C R, V P, P J, S M, S B, L B, V B, E P, M W
Kasabach-Merritt phenomenon: a single centre experience. [JOURNAL ARTICLE]
Eur J Haematol 2009 Nov 3.
Abstract
OBJECTIVE: Kasabach Merritt phenomenon (KMP) can lead to life-threatening bleeding and its optimum treatment has not been established. We review the experience of managing KMP in a single institution.
METHODS: A retrospective chart review on all children with KMP treated at the Hospital for Sick Children, Toronto, over an 18 year period.
RESULTS: All 15 patients had profound thrombocytopenia and hypofibrinoginameia at presentation, half had bleeding symptoms, and three had cardiac failure. All patients received corticosteroids. Five responded to steroids alone, given for an average of 13 weeks, increasing platelets to >20 x 10(9)/L at a mean of 6.2 days and fibrinogen > 1 g/dL at 25.6 days. Ten patients received at least one other therapeutic modality in addition to steroids, including Vincristine, Interferon, anti-platelet agents, and pentoxifylline. Five patients received Vincristine, for a mean of six weeks, with two patients responding. Eight patients received Interferon, for a mean of four months, with two responding. Overall, the mean time to increasing platelets > 20 x 10(9)/l was 56 days, to > 150 x 10(9)/L was 88 days, and fibrinogen > 1 g/dL 49 days. Ten patients showed a partial response to embolization, with a mean of 2.8 procedures performed. Thrombotic complications occurred in 7%. Twelve patients remain alive, with relapse in six patients, all treated successfully. One patient died, and two patients have been lost to follow up.
CONCLUSION: KMP is a rare condition, with significant morbidity and mortality. The therapeutic approach should include a multidisciplinary team and consensus on guidelines.
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