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  • Title: [Kasabach-Merritt syndrome. Intraoperative heparin therapy with follow-up combination therapy of heparin and ticlopidine for the control of intravascular coagulation].
    Author: Korte W, Blatter G, Ziegler JP, Hanselmann C, Schmid L.
    Journal: Dtsch Med Wochenschr; 1993 Mar 19; 118(11):371-6. PubMed ID: 8453908.
    Abstract:
    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.
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