[Kasabach-Merritt syndrome. Intraoperative heparin therapy with follow-up combination therapy of heparin and ticlopidine for the control of intravascular coagulation].
A 39-year-old woman with Kasabach-Merritt syndrome (cavernous vascular malformations with disseminated intravascular coagulation) sustained a displaced fracture of the femoral shaft. Despite pronounced hypofibrinogenemia (< 0.1 g/l) there was no significant bleeding. The concentration of D dimer was raised to > 32 < 64 mg/l and that of prothrombin fragments F1 and F2 to > 10 nmol/l. The platelet count was 102,000/microliters and other coagulation parameters were normal or only slightly abnormal. The consumption coagulopathy was successfully controlled by continuous intravenous infusion of heparin (17,000 I.U./d) without need for clotting factor replacement, and was subsequently stabilized by combined treatment with low molecular weight heparin (5,000 I.U./d s.c.) and ticlopidine (250 mg twice daily by mouth). Oral therapy with acetylsalicylic acid alone or in combination with ticlopidine proved insufficient, being rapidly followed by a renewed fall in fibrinogen level and platelet count. The findings suggest that fibrin formation in the abnormal vascular territories was the principal pathogenetic factor in this case.
Korte
W
W
Institut für Klinische Chemie und Hämatologie, Kantonsspitals St. Gallen.
Blatter
G
G
Ziegler
J P
JP
Hanselmann
C
C
Schmid
L
L
D000008:Abdominal Neoplasms / Q000150:complications*; D000328:Adult; D000347:Afibrinogenemia / Q000150:complications; D001780:Blood Coagulation Tests; D004211:Disseminated Intravascular Coagulation / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D004359:Drug Therapy, Combination; D005260:Female; D005264:Femoral Fractures / Q000601:surgery; D006392:Hemangioma, Cavernous / Q000150:complications*; D006493:Heparin / Q000008:administration & dosage*; D006801:Humans; D007430:Intraoperative Care; D013577:Syndrome; D013988:Ticlopidine / Q000008:administration & dosage*
D006493:Heparin; D013988:Ticlopidine