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Published on: September 20, 2018
Kasabach-Merritt syndrome: a case report
S A Bolde1, S S Shete, S S Dantkale
1Department of Pathology, Dr. VM Medical College, Solapur.
Indian Journal of Pathology & Microbiology
|June 9, 2006
Summary
Kasabach-Meritt syndrome, a rare condition involving thrombocytopenia and coagulopathy, was observed in a neonate with a rapidly enlarging hemangioma. Despite intensive care, the infant succumbed to complications including hemorrhage and septicemia.
Area of Science:
- Paediatric Medicine
- Oncology
- Hematology
Background:
- Kasabach-Merritt syndrome (KMS) is a rare clinical condition characterized by thrombocytopenia, hemolytic anemia, and consumptive coagulopathy.
- It is often associated with rapidly growing vascular tumors, primarily infantile hemangiomas.
Observation:
- A 5-day-old male infant presented with symptoms consistent with Kasabach-Merritt syndrome.
- Clinical manifestations included ecchymotic patches and umbilical stump bleeding.
- The infant exhibited severe thrombocytopenia and consumptive coagulopathy, complicated by septicemia.
Findings:
- The infant's condition rapidly deteriorated, leading to precipitous hemorrhage and expiration.
- Autopsy confirmed a retroperitoneal lesion identified as kaposiform hemangioendothelioma, a type of vascular tumor.
Implications:
- This case highlights the critical and often fatal nature of Kasabach-Merritt syndrome in neonates.
- Early recognition and management of associated vascular tumors are crucial for improving outcomes.
- Kaposiform hemangioendothelioma should be considered in the differential diagnosis of neonatal coagulopathies.
