Ectopic Medullary Hematopoiesis as a Cause of Ascites in Agnogenic Myeloid Metaplasia

Abstract

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 hemato-poietic 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.

Nonhepatosplenic Extramedullary Hematopoiesis: Associated Diseases, Pathology, Clinical Course, and Treatment

“…[37][38][39] Occasionally, an associated disease is not identified. 40,41 Clinically, NHS-EMH may present as an incidental finding or with a symptomatic disease or condition, including pleural effusion, 42,43 ascites, 14,44 neurologic deficit, 45 cardiac tamponade, 11,46 chronic renal failure, 47 acute respiratory failure, 48 orbital proptosis, 49 and subglottic stenosis. 8 Antemortem diagnosis can be made by tissue biopsy, fineneedle aspiration (FNA) biopsy, or radionuclide scanning.…”

Nontuberculous mycobacterial infection in a patient with myelofibrosis: case report and concise review

“…Symptomatic ascites in PM is rare and is often attributed to portal hypertension accompanied by ectopic hematopoiesis in the liver and spleen, and in some cases thrombosis of the portal vein [11]. In the previously reported cases, ascites occurred after splenectomy, as initial manifestation, or from 2 months to 17 years after the diagnosis of myelofibrosis was established [1,5,9].…”

“…Patients with symptomatic massive ascites caused by peritoneal extramedullary hematopoiesis have been reported previously, but are limited in number [5][6][7][8][9][10].…”

Extramedullary peritoneal hematopoiesis combined with tuberculosis in a patient with primary myelofibrosis

“…Extramedullary hematopoieses frequently occurs in the liver, spleen, and lymph nodes in patients with PMF, and rarely involves the skull, paranasal sinus, thyroid, pleura, vertebrae, kidneys, adrenal glands, mesentery, pelvic cavity, uterus, or skin [3]. Several case reports have described ascites developing as a consequence of extramedullary hematopoiesis, without evidence of superimposed infection [4]. Our patient presented with fever and abdominal distension.…”