Kasabach merritt syndrome: management with interferon.
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.
Department of Dermatology, Amala Institute of Medical Sciences,(AIMS), Thrissur, Kerala, India.
Sandhya
S
Acharya
Kalyani
K
Pillai
Abel
A
Francis
S
S
Criton
V K
VK
Parvathi