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

Janet Crane, Jackie Manfredo, Elisa Boscolo, Mara Coyan, Clifford Takemoto, Maxim Itkin, Denise M Adams, Timothy D Le Cras

Pediatric blood & cancer 2020 Sep

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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. © 2020 Wiley Periodicals, Inc.

Citation

Janet Crane, Jackie Manfredo, Elisa Boscolo, Mara Coyan, Clifford Takemoto, Maxim Itkin, Denise M Adams, Timothy D Le Cras. Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels. Pediatric blood & cancer. 2020 Sep;67(9):e28529