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

• Author(s): Mousinho, Filipa ; Santos, Paula ; Azevedo, Ana ; Pereira, José ; Lemos, Raquel ; Matos, Sónia ; Viana, João ; Lima, Fernando
• Date: 2018
• Persistent ID: http://hdl.handle.net/10451/48325
• Origin: Repositório da Universidade de Lisboa
• Subject(s): Myeloproliferative neoplasms; Essential thrombocythemia; Lymphoproliferative neoplasms; Monoclonal B cell lymphocytosis; JAK2V617F mutation; del(13q)
• Document Type: Journal article
• Language: English
• Contributor(s): Repositório Científico de Acesso Aberto da ULisboa

Description

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.