Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Sep;55(9):1119-1124.
doi: 10.1111/jpc.14370. Epub 2019 Jan 2.

Vincristine and sirolimus in the treatment of kaposiform haemangioendothelioma

Suhua Peng  1 , Kaiying Yang  1 , Zhicheng Xu  1 , Siyuan Chen  2 , Yi Ji  1
Affiliations

Vincristine and sirolimus in the treatment of kaposiform haemangioendothelioma

Suhua Peng et al. J Paediatr Child Health. 2019 Sep.

Abstract

Aim: Kaposiform haemangioendothelioma (KHE) is a rare, potentially life-threatening vascular tumour that is often associated with thrombocytopenia and coagulopathy, known as the Kasabach-Merritt phenomenon (KMP). Because of the rarity and complexity of KHE, the optimal paradigm for treating KHE has yet to be elucidated. We aim to assess the efficacy and safety of vincristine and sirolimus for the treatment of KHE.

Methods: A comprehensive review of the literature was conducted from January 1993 to June 2018. A total of 15 studies were selected for the meta-analysis. Five studies included 75 individuals and reported the response and side effects to vincristine in the treatment of KHE with or without KMP. A total of 10 studies that included 127 individuals reported the response and safety of sirolimus for treating KHE with or without KMP.

Results: The pooled odds ratio (OR) for the effectiveness of vincristine was 0.72. The pooled OR for the effectiveness of sirolimus was 0.91. The side effects associated with vincristine during the treatment included neuropathy, abdominal pain, loss of appetite and mild elevations of aspartate aminotransferase (AST) and alanine aminotransferase (ALT). The side effects associated with sirolimus therapy included bronchitis; lymphopenia; elevated AST, ALT and platelets; hyperlipidaemia; opportunistic infection; mild reversible leukopenia; mucositis; fever; pain and skin rash/vomiting and diarrhoea.

Conclusions: This systematic review showed a high efficacy of vincristine and sirolimus in the treatment of KHE. Based on the available data in the literature, it appears that sirolimus is potentially an efficacious and safe treatment option for KHE. Further randomised, controlled trials are recommended.

Keywords: Kasabach-Merritt phenomenon; kaposiform haemangioendothelioma; sirolimus; treatment; vincristine.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Enjolras O, Wassef M, Mazoyer E et al. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J. Pediatr. 1997; 130: 631-40.
    1. Enjolras O, Mulliken JB, Wassef M et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J. Am. Acad. Dermatol. 2000; 42: 225-35.
    1. Haisley-Royster C, Enjolras O, Frieden IJ et al. Kasabach-Merritt phenomenon: A retrospective study of treatment with vincristine. J. Pediatr. Hematol. Oncol. 2002; 24: 459-62.
    1. Drucker AM, Pope E, Mahant S, Weinstein M. Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma. J. Cutan. Med. Surg. 2009; 13: 155-9.
    1. Lopez V, Marti N, Pereda C et al. Successful management of kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine. Pediatr. Dermatol. 2009; 26: 365-6.

Publication types

Supplementary concepts

LinkOut - more resources