Splenomegaly and chronic disseminated intravascular coagulation in Osler-Weber-Rendu Disease: A case report

A 45-year-old Japanese woman with splenomegaly and thrombocytopenia was ref erred to our hospital. The diagnosis of Osler-Weber-Rendu disease (Osler's disease) was made because of spotty telangiectasia on her tongue, recurrent epistaxis since childhood, and a diathesis indicated by her family history . The patient's laboratory examination revealed anemia, thrombocytopenia, a nd other data consistent with chronic disseminated intravascular coagulatio n (DIC), Bone marrow examination was normal. Abdominal computed tomography showed marked enlargement of the spleen with deformity and calcified plaque , not homogeneously enhancing. Hypersplenism was not observed, platelet sci ntigraphy indicated a remarkable uptake in the spleen. She was diagnosed as having chronic DIC associated with vascular lesions of Osler's disease in the spleen, Splenectomy was performed and the subsequent pathological findi ngs indicated that fragility of the fine vascular architecture of the splen ic red pulp might have been responsible for pathogenesis, The large pooling of blood with coagulation was thought to be secondary. (C) 2000 Wiley-Liss , Inc.