Kamya Sankar1, Kristen Pettit2. 1. Division of Hematology/Oncology, University of Michigan, C300 Med Inn, SPC 5848, 1500 E. Medical Center Dr., Ann Arbor, MI, 48109-5848, USA. 2. Division of Hematology/Oncology, Rogel Cancer Center, University of Michigan, 1500 E. Medical Center Drive, Ann Arbor, MI, 48109-5848, USA. krpettit@med.umich.edu.
Abstract
PURPOSE OF REVIEW: Myelofibrosis is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly. Currently approved medical therapies do not improve splenomegaly in all patients and effects are not sustained. Thus, spleen-directed therapies (i.e., splenectomy and splenic irradiation) have been used in some cases to palliate the signs and symptoms of the disease. Here, we critically review the literature regarding palliative splenectomy and splenic irradiation in myelofibrosis, and discuss their position in the current treatment landscape. RECENT FINDINGS: Retrospective studies have demonstrated that splenectomy improves symptoms of splenomegaly, decreases complications of portal hypertension, and decreases transfusion dependence. However, it carries a significant peri-operative and long-term morbidity and mortality rate. Splenic irradiation reduces splenic size but is limited by duration of response and myelosuppression. Spleen-directed therapies in myelofibrosis may be considered for refractory symptoms and complications of massive splenomegaly after carefully weighing the associated risks, though overall survival may not be impacted. Development of medical therapies that target and reverse the underlying disease pathophysiology is required in order to have a significant impact on the natural history of the disease process.
PURPOSE OF REVIEW: Myelofibrosis is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly. Currently approved medical therapies do not improve splenomegaly in all patients and effects are not sustained. Thus, spleen-directed therapies (i.e., splenectomy and splenic irradiation) have been used in some cases to palliate the signs and symptoms of the disease. Here, we critically review the literature regarding palliative splenectomy and splenic irradiation in myelofibrosis, and discuss their position in the current treatment landscape. RECENT FINDINGS: Retrospective studies have demonstrated that splenectomy improves symptoms of splenomegaly, decreases complications of portal hypertension, and decreases transfusion dependence. However, it carries a significant peri-operative and long-term morbidity and mortality rate. Splenic irradiation reduces splenic size but is limited by duration of response and myelosuppression. Spleen-directed therapies in myelofibrosis may be considered for refractory symptoms and complications of massive splenomegaly after carefully weighing the associated risks, though overall survival may not be impacted. Development of medical therapies that target and reverse the underlying disease pathophysiology is required in order to have a significant impact on the natural history of the disease process.