Shu Ye,Huang Wenya,Luo Yongqi,et al.Summary of clinical features and treatment protocols of 10 infants with Kasabach-Merritt syndrome[J].Journal of Clinical Pediatric Surgery,,18():775-778.[doi:10.3969/j.issn.1671-6353.2019.09.014]
Summary of clinical features and treatment protocols of 10 infants with Kasabach-Merritt syndrome
- Keywords:
- Kasabach-Merritt Syndrome; Hemangioma; Pathological Conditions; Signs and Symptoms; Therapy; Infant
- CLC:
- R726;R732.2
- Abstract:
- Objective To summarize the clinical treatments of infantile Kasabach-Merritt syndrome.Methods The clinical data were retrospectively analyzed for 10 hospitalized children with Kamei syndrome from January 2014 to September 2018.The treatment and treatment outcomes were summarized.Results All 10 cases fulfilled the diagnostic criteria of Kamei syndrome and occurred within 1 year post-birth,including 2 newborns.There were 5 boys and 5 girls.Hemangioma was located at head & face (n=6),trunk (n=2) and buttocks & vulva (n=2).In 4 cases,2 cases improved after treating with methylprednisolone while 2 cases were unhealed; 4 cases were treated with methylprednisolone and bleomycin embolization; 2 cases received methylprednisolone and vincristine.Conclusion With a young age of onset,Kasabach-Merritt syndrome has an aggressive course.Glucocorticoids are preferred for drug therapy and some patients with steroid resistance may be treated with steroid combined vincristine or bleomycin embolizayim.
References:
1 Dasgupta R,Fishman SJ.ISSVA classification[J].Semin Pediatr Surg,2014,23(4):158-161.DOI:10.1053/j.sempedsurg.2014.06.016.
2 Fernandez Y,Bernabeu-Wittel M,Garcia-Morillo JS.Kaposiform hemangioendothelioma[J].Eur J Intern Med,2009,20(2):106-113.DOI:10.1016/j.ejim.2008.06.008.
3 廖清奎.血液病基础与临床[M].北京:人民卫生出版社,2001:702-703. Liao QK.Basic and Clinical Hematology[M].Beijing:People’s Medical Publishing House,2001:702-703.
4 夏红萍,何正娟,朱建幸,等.卡-梅综合征10例临床分析[J].中国实用儿科杂志,2011,26(2):125-127. Xia HP,He ZJ,Zhu JX,et al.Clinical analysis of 10 cases with Kasabach-Merritt syndrome[J].Chinese Journal of Practical Pediatrics,2011,26(2):125-127.
5 Drolet BA,Brandao LR,Chiu YE,et al.Consensus-derived practice standards plan for complicated Kaposiforrn hemangioendothelioma[J].J Pediatr,2013,163(1):285-291.DOI:10.1016/j.jpeds.2013.03.080.
6 Kim T,Roh MR,Cho S,et al.Kasabach-merritt syndrome arising from tufted angioma successfully treated with systemic corticosteroid[J].Ann Dermatol,2010,22(4):426-430.DOI:10.5021/ad.2010.22.4.426.
7 Jiang RS,Hu IL.Successful treatment of Kasabach Merritt syndrome arising from kaposiform hemangioendothelioma by systemic corticosteroid therapy and surgery[J].Int J Clin Oncol,2012,17(5):512-516.DOI:10.1007/s10147-011-0321-4.
8 Tan X,Chen M,Zhang J,et al.Treatment of corticosteroid-resistant vascular tumors associated with the Kasabach-Merritt phenomenon in infants:an approach with transcather arterial emblization plus vincristine therapy[J].J Vasc Interv Radiol,2016,27(4):569-575.DOI:10.1016/j.jvir.2015.12.012.
9 Wang Z,Li K,Yao W,et al.Steroid-resistant kaposiform hemangioendothelioma:A retrospective study of 37 patients treated with vincristine and long-term follow-up[J].Pediatr Blood Cancer,2015,62(4):577-580.DOI:10.1002/pbc.25296.
10 Fahrtash F,Mccahon E,Arbuckle S.Successful treatment of kaposifon hemangioendothelioma and tufted angioma with vincristine[J].J Pediatr Hematol Oncol,2010,32(6):506-510.DOI:10.1097/MPH.0b013e3181e001a9.
11 孙艳纯,郑姗,李凯,等.长春新碱治疗血管瘤伴血小板减少综合征的疗效探讨[J].临床小儿外科杂志,2009,8(5):3-5.DOI:10.3969/j.issn.1671-6353.2009.05.002. Sun YC,Zheng S,Li K,et al.Initial experience of treatment of Kasabach-Merritt syndrome with vincristine[J].J Clin Ped Sur,2009,8(5):3-5.DOI:10.3969/j.issn.1671-6353.2009.05.002.
12 Mora E,Smith EM,Donohoe C,et al.Vineristine-induced peripheral neuropathy in pediatric cancer patients[J].Am J Cancer Res,2016,6(11):2416-2430.
Memo
收稿日期:2018-10-19。
基金项目:湖南省医药卫生科研计划项目(编号:B2016039)
通讯作者:汤建萍,Email:jpingtang@126.com