KASABACH-MERRITT SYNDROME

By Gary M. White, MD


Kasabach-Merritt syndrome (KMS) is defined as decreased levels of circulating platelets and clotting factors associated with a vascular proliferation, either Kaposi’'s hemangioendothelioma or a tufted angioma.

Clinical

Swelling, induration, tenderness, and purpura of a large vascular lesion associated with a consumptive coagulopathy and thrombocytopenia is characteristic. Petechiae, purpura, bruising, and bleeding may occur. Tachycardia can be a sign of anemia. The entire skin surface should be examined for red, blue, or purple vascular lesions, but an internal vascular lesion (e.g., of the liver) may be the cause.

Treatment

Treatment is multidisciplinary and should be carried out by experts in this condition. Blood workup to define the severity of the anemia and coagulopathy (e.g., CBC with platelet count, PT, PTT, fibrinogen) are done along with imaging of the tumor (e.g., MRI, CT).

Oral sirolimus, which is directed against the mTOR downstream signaling pathway involved in lymphangiogenesis, has also shown promising results for lymphatic malformations and KMS [Eur J Pediatr. 2015 Dec;174:1579]. In another report, oral sirolimus was highly effective with minimal side effects for vincristine-resistant KMS [PD 2017;34;261].

Combining antiplatelet therapy with vincristine has given excellent results, without the need for steroid use. Other treatments that have been used in the past include surgical excision, arterial embolization, corticosteroids, vincristine, ticlopidine with aspirin, heparin, interferon-α, cyclophosphamide, and propranolol. Low-dose radiation therapy was used with success [PD 2014;31;595–598].

References

Great pictures showing the sudden increase in size with severe pain. At 8 months of age and then at 14 months. Indian J Dermatol 2010;55:281-3

Progressive swelling of a congenital vascular mark. Cases Journal 2008, 1:9

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