Massive ascites caused by peritoneal extramedullary hematopoiesis as the initial manifestation of myelofibrosis.

An 83-year-old man presented with massive ascites. Cytological examination of the ascites revealed primitive hematopoietic cells. The specimen of bone marrow biopsy disclosed diffuse fibrosis, which suggested myelofibrosis with extramedullary hematopoiesis as the cause of ascites. His symptoms resolved dramatically after administration of hydroxyurea. Ascites formation in myelofibrosis is often attributed to presinusoidal portal hypertension and occurs in the context of well-established disease. This case demonstrates that myelofibrosis may manifest as massive ascites caused by peritoneal implants of myeloid tissues. Extramedullary hematopoiesis should be included in the differential diagnosis of ascites.