ArticlePediatric blood & cancer2020
Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels.
Article in Pediatric blood & cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 26 citations in OpenAlex.
- RAS Inhibitor RMC-7977 Blocks Vascular Overgrowth of NRASJournal of cellular and molecular medicine · 2026Article
- Long-term low-dose sirolimus therapy and successful discontinuation in an adult with kaposiform lymphangiomatosis and disseminated intravascular coagulation: a case report.Frontiers in medicine · 2026Article
- Trametinib normalizes angiopoietin-2 levels and successfully treats kaposiform lymphangiomatosis.Journal of vascular anomalies · 2025Article
- Complex lymphatic anomalies.Pediatric radiology · 2025Review
- Perioperative Considerations for a Patient with Juvenile Idiopathic Scoliosis and Kaposiform Lymphangiomatosis Undergoing Spinal Fusion: A Case Report.Journal of orthopaedic case reports · 2025Article
- RAS Pathway Mutations and Therapeutics in Vascular Anomalies.Pediatric blood & cancer · 2025Review
- NRASPediatric blood & cancer · 2024Article
- How we use angiopoietin-2 in the diagnosis and management of vascular anomalies.Pediatric blood & cancer · 2024Article
- Capillary malformations.The Journal of clinical investigation · 2024Review
- Targeted therapies for vascular malformations.Frontiers in medicine · 2024Review
- Treatment of severe Kaposiform lymphangiomatosis positive for NRAS mutation by MEK inhibition.Pediatric research · 2023Article
- Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.Pediatric blood & cancer · 2023Review
- Refractory kaposiform lymphangiomatosis relieved by splenectomy.Frontiers in pediatrics · 2023Article
- NRASAngiogenesis · 2022Article
- How we approach the use of sirolimus and new agents: Medical therapy to treat vascular anomalies.Pediatric blood & cancer · 2022Article
- Osteopathy in Complex Lymphatic Anomalies.International journal of molecular sciences · 2022Review
- Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.The American journal of surgical pathology · 2022Article
- Kaposiform lymphangiomatosis presenting with a Group ABMJ case reports · 2022Article
- Sirolimus in the treatment of kaposiform lymphangiomatosis.Orphanet journal of rare diseases · 2021Article
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Authors and funding
8 authors at 5 institutions in 1 country.
Funding
Abstract
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.
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