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Panminerva Medica 1998 Giugno;40(2):128-31

lingua: Inglese

Kasabach-Merrit syndrome in ­infants

Sarihan H., Mocan H.*, Abeys M., Akyazucu R., Cay A., Imamoglu M.

From the Department of Pediatric Surgery and * Pediatric Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey


Methods. Four ­infants ­with Kasahach-Merrit syndrome ­have ­been ­treated at the University Hos­pital, Trabzon. They had ­large ­varied-­site cuta­neous heman­giomas. Diagnosis was per­formed ­with clin­ical and labor­a­tory ­studies. All ­patients has ­severe thom­bo­cy­to­penia and ­anemia. Fibrinogen and ­fibrin ­split prod­ucts ­were exam­ined in two ­patients and ­lower ­fibrinogen and ­over ­fibrin ­split prod­ucts ­levels ­were ­detected in ­them. All ­patients ­were ­admitted to the inten­sive ­care ­unit and ­they ­were ­treated ­with anti­bi­o­tics, ­fresh ­blood trans­fu­sion and thom­bo­cyte sus­pen­sion. Two out of ­four ­patients ­were pre­vi­ously ­treated ­with ster­oids unsuc­cess­fully and one ­patient ­died due to dis­sem­i­nated intra­vas­cular coag­u­lop­athy. Three ­patients ­were ­treated ­with inter­feron ­alfa-2a and com­pres­sion.
Results. In two ­patients the ­lesions ­regressed 60-80% fol­lowing the ­five ­months ­therapy and in the ­other ­patient the ­lesion was com­pletely ­excised after one ­month ­therapy.
Conclusions. Interferon ­alfa-2a and com­pres­sion ­were ­found to be remark­ably effec­tive in the treat­ment of Kasabach-Merrit syndrome.

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