Essential thrombocythemia terminating in pure erythroleukemia

Abstract

Transformation into acute leukemia is a rare event in essential thrombocythemia (ET). The blasts are usually of myeloid, rarely of megakaryoblastic differentiation. We present the case of a patient with pure erythroleukemia after a nearly 10-year course of ET, which was treated with hydroxyurea. The patient, a 58-year-old male, presented with an elevated thrombocyte count (926,000/mL) and normal values of hemoglobin and leukocytes. After 10 years of therapy with hydroxyurea, the patient developed acute leukemia of solely erythroid differentiation. Chemotherapy with cytarabine and daunorubicin resulted in incomplete remission. The patient died 2 months after diagnosis of acute erythroleukemia. Transformation of ET into erythroleukemia may demonstrate the pluripotent potential of the neoplastic hemopoietic stem cell, with the ability to cause acute leukemia not only of myeloid or megakaryoblastic but also of erythroid lineage. Am.

The Incidence of Myelofibrosis in Essential Thrombocythaemia, Polycythaemia vera and Chronic Idiopathic Myelofibrosis: A Retrospective Evaluation of Sequential Bone Marrow Biopsies

“…Like Thiele et al [22]we found only one ET patient developing MF in our cohort. However, since this event was associated with a leukaemic transformation and the patient was the only one with MF in the group of ET patients, the fibre increase may not be a feature of ET but rather induced by the blasts [23]. …”

Acute biphenotypic leukemia arising in a patient with essential thrombocythemia

“…Transformation of ET to leukemia demonstrates the pluripotent potential of the neoplastic hemopoietic stem cell, with the ability to transform to leukemia not only of the myeloid but also lymphoid lineage [20]. In this case report, we demonstrate a case of transformation to acute biphenotypic leukemia (BAL).…”

“…BAL is believed to arise from a multipotent progenitor cell and carries a poor prognosis. The cytogenetic abnormality of del (20) and the need to use multiple agents to control thrombocytosis in our patient reflect a more aggressive disease. Hence, it is not surprising to see transformation to BAL.…”

“…It will be difficult to evaluate whether hydroxyurea is the main leukemogenic agent or, perhaps, an interplay of drugs potentiated another's leukemogenic potential. The requirement of multiple agents to control thrombocytosis and del (20) cytogenetic abnormality at presentation (usually seen in MDS/AML patients) reflect a biologically more aggressive disease that is inherently more likely to transform. The added abnormalities of t(8;21), der(9), t(1;9), add (14) in all 20 metaphases at transformation to acute biphenotypic leukemia could possibly be the result of a combination of an originally more aggressive ET disease and the subsequent use of multiple cytoreductive agents.…”

Wild-Type JAK2 Secondary Acute Erythroleukemia Developing after JAK2-V617F-Mutated Primary Myelofibrosis

“…Previously, we had reported another case of ET with subsequent progression to AE [5], which retrospectively turned out to harbor the wild type of JAK2 in the chronic phase of the disease at diagnosis as well as in AE.…”