Summary
Kasabach-Merritt syndrome or phenomenon is characterized by thrombocytopenia, consumptive coagulopathy and a microangiopathic hemolytic anemia, seen in patients with an enlarging vascular lesion (especially Kaposiform haemangioendothelioma; khe). Due to the coagulopathy the mortality rate is high. Many treatments have been tried showing different results. This article describes succesful treatment with vincristine.
samenvatting
Het Kasabach-Merritt-syndroom of -fenomeen (kmf) wordt gekenmerkt door trombocytopenie, verbruikscoagulopathie en een microangiopathische hemolytische anemie bij patiënten met een groeiende vasculaire laesie (vooral het Kaposiform haemangioendothelioma; khe). Tengevolge van de stollingsafwijkingen is er een hoge mortaliteit. Verschillende therapieën zijn tot dusver met wisselend succes geprobeerd. In dit artikel wordt een casus beschreven waarbij uiteindelijk vincristine succesvol was.
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J.T.C.M. Verhallen, arts-assistente, afdeling Kindergeneeskunde, Erasmus MC-Sophia, Rotterdam.
Mw. J.T.C.M. Verhallen, arts-assistente, afdeling Kindergeneeskunde, Erasmus MC-Sophia, Dr. Molenwaterplein 60, 3015 GJ Rotterdam.
P.C.J. de Laat, kinderarts, Erasmus MC-Sophia, Rotterdam.
G.A. Madern, kinderchirurg, Erasmus MC-Sophia, Rotterdam.
C. van Eden, kinderarts, Medisch Centrum Leeuwarden.
A.P. Oranje, dermatoloog, Erasmus MC-Dijkzigt/Sophia, Rotterdam.
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Verhallen, J.T.C.M., de Laat, P.C.J., Devillers, A.C.A. et al. Het Kasabach-Merritt-syndroom. KIND 71, 123–126 (2003). https://doi.org/10.1007/BF03061442
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DOI: https://doi.org/10.1007/BF03061442