukemia 20(1):182–185
Papageorgiou MV, Alexopoulou A, Kontopidou F, Filiotou A, Koskinas J, Pectasides D (2011) Concomitant diagnosis of myeloproliferative neoplasm and non-Hodgkin’s lymphoma in a patient with portal vein thrombosis. Anticancer Res 31(4):1467–1469
Malhotra J, Kremyanskaya M, Schorr E, Hoffman R, Mascarenhas J (2014) Coexistence of myeloproliferative neoplasm and plasma-cell dyscrasia. Clin Lymphoma Myeloma Leuk 14(1):31–36
Elli EM, Belotti A, Cecchetti C, Realini S, Fedele M, Parma M, Pogliani EM (2013) Development of JAK2V617F-positive polycythemia vera after chemotherapy-induced remission of primary central nervous system diffuse large B cell non-Hodgkin’s lymphoma: a case report and review of the literature. Acta Haematol 130(3):142–145
Sutherland ND, Gonzalez-Peralta R, Douglas-Nikitin V, Hunger SP (2004) Polycythemia vera in a child following treatment for acute lymphoblastic leukemia. J Pediatr Hematol Oncol 26(5):315–319
Hann HW, Festa RS, Rosenstock JG, Cifuentes E (1979) Polycythemia vera in a child with acute lymphocytic leukemia. Cancer 43(5):1862–1865
Danish EH, Rasch CA, Harris JW (1980) Polycythemia vera in childhood: case report and review of the literature. Am J Hematol 9(4):421–428
Najfeld V, Cozza A, Berkofsy-Fessler W, Prchal J, Scalise A (2007) Numerical gain and structural rearrangements of JAK2, identified by FISH, characterize both JAK2617V > F-positive and -negative patients with Ph-negative MPD, myelodysplasia, and B-lymphoid neoplasms. Exp Hematol 35(11):1668–1676
Yu W, Chen L, Li J, Zhu H (2014) Essential thrombocythemia with JAK2 V617F mutation transformed into acute myeloid leukemia without JAK2 V617F mutation: case report and literature review. Zhonghua Xue Ye Xue Za Zhi. 35(12):1122–1123
Ohanian M, Leventaki V, Verstovsek S, Estrov Z, Lin P, Yin C, Kantarjian H, Huh Y, Ravandi F (2012) Acute lymphoblastic leukemia arising in post-polycythemic myelofibrosis: a rare entity. Leuk Lymphoma 53(9):1839–1841
Polliack A, Prokocimer M, Matzner Y (1980) Lymphoblastic leukemic transformation (lymphoblastic crisis) in myelofibrosis and myeloid metaplasia. Am J Hematol 9(2):211–220
Tefferi A, Lasho TL, Jimma T, Finke CM, Gangat N, Vaidya R, Begna KH, Al-Kali A, Ketterling RP, Hanson CA, Pardanani A (2012) One thousand patients with primary myelofibrosis: the mayo clinic experience. Mayo Clin Proc 87(1):25–33
Mesa RA, Li CY, Ketterling RP, Schroeder GS, Knudson RA, Tefferi A (2005) Leukemic transformation in myelofibrosis with myeloid metaplasia: a single-institution experience with 91 cases. Blood 105(3):973–977
Arrago JP, Poirier O, Chomienne C, D’Agay MF, Najean Y (1986) Type III aminoterminal propeptide of procollagen in some haematological malignancies. Scand J Haematol 36(3):288–294
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Wu, D., Ye, B., Shen, J. et al. Acute Lymphoblastic Leukemia in the Course of Polycythemia Vera: A Case Report and Review of Literature. Indian J Hematol Blood Transfus 32 (Suppl 1), 50–55 (2016). https://doi.org/10.1007/s12288-015-0598-y
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DOI: https://doi.org/10.1007/s12288-015-0598-y