[Cavernous hemangioma of the spleen with a localized intravascular coagulation syndrome (Kasabach-Merritt syndrome). Ultrastructural study].
Haemangioma is the commonest non-malignant tumour of the spleen. Cytopenia and coagulopathy, secondary to cell trapping and coagulation factors consumption inside the haemangioma, respectively. May occasionally occur as accompanying phenomena. A woman is presented who had splenic haemangioma associated with leucopenia and thrombocytopenia along with decrease fibrinogen and prolonged prothrombin time as well as low complement rates. She had massive splenomegaly with portal hypertension, and the partial oxygen pressure values in mesenteric and portal blood were concurrent with the presence of arteriovenous shunts. The leucocyte and platelet count, the serum complement, the fibrinogen rate and the prothrombin time recovered after splenectomy. The cavernous characteristics of the spleen, containing multiple thrombi, were confirmed upon scanning electron microscope examination. The pathophysiology of the cytopenias and clotting factors consumption is discussed, stress being laid on the ultrastructural findings. A review is presented of the clinical aspects, differential diagnosis and treatment.
Tassies
D
D
Servicio de Hematología, Hospital Clinic, Facultad de Medicina, Universidad de Barcelona.
Berga
L
L
Feliu
E
E
Ferrán
M
M
Nomdedeu
B
B
Bombí
J A
JA
Vives-Corrons
J L
JL
Rozman
C
C
D000328:Adult; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006392:Hemangioma, Cavernous / Q000097:blood / Q000150:complications* / Q000601:surgery / Q000648:ultrastructure; D006801:Humans; D007970:Leukopenia / Q000209:etiology; D008855:Microscopy, Electron, Scanning; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000097:blood / Q000150:complications* / Q000601:surgery / Q000648:ultrastructure; D013163:Splenomegaly / Q000209:etiology; D013577:Syndrome; D013921:Thrombocytopenia / Q000209:etiology
Hemangioma cavernoso del bazo con síndrome de coagulación intravascular localizada (síndrome de Kasabach-Merritt). Estudio ultraestructural.