Treatment options for hydroxyurea‐refractory disease complications in myeloproliferative neoplasms: JAK2 inhibitors, radiotherapy, splenectomy and transjugular intrahepatic portosystemic shunt
Open Access
- 12 August 2010
- journal article
- review article
- Published by Wiley in European Journal of Haematology
- Vol. 85 (3), 192-199
- https://doi.org/10.1111/j.1600-0609.2010.01480.x
Abstract
Clinical care of patients with polycythemia vera, essential thrombocythemia and myelofibrosis (MF) requires not only a broad understanding of general treatment principles but also familiarity with the management of hydroxyurea‐refractory disease complications. The latter include progressive splenomegaly, symptomatic portal hypertension (e.g. ascites, variceal bleeding), pulmonary hypertension, bone pain, intractable pruritus, constitutional symptoms (e.g. fatigue, night sweats) and cachexia (i.e. loss of lean body mass, general ill health, poor appetite). Some of these symptoms are directly or indirectly related to extramedullary hematopoiesis (EMH) and others to proinflammatory cytokine excess. Results from recent clinical trials of JAK inhibitors suggest remarkable activity in MF‐associated constitutional symptoms, cachexia, pruritus and hydroxyurea‐refractory splenomegaly. Involved‐field radiotherapy is best utilized in the setting of EMH‐associated symptoms, including ascites, bone (extremity) pain and pulmonary hypertension. Splenectomy is indicated in the presence of drug‐refractory splenomegaly and frequent red cell transfusion requirement. Transjugular intrahepatic portosystemic shunt is used to alleviate symptoms of portal hypertension.Keywords
This publication has 63 references indexed in Scilit:
- Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1Leukemia, 2010
- Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shuntBMC Gastroenterology, 2010
- Mature Survival Data for 176 Patients Younger Than 60 Years With Primary Myelofibrosis Diagnosed Between 1976 and 2005: Evidence for Survival Gains in Recent YearsMayo Clinic Proceedings, 2009
- The JAK2 Inhibitor AZD1480 Potently Blocks Stat3 Signaling and Oncogenesis in Solid TumorsCancer Cell, 2009
- Pegylated Interferon Alfa-2a Yields High Rates of Hematologic and Molecular Response in Patients With Advanced Essential Thrombocythemia and Polycythemia VeraJournal of Clinical Oncology, 2009
- Evidence and expertise in the management of polycythemia vera and essential thrombocythemiaLeukemia, 2008
- Hydroxyurea Compared with Anagrelide in High-Risk Essential ThrombocythemiaNew England Journal of Medicine, 2005
- A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia veraNature, 2005
- Nonhepatosplenic Extramedullary Hematopoiesis: Associated Diseases, Pathology, Clinical Course, and TreatmentMayo Clinic Proceedings, 2003
- Splenic artery embolization prior to splenectomy in end-stage polycythemia veraAmerican Journal of Hematology, 1980