Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma.
Pediatr Dermatol
; 35(5): 635-638, 2018 Sep.
Article
em En
| MEDLINE
| ID: mdl-29999213
ABSTRACT
BACKGROUND:
Sirolimus has been used to manage various complex vascular anomalies. Kaposiform hemangioendothelioma and tufted angioma may develop Kasabach-Merritt phenomenon in infancy.METHODS:
We retrospectively reviewed the clinical and laboratory data of eight patients with kaposiform hemangioendothelioma and tufted angioma who were initially treated using oral sirolimus in our center, including six with Kasabach-Merritt phenomenon.RESULTS:
Five girls and three boys seen between September 2012 and March 2015 were included. Age at initiation of sirolimus ranged from 30 days to 14 weeks (mean±SD 8.6 ± 3.5 weeks). Six of these eight patients had kaposiform hemangioendothelioma, and two had a tufted angioma. Platelet count before start of oral sirolimus ranged from 5 × 109 /L to 189 × 109 /L ((78.8 ± 65.2)×109 /L) and fibrinogen level from 68 to 215 mg/dL (123.1 ± 50.5 mg/dL). All patients received standard doses of sirolimus (0.05 mg/kg orally, twice daily) as initial therapy. All patients with thrombocytopenia or hypofibrinogenemia reached a normal platelet count and a normal fibrinogen level within 3 to 4 weeks after sirolimus treatment. Length of treatment ranged from 12 to 79 weeks (39.9 ± 15.3 weeks). Two patients developed grade 2 oral mucositis during treatment.CONCLUSION:
Sirolimus as first-line therapy shows great promise in the treatment of kaposiform hemangioendothelioma and tufted angioma.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Sarcoma de Kaposi
/
Neoplasias Cutâneas
/
Sirolimo
/
Síndrome de Kasabach-Merritt
/
Hemangioendotelioma
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Hemangioma
/
Imunossupressores
Tipo de estudo:
Observational_studies
/
Risk_factors_studies
Limite:
Female
/
Humans
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Infant
/
Male
Idioma:
En
Revista:
Pediatr Dermatol
Ano de publicação:
2018
Tipo de documento:
Article
País de afiliação:
China