Successful treatment of severe extremity pain in myelofibrosis with low-dose single-fraction radiation therapy.
ABSTRACT Myelofibrosis (MF) is characterized by cytopenias/cytoses, leukoerythroblastic blood picture, bone marrow fibrosis, and extramedullary hematopoiesis. Extremity bone pain can be a feature of MF. There has been one case report of a patient with MF and bone pain treated with radiation. We treated five patients with MF and extremity pain with radiation at our institution between 2004 and 2010. All patients had severe pain unresponsive to narcotic medication. Radiation was one fraction of 100 to 600 cGy. Most patients saw improvement in the pain within days and achieved a complete response within days to weeks.The response lasted between 1 month and 2 years. Retreatment was performed in four patients and achieved a durable response in three patients. No patients reported any acute or late side effects from radiation.Bone involvement in MF can cause disabling pain, but single-fraction low-dose radiation is a safe and effective treatment, often leading to a durable response. Retreatment is also safe and effective inpatients who experience recurrent pain.
Article: Treatment options for hydroxyurea-refractory disease complications in myeloproliferative neoplasms: JAK2 inhibitors, radiotherapy, splenectomy and transjugular intrahepatic portosystemic shunt.[show abstract] [hide abstract]
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.European Journal Of Haematology 09/2010; 85(3):192-9. · 2.61 Impact Factor