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一例罕见的 Klippel-Trenaunay 综合征合并慢性髓性白血病。

A rare case of Klippel-Trenaunay syndrome presenting with chronic myeloid leukemia.

机构信息

Department of Pediatric Hematology, Hacettepe University Faculty of Medicine, Ankara, Türkiye.

出版信息

Turk J Pediatr. 2023;65(1):124-128. doi: 10.24953/turkjped.2022.254.

Abstract

BACKGROUND

Klippel-Trenaunay syndrome (KTS) is an overgrowth syndrome associated with capillary/venous/ lymphatic malformations with limb hypertrophy and cancer risk. Various cancers, mostly Wilms tumor, have been reported in patients with KTS, but not leukemia. Chronic myeloid leukemia (CML) is also a rare disease in children, where there is no known disease or syndrome to predispose to CML.

CASE

We report a case of CML incidentally diagnosed in a child with KTS when he was bleeding from surgery of the left groin for vascular malformation.

CONCLUSIONS

This case reflects the variety of cancer types that may accompany KTS and provides information about CML prognosis in such patients.

摘要

背景

Klippel-Trenaunay 综合征(KTS)是一种与肢体肥大和癌症风险相关的毛细血管/静脉/淋巴管畸形过度生长综合征。患有 KTS 的患者已报告发生各种癌症,主要是肾母细胞瘤,但没有白血病。慢性髓细胞白血病(CML)在儿童中也是一种罕见疾病,目前尚无已知的疾病或综合征易患 CML。

病例

我们报告了一例 KTS 患儿在因左侧腹股沟血管畸形手术出血时意外诊断为 CML 的病例。

结论

该病例反映了可能伴随 KTS 的多种癌症类型,并提供了有关此类患者 CML 预后的信息。

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