Kasabach-Merritt phenomenon: a single centre experience

Area of Science:

• Pediatric Hematology
• Vascular Anomalies
• Pediatric Oncology

Background:

• Kasabach-Merritt phenomenon (KMP) is a rare, life-threatening condition characterized by severe thrombocytopenia and coagulopathy.
• Optimal management strategies for KMP remain undefined, necessitating further clinical investigation.

Purpose of the Study:

• To review the institutional experience in managing Kasabach-Merritt phenomenon (KMP) in pediatric patients.
• To evaluate the efficacy and outcomes of various therapeutic modalities employed in KMP management.

Main Methods:

• A retrospective chart review was conducted on all pediatric patients diagnosed with KMP over an 18-year period.
• Data collected included clinical presentation, treatment interventions, laboratory values, and patient outcomes.

Main Results:

• Fifteen patients with KMP presented with profound thrombocytopenia and hypofibrinogenemia; 50% experienced bleeding, and 20% had cardiac failure.
• Corticosteroids were administered to all patients. Five patients responded to steroids alone. Other treatments included vincristine, interferon, and embolization, with varying response rates.
• The mean time to platelet recovery above 20 x 10(9)/L was 56 days, and above 150 x 10(9)/L was 88 days. Thrombotic complications occurred in 7% of patients.

Conclusions:

• Kasabach-Merritt phenomenon (KMP) is a rare condition associated with significant morbidity and mortality.
• Effective management necessitates a multidisciplinary team approach and the development of consensus-based treatment guidelines.
• While corticosteroids are a primary treatment, combination therapies and interventions like embolization may be required for optimal outcomes.