Kasabach–Merritt syndrome, not only a pediatric condition: An autopsy case report and review of literature

Kasabach–Merritt syndrome (KMS), also known as hemangioma with thrombocytopenia, is a rare vascular disease characterized by thrombocytopenia, anemia, disseminated intravascular coagulation (DIC), and vascular lesions. It often occurs during infancy and the neonatal period and rarely in adults. The syndrome presents with consumptive coagulopathy initiated within a vascular tumor, mainly tufted angiomas or Kaposiform hemangioendotheliomas, and less commonly, hepatic and/or biliary hemangiomas. We report a case of a 41-year-old female with a recent diagnosis of biopsy proven biliary-type hemangioma of the liver, undergoing fertility treatment, who developed consumptive coagulopathy and disseminated intravascular coagulation shortly following a liver biopsy procedure, and ultimately demised. Based on the review of the literature, we present the first adult case of KMS where the diagnosis was made post-mortem. Given the rarity of this condition, particularly in the adult population, we believe our case adds value to the field of critical care medicine, coagulopathies, and forensic medicine.

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Published
2025-02-04
DOI
https://doi.org/10.5348/100085z11zz2025cr
Primary Topic
Vascular Malformations and Hemangiomas
Type
article
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article

Kasabach–Merritt syndrome, not only a pediatric condition: An autopsy case report and review of literature

Roberto J. Rivero-Soto, Zarrin Hossein-Zadeh, Jane Date C Hon
Vascular Malformations and Hemangiomas
article

Kasabach–Merritt syndrome, not only a pediatric condition: An autopsy case report and review of literature

Roberto J. Rivero-Soto, Zarrin Hossein-Zadeh, Jane Date C Hon
article en

Abstract

Kasabach–Merritt syndrome (KMS), also known as hemangioma with thrombocytopenia, is a rare vascular disease characterized by thrombocytopenia, anemia, disseminated intravascular coagulation (DIC), and vascular lesions. It often occurs during infancy and the neonatal period and rarely in adults. The syndrome presents with consumptive coagulopathy initiated within a vascular tumor, mainly tufted angiomas or Kaposiform hemangioendotheliomas, and less commonly, hepatic and/or biliary hemangiomas. We report a case of a 41-year-old female with a recent diagnosis of biopsy proven biliary-type hemangioma of the liver, undergoing fertility treatment, who developed consumptive coagulopathy and disseminated intravascular coagulation shortly following a liver biopsy procedure, and ultimately demised. Based on the review of the literature, we present the first adult case of KMS where the diagnosis was made post-mortem. Given the rarity of this condition, particularly in the adult population, we believe our case adds value to the field of critical care medicine, coagulopathies, and forensic medicine.

Vol. 11(1)
Lincoln Medical Center (US), Northwell Health (US), Long Island University (US), New York University (US)
Openalex Percentile: Top 97%
Vascular Malformations and Hemangiomas
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