All issues > Volume 34(3); 1991

Original Article

J Korean Pediatr Soc. 1991;34(3):371-379. Published online March 31, 1991.

Clinical study of Kasabach-Merritt syndrome.

Eun Jung Bae¹, Young Ah Lee¹, Hee Young Shin¹, Hyo Seop Ahn¹

¹Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

Received: July 12, 1990;  Accepted: October 17, 1990.

Abstract

A retrospective analysis of clinical findings in 20 patients with Kasabach-Merritt syndrome who visited Department of Pediatrics, Seoul National University Hospital from January, 1979 to June, 1990 was performed. The average age of occurrence was 6.4 month and male to female ratio was 1: 1.2. The mean size of hemangioma was 11.0x8.5 cm and the locations were cutanous in 18 (extrem- ities 5, trunk 4, head and neck 3, combined cutaneous 4, multiple cutaneous 2) and non-cutaneous in 2 (spleen 1, retroperitoneum 1). The initial platelet count was 35,900 ±11,000 per mm3 and hypofi- brinogenemia (below 160 mg/dl) was noted in 17 (85%). Among 20 cases, surgical resection was performed in 2. Steroid was tried initially in 13 over 1 week, and 30% (4/13) showed initial response on 4.0±1.2 days. Steroid and radiation were given in 15 with initial response rate of 80% (12/15) on 8.2 ±3.9 days. Till now 17 out of 20 patients experienced resolution of their lesions (including sponta- neous regression of a patient) with improvement of thrombocytopenia and coagulopathy. The clear comparison of the effectiveness between steroid therapy and radiotherapy couldn’t be possible, but the combination therapy of steroid and radiotherapy was superior to single steroid therapy (p<0,05). So we recommend the first choice of therapy might be steroid, but if response was not found within 1 week, consider the combination therapy with radiation.

Keywords :Kasabach-Merritt syndrome, Hemangioma, Thrombocytopenia, Steroid, Radiotherapy