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Characterized by the association of a vascular tumor with thrombocytopenia and coagulopathy. Histologically, it is either a kaposiform hemangioendothelioma or a tuft angioma, not a hemangioma.

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Hemangioma-Thrombocytopenia Syndrome.

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Approximately 300 cases have been reported in the literature since 1940. Slightly more frequent in females. No racial predilection.

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Unknown; seems to be more frequent in Asia.

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Ecchymotic tumor develops on a vascular lesion; it usually starts in the neonatal period and grows very quickly; tumoral growth is accompanied by thrombocytopenia and hypofibrinogenemia. This disseminated intravascular coagulation (DIC) picture is secondary to entrapment of platelets within the vascular tumor.

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The triad of thrombocytopenia, coagulopathy, and a vascular tumor should be highly suggestive.

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Enlarging hemangioma-like lesion, mainly cutaneous (especially on the extremities) but also visceral. DIC (decreased platelets and fibrinogen, presence of increased d-dimers). Often inoperable. Treatment options: vincristine, interferon-α, steroids, ticlopidine with aspirin, or radiotherapy. Untreated Kasabach-Merritt syndrome has a 10 to 37% mortality rate.

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Examine for airway involvement. Check complete blood count (platelet count), coagulation profile, bleeding time, and cross-match for blood even for minor surgical procedures.

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Potential difficult airway if oropharyngeal or neck involvement is present. Anticipate the need for blood products to treat anemia and/or coagulopathy. Do not transfuse platelets because they will immediately be trapped within the tumor with an ensuing increase in size. Effect of chemotherapy on the cardiac and respiratory system must be considered.

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No specific pharmacological implications with this condition; however, patients treated with chemotherapy and corticosteroids (perioperative stress dose) must be prepared for anesthesia accordingly. Avoid nonsteroidal antiinflammatory drugs secondary to their effects on platelets.

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Congenital Hemangiomas: Share many similarities with Kasabach-Merritt syndrome, but without the coagulation disorders; they usually regress between 5 and 9 years of age.

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Klippel-Trenaunay Syndrome: Congenital arteriovenous malformation usually located on a single extremity resulting in skeletal and soft tissue hypertrophy.

David TJ: Hemangioma with thrombocytopenia (Kasabach-Merritt syndrome). Arch Dis Child 58:1022, 1953.
Enjolras O, Wassef M, Mazoyer E, et al: Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 130:631, 1997.  [PubMed: 9108863]
Maguiness S, Guenther L: Kasabach-Merritt syndrome. J Cutan Med Surg 6:335, 2002.  [PubMed: 11951132]

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