Chronic consumption coagulopathy due to an occult splenic haemangioma: Kasabach-Merritt syndrome.
We report an 11-year-old girl with a 2-year history of bruising associated with thrombocytopenia and dysfibrinogenaemia. On admission she presented with a large subcutaneous haematoma and splenomegaly and was severely anaemic. Laboratory investigations revealed signs of consumption coagulopathy. Radiological examination showed splenic, retroperitoneal and intra-ossal haemangiomas. After splenectomy, platelet count and coagulation parameters returned to normal.


CONCLUSIONS
Contrary to widely held views, occult visceral haemangioma can lead to Kasabach-Merritt syndrome beyond infancy and is not necessarily associated with visible cutaneous haemangioma. It should be included in the differential diagnosis of chronic thrombocytopenia at any age. Early determination of fibrinogen degradation product levels is advised in order to detect an underlying chronic consumption coagulopathy prompted by an extensive search for multifocal haemangioma.
Hoeger
P H
PH
Universität Hamburg, Kinderklinik, Germany.
Helmke
K
K
Winkler
K
K
D000347:Afibrinogenemia / Q000150:complications; D002648:Child; D002908:Chronic Disease; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000209:etiology* / Q000601:surgery; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000378:metabolism*; D006392:Hemangioma, Cavernous / Q000150:complications*; D006403:Hematologic Tests; D006801:Humans; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000150:complications*; D013163:Splenomegaly / Q000150:complications; D013577:Syndrome; D013921:Thrombocytopenia / Q000209:etiology
D005338:Fibrin Fibrinogen Degradation Products