ArticleBritish journal of clinical pharmacology2022
Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.
Article in British journal of clinical pharmacology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 28 citations in OpenAlex.
- Sirolimus for vascular anomalies in the first year of life: a systematic review.Journal of perinatology : official journal of the California Perinatal Association · 2024Pooled it
- Sirolimus to the rescue: rapid therapeutic response in kaposiform hemangioendothelioma - case report and literature overview.BMC pediatrics · 2026Review
- Kaposiform hemangioendothelioma: Diagnosis and treatment.Pediatric investigation · 2026Review
- Sirolimus Treatment for Complex Fetal and Neonatal Vascular Anomalies: Emerging Evidence and Safety Considerations.Journal of clinical medicine · 2026Review
- Bleomycin electrosclerotherapy for kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in an adult.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2026Article
- Clinical effect evaluation of interventional embolization combined with pingyangmycin injection in the treatment of trunk and limbs Kaposi type hemangioendothelioma.American journal of cancer research · 2026Article
- The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma.European journal of pediatrics · 2025Article
- Corticosteroid-to-sirolimus sequential therapy: a case series of infantile kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon.Translational pediatrics · 2025Article
- Rapamycin suppresses small bowel adenocarcinoma HUTU 80 cells proliferation by inhibiting hypoxia-inducible factor-1α mediated metabolic reprogramming.World journal of gastrointestinal oncology · 2025Article
- Effective Sirolimus Use in Prenatal and Postnatal Management of Symptomatic Extensive Congenital Capillary-Lymphatic-Venous Malformations (CLVMs): A Report of Two Cases.Paediatric drugs · 2025Article
- Successful Management of Neonatal and Infantile Kaposiform Hemangioendothelioma with Low-Dose Sirolimus Therapy.Journal of pediatrics. Clinical practice · 2025Article
- Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: A Rare Diagnosis of Neck Mass in a Child.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2025Article
- Rapid response with good toleration of sirolimus for life-threatening neonatal lymphatic malformations.Pediatric investigation · 2023Article
- Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon in an infant girl.Clinical case reports · 2023Article
- Current concepts of vascular anomalies.Archives of craniofacial surgery · 2023Article
- Oral antibiotic prophylaxis for infection in patients with vascular anomalies receiving sirolimus treatment: a multicenter retrospective study.Orphanet journal of rare diseases · 2023Article
- Retroperitoneal kaposiform hemangioendothelioma with kasabach-merritt phenomenon in children: A case report and review of the literature.Frontiers in pediatrics · 2023Article
- Monitoring of sirolimus in the whole blood samples from pediatric patients with lymphatic anomalies.Open medicine (Warsaw, Poland) · 2023Article
- Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.British journal of clinical pharmacology · 2022Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
aimsManagement of kaposiform haemangioendotheliomas (KHE) with Kasabach-Merritt phenomenon is challenging in young infants who are subjected to developmental pharmacokinetic changes. Sirolimus, sometimes combined with corticosteroids, can be used as an effective treatment of KHE. Simultaneously, toxicities such as interstitial pneumonitis related to the use of sirolimus may be fatal. As infants have a very low CYP3-enzyme expression at birth, which rises during ageing, we hypothesize that a reduced metabolization of sirolimus might lead to high sirolimus serum levels and low dose may be sufficient without the side effects.
methodsA case series of 5 infants with kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon was analysed retrospectively. All infants were treated with sirolimus 0.2 mg/m
resultsIn all patients, low dose of sirolimus led to therapeutic sirolimus levels (4-6 ng/mL). All infants (aged 4 days-7 months) had a complete haematological response, without serious adverse events. In all patients, the Kasabach-Merritt phenomenon resolved, the coagulation profile normalized and tumour size reduction was seen.
conclusionLow-dose sirolimus treatment is safe for infants with kaposiform haemangioendothelioma and Kasabach-Merritt phenomenon. It is essential to realize that during the first months of life, metabolism is still developing and enzymes necessary to metabolise drugs like sirolimus still have to mature. To avoid toxic levels, the sirolimus dosage should be based on age and the associated pharmacological developments.
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