Antifibrinolytic therapy in the management of the kasabach merritt syndrome

Abstract

The Kasabach Merritt syndrome consists of thrombocytopenia, microangiopathic hemolytic anemia, and a localized consumption coagulopathy that develops within the abnormal vascular channels of a hemangioma. In general, these patients demonstrate only mild abnormalities of screening clotting tests, but they can potentially develop life-threatening complications. We present a patient who developed a severe anemia that was refractory to erythrocyte transfusions. Treatment with epsilon-aminocaproic acid to inhibit fibrinolysis and cryoprecipitate to replenish his deficient circulating fibrinogen interrupted the cycle of his systemic coagulopathy and enabled us to transfuse him to a normal hematocrit.

Treatment of Kasabach-Merritt syndrome by embolisation of a giant liver hemangioma

“…With the aim to induce thrombosis of the neoplastic vasculature, the use of antifibrinolytic agents has been suggested [3,4], although, as described in the few cases in the literature, such an approach was successful only with the concomitant administration of cryoprecipitate as source of clotting material for the direct peripheric embolisation of the hemangioma. Nevertheless, this therapeutic strategy is not without potentially severe adverse effects, i.e., acute renal failure secondary to glomerular capillary thrombosis, arteriovenous occlusion of the extremities with gangrene, and pulmonary emboli.…”

Kasabach–Merritt Syndrome

“…Systemic steroids, usually at doses of 2±4 mg/kg/day have been used in almost all reported cases of KMS. 3,8,11,12,16,18±22 Anti®brinolytics, such as e-aminocaproic acid and transexamic acid, were used in KMS to reduce the risk of bleeding 18,21,22 in addition to antiplatelet agents, such as acetylsalicylic acid and dypyridamole. 17,21,22 These agents were previously used more frequently in conjunction with steroids and are theoretically useful in preventing platelet aggregation within the body of the tumor.…”

Treatment of lymphangioma with interferon-alpha-2a

“…Isso poderia trazer alguma vantagem aos nossos pacientes, tanto no que diz respeito às lesões hemangiomatosas quanto às lesões linfangiomatosas. O ácido épsilon-aminocapróico, interferindo no mecanismo fibrinolítico, cuja ativação dentro dos hemangiomas preveniria a ocorrência de trombose, evitaria a dissolução de trombos formados na microcirculação dos hemangiomas 18,19 . Como os pacientes em questão não apresentaram nenhuma vantagem aparente sobre aqueles em uso apenas do a-2a-INF, essas drogas foram suspensas.…”