Clinical Review: Management of Adult Kasabach-Merritt Syndrome Associated with Hemangiomas

Abstract

Kasabach-Merritt syndrome (KMS) consists of a clinical trial of capillary hemangioma, thrombocytopenia and disseminated intravascular coagulation. KMS occurs most commonly in the pediatric population, and its occurrence in adults is rare. Specific guidelines or randomized clinical trials guiding clinical management of KMS in adult patients are lacking. This manuscript provides a comprehensive review of KMS and discusses recent advances in the medical management of KMS. We also propose a systematic therapeutic approach which would serve as a guide in the management of adult patients with KMS caused by hemangiomas.

Massive liver haemangioma causing Kasabach–Merritt syndrome in an adult

“…In addition to the surgical options discussed above, chemotherapy, steroids, anticoagulants, radiation therapy, aspirin, propranolol, and others have all been reported as possible treatment options for Kasabach-Merritt syndrome. 4 Liver haemangiomas can reach astounding sizes, as in our patient, and may have dramatic unexpected complications like Kasabach-Merritt syndrome. It is vital for surgeons, radiologists and internists alike to know of such possibilities to correctly diagnose and treat them when encountered.…”

“…The pathophysiology is poorly understood, but it is thought that the abnormal endothelium within the tumour leads to platelet trapping and activation with subsequent consumption and activation Figure 3 Intraoperative images of the liver haemangioma of the coagulation cascade, leading to the laboratory findings of consumptive coagulopathy. 4 The process seems to be localised in the tumour rather than generalised, as suggested by previous studies, but it may be exacerbated by surgery or biopsies, extending into a more disseminated process, manifesting as further thrombocytopenia, anaemia or developing venous thrombosis. 5 In the few reported adult cases, the presenting symptoms include abdominal pain, increased mass size, disseminated intravascular coagulopathy, bleeding, fractures and high output cardiac failure.…”

“…3 Kasabach-Merritt syndrome, first described in an infant by Kasabach and Merritt in 1940, is a syndrome that associates giant haemangiomas with consumptive coagulopathy. 4 Associated laboratory abnormalities include thrombocytopenia, hypofibrinogenaemia, increased fibrinolysis, prolonged INR and elevated D-dimer levels. The syndrome is usually described in children in association with tufted angiomas and kaposiform haemangioendotheliomas.…”

Rare Benign Focal Lesions of the Liver: Report of 2 Cases and Literature Review

“…Most often, this syndrome manifests itself in childhood, it is very rare in adults, but there have been documented cases when it was accompanied by the presence of a giant hemangioma [ 18 ]. The pathogenesis of Kasabach-Merritt syndrome most likely involves both primary and secondary hemostatic mechanisms, leading to platelet clumping, activation, and aggregation and thus to their consumption, along with activation of the coagulation cascade within the abnormal vascular structure of the hemangioma [ 19 ].…”