Ectopic medullary hematopoiesis as a cause of ascites in agnogenic myeloid metaplasia. Case report and review of the literature.

We report an unusual case of a 44-year-old female patient with 'malignant' ascites caused by ectopic foci of extramedullary hematopoiesis in the course of agnogenic myeloid metaplasia. The patient had suffered also from severe Coombs-positive acquired hemolytic anemia and had been splenectomized. Two years after splenectomy, ascites caused by peritoneal implants of hematopoietic tissue appeared. The ascites responded promptly to treatment with busulfan and hydroxyurea. The clinical picture, treatment and a review of the literature concerning the mechanisms of this uncommon evolution are discussed.