Kasabach-Merritt phenomenon with cellulitis in infant

Area of Science:

• Pediatric Hematology
• Vascular Anomalies
• Coagulopathy

Background:

• Kasabach-Merritt phenomenon (KMP) is a severe coagulopathy associated with specific vascular tumors, namely kaposiform hemangioendothelioma (KHE) and tufted angioma.
• This condition carries a high mortality rate and commonly affects the trunk, limbs, and retroperitoneum, presenting with symptoms like purpura, edema, and ecchymosis.

Observation:

• A 9-month-old boy presented with a rapidly enlarging violaceous leg tumor present since birth, leading to limb discrepancy.
• The tumor worsened with alternative massage treatments, resulting in bruising and wounds.
• Clinical presentation included severe thrombocytopenia, normocytic anemia, and elevated D-dimer levels, indicative of KMP with superimposed cellulitis.

Findings:

• The patient was diagnosed with Kasabach-Merritt phenomenon (KMP) complicated by cellulitis.
• Laboratory findings revealed severe thrombocytopenia and elevated D-dimer, confirming consumptive coagulopathy.
• Successful treatment involved a combination of oral corticosteroids, propranolol, and antibiotics.

Implications:

• This case highlights the importance of early clinical and laboratory diagnosis of KMP.
• Combination therapy using corticosteroids and propranolol demonstrates a viable treatment strategy for KMP.
• Prompt management is crucial for improving prognosis in infants with KMP and associated vascular lesions.