Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels

Pediatr Blood Cancer. 2020 Sep;67(9):e28529. doi: 10.1002/pbc.28529. Epub 2020 Jul 7.


Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. We report a 7-year-old male child with easy bruising, inguinal swelling, and consumptive coagulopathy, diagnosed with KLA. A multimodal treatment regimen of prednisone, sirolimus, vincristine, and adjunctive zoledronate was used. Plasma ANG2 levels were highly elevated at diagnosis but decreased during treatment. The patient showed significant clinical improvement over a 38-month period and normalization of ANG2 levels correlated with resolution of the coagulopathy.

Keywords: angiogenesis; vascular malformations; vincristine.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Angiopoietin-2 / blood*
  • Child
  • Combined Modality Therapy
  • Hemangioendothelioma / blood
  • Hemangioendothelioma / pathology
  • Hemangioendothelioma / therapy*
  • Humans
  • Kasabach-Merritt Syndrome / blood
  • Kasabach-Merritt Syndrome / pathology
  • Kasabach-Merritt Syndrome / therapy*
  • Male
  • Prognosis
  • Sarcoma, Kaposi / blood
  • Sarcoma, Kaposi / pathology
  • Sarcoma, Kaposi / therapy*
  • Thrombosis / blood
  • Thrombosis / pathology
  • Thrombosis / prevention & control*


  • ANGPT2 protein, human
  • Angiopoietin-2

Supplementary concepts

  • Kaposiform Hemangioendothelioma