KASABACH-MERRITT SYNDROME – A COMPREHENSIVE REVIEW

Haemangiomas are vascular lesions caused by abnormal blood vessel proliferation, especially in children. Kasabach-Merritt syndrome (KMS) is a rare and life-threatening thrombocytopenia and consumptive coagulopathy associated with aggressive vascular tumors in infancy and younger children. Kasabach-Merritt phenomena is a unique phenomenon in kaposiform hemangioendotheliomas (KHE) and tufted angiomas (TA), occurring in 70% of KHE cases and less often in TA instances. Risk factors for KMS include patient age under 6 months, lesion size greater than 5.0 cm, and the presence of a mixed lesion. Diagnosis of KMS is essential for preventing hemorrhagic complications and establishing a treatment plan. Physical examination, imaging, and biopsy are essential for identifying the underlying vascular tumors. Severe thrombocytopenia, a component of Kaposiform hemangiomas, is a potentially fatal side effect of vascular tumors in children. Treatment aims to manage thrombocytopenia and avoid lethal outcomes. According to SEOP (Spanish Society of Pediatric Oncology) guidelines for vascular tumors complicated by KMS, including vincristine, aspirin, and ticlopidine (VAT therapy).  Corticosteroids are the most commonly used acute treatment for KMP, with vincristine being suggested as an effective option.