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Kasabach-Merritt phenomenon

Khalid A Al-Mazrou1, Mark A Richardson

  • 1Department of ORL, King Saud University and University Hospitals, Riyadh, Kingdom of Saudi Arabia. kmazrou@ksu.edu.sa

Saudi Medical Journal
|August 30, 2005
PubMed

Insights

Kasabach-Merritt phenomenon (KMP), a serious vascular lesion complication, can be effectively treated. Mega-dose corticosteroids resolved coagulopathy and improved the vascular lesion in a pediatric case.

Area of Science:

  • Vascular Medicine
  • Pediatric Hematology
  • Oncology

Background:

  • Kasabach-Merritt phenomenon (KMP) is a rare, life-threatening coagulopathy associated with vascular tumors.
  • KMP presents with thrombocytopenia, microangiopathic hemolytic anemia, and disseminated intravascular coagulopathy.
  • Traditional treatments include medication, radiation, embolization, and surgery, with corticosteroids as a mainstay.

Observation:

  • A 4-month-old infant presented with an extensive vascular lesion in the head and neck.
  • The infant also exhibited symptoms consistent with Kasabach-Merritt phenomenon.
  • Screening for KMP is critical due to its severity.

Findings:

  • The patient was treated with mega-dose corticosteroids.
  • The corticosteroid treatment led to the resolution of the infant's coagulopathy.
  • Significant improvement in the size and extent of the vascular lesion was observed.

Implications:

  • Mega-dose corticosteroids represent a viable and effective treatment for Kasabach-Merritt phenomenon in infants.
  • This approach offers a less invasive option compared to surgery or embolization.
  • Further research into optimal corticosteroid dosing and long-term outcomes is warranted.

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