Non-Pharmacologic Management of Splenomegaly for Patients with Myelofibrosis: Is There Any Role for Splenectomy or Splen
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MYELOPROLIFERATIVE NEOPLASMS (B STEIN, SECTION EDITOR)
Non-Pharmacologic Management of Splenomegaly for Patients with Myelofibrosis: Is There Any Role for Splenectomy or Splenic Radiation in 2020? Kamya Sankar 1 & Kristen Pettit 2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
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. Summary 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. Keywords Myelofibrosis . Splenomegaly . Splenectomy . Splenic irradiation
Introduction The Philadelphia chromosome–negative myeloproliferative neoplasms (MPN) including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF) are clonal hematopoietic disorders characterized by abnormal expansion of myelopoiesis, bleeding, thrombosis, and potential for transformation into acute myeloid leukemia (AML). MF is caused by a clonal multipotent hematopoietic stem cell This article is part of the Topical Collection on Myeloproliferative Neoplasms * Kristen Pettit [email protected] 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
(HSC) driving myeloproliferation and fibrogenesis in the bone marrow. It can lead to variable degrees of cytopenias and abnormal extramedullary hematopoiesis (EMH). EMH most commonly occurs in the liver and spleen, though it can involve any organ and can occur in the presence or absence of an underlying clonal hematopoietic disorder. MF can develop idiopathically (i.e., primary MF or PMF) or evolve from a preexisting diagnosis of PV or ET (i.e., post-PV MF or post-ET MF) [1•]. While
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