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Case Report: Kasabach-Merritt phenomenon in a 7-month-old Cambodian infant
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  • Vireak Prom,
  • Chinith Phauk,
  • Angkeabos Nhep,
  • Sandro Vento
Vireak Prom
University of Puthisastra

Corresponding Author:[email protected]

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Chinith Phauk
National Pediatric Hospital
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Angkeabos Nhep
National Pediatric Hospital
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Sandro Vento
University of Puthisastra
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Abstract

Introduction. Kasabach-Merritt phenomenon (KMP) is a rare condition associated with vascular tumors such as kaposiform hemangioendothelioma and tufted angioma; it can be life threatening, due to its consumptive coagulopathy. Thrombocytopenia and hypofibrinogenemia are characteristic of KMP, and anemia and raised d-dimer levels can also be detected. Here, we report a 7-month-old Cambodian with the condition. Case Presentation. The infant was admitted to the National Pediatric Hospital in Phnom Penh because of a mass on the right side of the neck that had been progressively enlarging. The patient had severe thrombocytopenia (8,000/µL), anemia (Hb 7.6g/dL) and reduced fibrinogen level (1.5g/L). CT scan and histology of the lesion confirmed a diagnosis of hemangioma. Kasabach-Merritt phenomenon was diagnosed, and the infant was treated with platelets and fresh frozen plasma infusions, prednisolone (2mg/kg/day) and propranolol (2.5mg/kg/day). After eight weeks of therapy, platelets raised to 102,000/µL. The infant developed Cushing’s syndrome after 6 months of treatment and prednisolone was scaled down to a maintenance dose of 0.5mg/kg/day. Fibrinogen levels went back to normal (2.14g/L) after seventeen months of treatment, and the tumor shrinked significantly. Conclusion. This case report shows that a combination of prednisolone and propranolol has been effective for KMP and kaposiform hemangioendothelioma. Timely recognition and treatment of Kasabach-Merritt phenomenon’s are essenti
10 Apr 2023Submitted to Clinical Case Reports
13 Apr 2023Submission Checks Completed
13 Apr 2023Assigned to Editor
17 Apr 2023Reviewer(s) Assigned
23 Apr 2023Review(s) Completed, Editorial Evaluation Pending
03 May 2023Editorial Decision: Revise Minor