Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular
tumor. The objectives of treatment of KMS are to prevent
bleeding from consumptive coagulopathy and induce vascular
tumor regression. A 14-month old female child was brought with a reddish lesion on the left scapular area noticed at birth, which suddenly increased in size since 3 days. Hemogram revealed
anemia severe
thrombocytopenia, prolongation of
bleeding, clotting time and increased
fibrin degradable products, suggestive of KMS. Coagulopathy was managed by transfusing fresh frozen plasma and platelets. Oral
prednisolone up to 5mg/kg/day for four weeks yielded no effect on
thrombocytopenia or regression of
tumor size. Embolization of feeding artery was attempted but not feasible. We used
Interferon -alpha- 2b (IFN α 2b), in a dosage of 3million IU/m(2) /day subcutaneously. Within a month the platelet count increased and the vascular
tumor started regressing. This case signifies the importance of step wise management of KMS.