Year: 2024 | Month: May | Volume: 14 | Issue: 5 | Pages: 219-223
DOI: https://doi.org/10.52403/ijhsr.20240527
A Case Report of Kasabach-Merritt Syndrome with Splenomegaly and Splenic Hemangioma
Beny Margrat1, A. Priya2, K. Arun Chander3
1,2Department of Clinical Pharmacology, Apollo Children’s Hospital, Greams Road, Thousand Lights, Chennai
3Consultant and Head, Department of clinical pharmacology Apollo Children’s Hospital, Greams Road, Thousand Lights, Chennai
Corresponding Author: Dr. K. Arun Chander
ABSTRACT
Kasabach-Merritt syndrome is characterized by a combination of thrombocytopenia, microangiopathic hemolytic anemia, hemangioma, and hypofibrinogenemia. KMP (Kasabach- Merritt Phenomena) usually presents with an enlarging purpuric lesion involving the trunk extremities, face, and retroperitoneum. Hemangiomas are benign neoplasms characterized by the abnormal proliferation of blood vessels. It can occur anywhere in the body. Hemangioma can involve numerous solid organs, including the liver, spleen, gastrointestinal tract, brain, and lungs. The term hemangiomatosis may be applied in the setting of very large or numerous hemangiomas. Intralesional fibrinolysis is the main reason for abnormal laboratory values, including elevation of the international normalized ratio and D-dimer level. As a consequence, Kasabach-Merritt syndrome can result in severe disturbances of blood coagulation, such as disseminated intravascular coagulation. Disseminated intravascular coagulation is characterized by the widespread activation of coagulation, which results in the intravascular formation of fibrin and ultimately the thrombotic occlusion of small and midsize vessels. The aim of this report is to describe the clinical course of the patients before and after the treatment, which will hopefully guide future treatment in Kasabach-Merritt syndrome with splenomegaly and splenic hemangioma. There is no universally applicable treatment or procedure for Kasabach-Merritt syndrome, but based on several studies, some treatments have been found to be effective, which include embolization, radiation therapy, laser therapy, sclerotherapy, the use of corticosteroids, interferon, and chemotherapy.
Key words: Kasabach-Merritt syndrome, Video-assisted thoracoscopic surgery, Disseminated intravascular coagulation, Kaposiform hemangioendothelioma, Tufted Angioma, Fresh Frozen Plasma.