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[Kasabach-Merritt syndrome in infants. 2 cases]
Abstract:
Two infants presenting with Kasabach-Merritt syndrome point out several points: the hemangioma may look innocuous with small proportions. The coagulopathy may be represented by a simple thrombopenia or an intravascular coagulation (PDF, ethanol test) of bad prognosis. A review of 150 observations of Kasabach-Merritt syndrome suggests a simple therapeutic approach: corticotherapy (2 to 4 mg/kg) for 2 to 4 weeks or, if failure, antiaggregant drugs. Radiotherapy is proposed in cases of emergency. Heparin and antifibrinolytic treatments are restricted to cases with severe coagulopathy.
Insights
Kasabach-Merritt syndrome in infants can present with subtle hemangiomas and serious coagulopathy. Treatment involves corticotherapy or antiplatelet drugs, with radiotherapy for emergencies.
Area of Science:
- Pediatric Hematology
- Vascular Anomalies
- Coagulation Disorders
Background:
- Kasabach-Merritt syndrome (KMS) is a rare condition characterized by a vascular tumor and severe coagulopathy.
- Infantile hemangiomas associated with KMS can vary in size and presentation.
- Coagulopathy in KMS can range from simple thrombocytopenia to disseminated intravascular coagulation (DIC).
Observation:
- Two infant cases highlighted the deceptive appearance of hemangiomas in KMS.
- The severity of coagulopathy, indicated by thrombocytopenia or DIC, is a critical prognostic factor.
- A review of 150 KMS cases informed a therapeutic strategy.
Findings:
- A straightforward therapeutic approach for KMS includes corticotherapy (2-4 mg/kg for 2-4 weeks).
- Antiplatelet agents are recommended if initial corticotherapy fails.
- Radiotherapy is reserved for emergency situations.
Implications:
- This study proposes a simplified treatment algorithm for Kasabach-Merritt syndrome.
- Early recognition of coagulopathy is crucial for effective management.
- The findings guide clinicians in managing this complex pediatric condition.