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Development of Kasabach-Merritt phenomenon following vaccination: More than a coincidence?

Yi Ji1, Siyuan Chen2, Kaiying Yang1

  • 1Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.

The Journal of Dermatology
|August 18, 2018
PubMed

Insights

Kasabach-Merritt phenomenon (KMP) in kaposiform hemangioendothelioma (KHE) may be triggered by external factors, such as vaccination. Sirolimus effectively treated KHE with KMP, reducing lesion size and normalizing platelet counts.

Area of Science:

  • Vascular Anomalies
  • Pediatric Oncology
  • Hematology

Background:

  • Kasabach-Merritt phenomenon (KMP) is a rare complication associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA).
  • KMP involves thrombocytopenia, coagulopathy, and consumptive anemia, posing significant clinical challenges.

Observation:

  • Two pediatric patients with KHE in the right upper arm presented with rapid lesion enlargement and severe KMP following vaccination.
  • The clinical course suggested a potential link between extrinsic factors and KMP development in KHE.

Findings:

  • Sirolimus treatment led to rapid normalization of platelet levels in both patients.
  • Significant reduction in KHE lesion size was observed after sirolimus therapy.
  • These outcomes highlight the efficacy of sirolimus in managing KHE-associated KMP.

Implications:

  • The findings suggest that extrinsic factors, possibly including vaccinations, may precipitate KMP in individuals with pre-existing KHE.
  • Sirolimus emerges as a promising therapeutic option for KHE with KMP, offering both hematologic and structural improvements.
  • Further research is warranted to elucidate the precise mechanisms underlying KMP precipitation and the role of sirolimus in KHE treatment.
Keywords:
Kasabach-Merritt phenomenonkaposiform hemangioendotheliomaplatelet levelsirolimusvaccination

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