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Rivista di Medicina Interna
Indexed/Abstracted in: BIOSIS Previews, Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,6
Panminerva Medica 1998 Giugno;40(2):128-31
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.