KASABACH MERRITT SYNDROME IN KAPOSI HEMANGIO-ENDOTHELIOMA

Abstract

Kasabach Merritt syndrome (KMS) is also known as hemangioma- thrombocytopenia syndrome. It is a rare disorder characterised by profound thrombocytopenia, microangiopathic hemolytic anemia, subsequent consumptive coagulopathy in association with vascular tumours -especially kaposiform hemangioendothelioma/ tufted angiomas. KMS is usually caused by kaposiform hemangio-endothelioma or tufted angioma and other vascular tumours like infantile hemangioma.

KHE and tufted angioma are intermediate locally aggressive childhood vascular tumours that are locally aggressive and often spread to the area around tumour.

Pathophysiology of KMS is poorly understood and complicated. The proposed mechanism involves both primary and secondary hemostatic mechanisms leading to platelet trapping, platelet activation/ aggregation and platelet consumption within the abnormal vascular structures.

We report a case of two day old male child presented with multiple areas of petechial haemorrhage on plantar aspect of both feet, right leg, both upper limbs, forehead and bilateral cheeks and large hemangiomas in left lower limb, lateral pelvic wall and abdominal wall. Hemogram showed thrombocytopenia with platelet count of 20,000/ cumm. Ultrasonography and MRI showed diffuse hemangioma in left leg extending in pelvis along left lateral pelvic wall, medial to the iliacus muscle and extending in the retroperitoneum, encasing aorta upto the level of renal hilum. Diagnosis of kasabach meritt syndrome in Kaposi hemangio-endothelioma was made.