Kasabach–Merritt syndrome, particularly in severe cases, can be complex and require the joint effort of multiple subspecialists. This is a rare disease with no consensus treatment guidelines or large randomized controlled trials to guide therapy. Supportive care Patient with Kasabach–Merritt syndrome can be extremely ill and may need intensive care. They are at risk of bleeding complications including intracranial hemorrhage. The thrombocytopenia and coagulopathy are managed with platelet transfusions and fresh frozen plasma, although caution is needed due to the risk of fluid overload and heart failure from multiple transfusions. The possibility of disseminated intravascular coagulation, a dangerous and difficult-to-manage condition, is concerning. Anticoagulant and antiplatelet medications can be used after careful assessment of the risks and benefits. Definitive treatment Generally, treatment of the underlying vascular tumor results in resolution of Kasabach–Merritt syndrome. If complete surgical resection is feasible, it provides a good opportunity for cure (although it can be dangerous to operate on a vascular tumor in a patient prone to bleeding, even with appropriate surgical subspecialists involved). If surgery is not possible, various other techniques can be used to control the tumor: embolization (by interventional radiology) can limit the tumor's blood supply external compression bandages can have similar effects certain medications, including: corticosteroids alpha-interferon chemotherapy (e.g. vincristine) radiation therapy has been used, often successfully, but now is avoided whenever possible due to the risk of long-term adverse effects (e.g. risk for future cancer). Prognosis Kasabach–Merritt syndrome has a mortality rate of about 30%. For patients that survive the acute disease, supportive care may be required through a gradual recovery.Furthermore, patients may need care from a dermatologist or plastic surgeon for residual cosmetic lesions or an otolaryngologist for head & neck/airway involvement. On long-term followup, most patients have skin discoloration and/or mild disfiguration from the dormant tumor. See also List of cutaneous conditions References External links Coagulopathies Rare syndromes Dermal and subcutaneous growths