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Published on: June 5, 2019
Kasabach-Merritt syndrome with profound platelet support
J D Henley1, C F Danielson, S S Rothenberger
1Department of Pathology, Indiana University Medical Center, School of Medicine, Indianapolis 46202-5283.
Insights
Kasabach-Merritt Syndrome involves severe thrombocytopenia (low platelets) in infants with giant hemangiomas. This case highlights extreme platelet transfusion needs in a prolonged, life-threatening infant case.
Area of Science:
- Pediatric Hematology
- Vascular Anomalies
Background:
- Kasabach-Merritt Syndrome (KMS) is a rare condition characterized by severe thrombocytopenia associated with infantile hemangiomas.
- The associated thrombocytopenia can lead to significant morbidity and mortality due to bleeding complications.
Abstract:
The Kasabach-Merritt Syndrome describes thrombocytopenia occurring in patients with giant hemangiomata. The resultant thrombocytopenia may be profound and occasionally even life-threatening. An 11-month-old infant with prolonged thrombocytopenia whose course was complicated by recurrent hemorrhaging requiring intense platelet transfusions is reported. During her 19-month hospitalization she received 6,622 platelet concentrates. This represents the most extensive platelet support ever given to an infant with this syndrome.
