A case of Myelofibrosis with Splenohepatomegaly

Abstract

A 63-year old woman affected by splenomegaly, anemia and moderate leukocytosis was submitted to splenectomy for a supposed Banti disease. Histological examinations showed splenic invasion by undifferentiated mesenchymal cells with partial myeloid differentiation and with arteriolar hyalinosis; hepatic biopsy revealed only a moderate phlogosis. Following splenectomy a rapid enlargement of the liver was observed, with increase of erythrocytes to 5.6 millions per mm³ and appearance of erythroblasts in the circulating blood; neutrophilic leukocytosis to 200,000 cells per mm³ with presence of myelocytes and metamyelocytes and of occasional atypical cells; increase of platelets to 500,000 elements per mm³ with giant thrombocytes; bone marrow showed areas of fibrosis and a normal formula. The patient died 27 months after splenectomy with a terminal period of profuse enterorrhagia and anemia; post-mortem examination of the liver showed infiltration of cells of erythroblastic type and diffuse cirrhotic fibrosis. The case has been ascribed to the myelofibrosis and defined as a « panmyelosis with fibrotic evolution », with prevailing spleno-hepatic involvement, erythro-leuko-thrombocytemia and terminal liver cirrhosis.