[A case of Epstein-Barr virus-related smooth muscle tumor secondary to Wiskott-Aldrich syndrome treated by allogeneic hematopoietic stem cell transplantation]

L T Xuan; S Q Feng; J G Yang; J H Li; Z X Zhang; R Liu

doi:10.3760/cma.j.cn112140-20220907-00786

[A case of Epstein-Barr virus-related smooth muscle tumor secondary to Wiskott-Aldrich syndrome treated by allogeneic hematopoietic stem cell transplantation]

Zhonghua Er Ke Za Zhi. 2023 May 2;61(5):464-466. doi: 10.3760/cma.j.cn112140-20220907-00786.

[Article in Chinese]

Authors

L T Xuan¹, S Q Feng¹, J G Yang², J H Li¹, Z X Zhang¹, R Liu¹

Affiliations

¹ Department of Hematology, Children's Hospital,Capital Institute of Pediatrics, Beijing 100020, China.
² Department of Nuclear Medicine, Beijing Friendship Hospital, Capital Medical University,Beijing 100050, China.

PMID: 37096268
DOI: 10.3760/cma.j.cn112140-20220907-00786

Abstract

患儿男，1月龄出现湿疹，4月龄时发现血小板减少，1岁5月龄全外显子测序发现WAS基因半合子突变，WAS蛋白阴性，诊断Wiskott-Aldrich综合征（WAS），间断予糖皮质激素及丙种球蛋白等治疗5年余。6岁余出现肝脏肿大，影像学提示为肝脏多发结节，肺、脾、淋巴结同时受累，完善肝脏结节穿刺活检，诊断为EB病毒相关平滑肌肉瘤；7岁余予后置环磷酰胺方案单倍体异基因造血干细胞移植治疗，随访1年，患儿免疫功能恢复，肿瘤得到控制。.

Publication types

Case Reports

MeSH terms

Epstein-Barr Virus Infections* / complications
Hematopoietic Stem Cell Transplantation*
Herpesvirus 4, Human
Humans
Smooth Muscle Tumor* / complications
Smooth Muscle Tumor* / therapy
Wiskott-Aldrich Syndrome*