Concomitant myeloproliferative and lymphoproliferative neoplasms, distinct progenitors: A case report and review of the literature

Abstract

Patients with a Philadelphia chromosome-negative myeloproliferative neoplasm may develop a lymphoproliferative disorder; however, the clinical and molecular determinants and the chronological onset of the two events remain unknown. We herein report the case of a 64-year-old man with concomitant diagnosis of high-risk essential thrombocythemia with evidence of a thrombotic event and high-count monoclonal B-cell lymphocytosis (high-count MBL). The patient harbored a JAK2V617F mutation and one of the most common genetic alterations found in chronic lymphocytic leukemia (CLL) (del 13q), which may represent a sign of disease progression. He was initiated on cytoreductive therapy with hydroxyurea 500 mg 3 times per week and hypocoagulation treatment, and is currently under regular surveillance of MBL without CLL criteria.

Non-receptor Tyrosine Kinases Role and Significance in Hematological Malignancies

“…Some reports have also suggested JAK2 V617F clonal involvement of B [143,144], T [143], and NK lymphocytes [83], also confirming the stem cell nature of JAK2 V617F MPNs [102]. Lower frequencies of V617F mutation occur in PN-CML, chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, and rare cases of AML (megakaryocytic and in combination with other well-defined genetic abnormalities, such as BCR-ABL1) [145].…”