Kasabach-Merritt Syndrome

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Clinical Kasabach-Merritt

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Inheritance

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Sporadic

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Prenata

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None listed

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Incidence

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Over 175 cases reported in medical literature in English; F:M = 2 3:1

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Age at Presentation

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Birth to first few weeks old (median age, 5 weeks old)

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Pathogenesis

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Consumption coagulopathy within a kaposiform hemangioenclotheliorna or tufted angioma

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Clinical

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Skin
Large, rapidly growing, tender, bruising reddish purple soft tissue, vascular mass with purpura; most common vascular tumors include kaposiform hemangioen¬dotheliomas or tufted angiomas; may occur with a lymphatic malformation; pe¬techiae, ecchymoses; tumor usually leaves residual stain, fibrotic plaque, papules. swelling

Hematologic Thrombocytopenia, microangiopathic hemolytic anemia, disseminated intravascu¬lar coagulation Acute hemorrhage gastrointestinal, pleural, pulmonic, CNS

Cardiac
Congestive heart failure (CHF)

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D/Dx

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Hemangioma with secondary sepsis, disseminated intravascular coagulation (DIC)

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Lab

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Complete blood cell count Prothrombin (PT)/activated partial thromboplastin time (aPTT) prolonged; fibrinogen level decreased; fibrin degradation products increased

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Management

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Prednisone
Vincristine
Interferon a
Referral to pediatric hematologist transfusions, infusions of fibrinogen and fresh frozen plasma; platelet transfusions only if active bleeding (may enlarge mass and worsen condition) Referral to pediatric surgeon embolization, surgical excision
Referral to pediatric cardiologist management of CHF

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Prognosis

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Up to 20% mortality secondary to hemorrhage, infection, or iatrogenic causes