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 referred 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 hi...

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Bibliographic Details
Published in:American journal of hematology 2000-12, Vol.65 (4), p.315-318
Main Authors: Notoya, A., Bohgaki, T., Mukai, M., Kohno, M., Sato, H., Sawada, K.
Format: Article
Language:English
Subjects:
Biological and medical sciences
chronic DIC
Disseminated Intravascular Coagulation - complications
Female
Hematologic and hematopoietic diseases
Humans
Medical sciences
Middle Aged
Osler's disease
Platelet diseases and coagulopathies
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
splenomegaly
Splenomegaly - complications
Telangiectasia, Hereditary Hemorrhagic - complications
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Summary:A 45‐year‐old Japanese woman with splenomegaly and thrombocytopenia was referred 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, and other data consistent with chronic disseminated intravascular coagulation (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 scintigraphy 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 findings indicated that fragility of the fine vascular architecture of the splenic red pulp might have been responsible for pathogenesis. The large pooling of blood with coagulation was thought to be secondary. Am. J. Hematol. 65:315–318, 2000. © 2000 Wiley‐Liss, Inc.
ISSN:0361-8609
1096-8652
DOI:10.1002/1096-8652(200012)65:4<315::AID-AJH12>3.0.CO;2-8