ArticleOrphanet journal of rare diseases2021
Sirolimus in the treatment of kaposiform lymphangiomatosis.
Article in Orphanet journal of rare diseases, 2021. 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.
- Interventional embolization for hemorrhagic pleural effusion in an infant with kaposiform lymphangiomatosis: a case report.Frontiers in medicine · 2026Article
- Kaposiform lymphangiomatosis-the effects of long-term treatment with sirolimus: case series study and review of the literature.Frontiers in medicine · 2026Article
- Vascular malformations: from genetics to therapeutics.EMBO molecular medicine · 2026Review
- 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
- An Examination of Sirolimus's Role in Endothelial Cells of Kaposiform Haemangioendothelioma.Journal of cellular and molecular medicine · 2025Article
- Perioperative Considerations for a Patient with Juvenile Idiopathic Scoliosis and Kaposiform Lymphangiomatosis Undergoing Spinal Fusion: A Case Report.Journal of orthopaedic case reports · 2025Article
- Case Report: 3 cases of abdominal lesions with lower limb lymphedema-rare mutations in complex lymphatic anomalies.Frontiers in oncology · 2025Article
- Editorial: Vascular malformations: advancements, debates, and consensus.Frontiers in medicine · 2025Article
- Clinical characteristics and risk factors for acute abdomen in patients with abdominal lymphatic malformations.Journal of vascular surgery. Venous and lymphatic disorders · 2025Article
- Kaposiform Lymphangiomatosis as a Cause of Vaginal Bleeding & Discharge: A Case Report.Journal of pediatric and adolescent gynecology · 2024Article
- Updates in Genetic Testing for Head and Neck Vascular Anomalies.Oral and maxillofacial surgery clinics of North America · 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
- Oral antibiotic prophylaxis for infection in patients with vascular anomalies receiving sirolimus treatment: a multicenter retrospective study.Orphanet journal of rare diseases · 2023Article
- Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.Pediatric blood & cancer · 2023Review
- Refractory kaposiform lymphangiomatosis relieved by splenectomy.Frontiers in pediatrics · 2023Article
- Recurrent haemoptysis: a rare diagnosis of kaposiform lymphangiomatosis and review of literature.BMJ case reports · 2022Article
- Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.The American journal of surgical pathology · 2022Article
- Kaposiform Lymphangiomatosis in a Male Adolescent: A Clinical Challenge and the Role of Genetics.Journal of investigative medicine high impact case reportsArticle
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundKaposiform lymphangiomatosis (KLA), which is a new subtype of generalized lymphatic anomaly, is a rare disease with a poor prognosis. Currently, there is no standard treatment due to the poor understanding of KLA. Sirolimus, which is an inhibitor of mammalian target of rapamycin, has been shown to have promising potential in the treatment of complicated vascular anomalies. The aim of this study was to introduce the use of sirolimus for the treatment of KLA and to highlight the challenges of managing this refractory disease.
resultsWe reported seven patients with KLA who received sirolimus therapy in our center. Combined with previously reported cases, 58.3% achieved a partial response, 25.0% had stable disease, and 16.7% experienced disease progression. No severe sirolimus-related adverse events occurred during treatment.
conclusionsThis study suggests that sirolimus is currently an option for the treatment of KLA, and it is hoped that more specific therapies will be developed in the future. Rapid advances in basic science and clinical practice may facilitate the development of important new treatments for KLA.
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