The Turkish Journal of Pediatrics 2023 , Vol 65 , Num 1
A rare case of Klippel-Trenaunay syndrome presenting with chronic myeloid leukemia
Çağrı Coşkun 1 ,Tekin Aksu 1 ,Fatma Gümrük 1 ,Şule Ünal 1
1 Department of Pediatric Hematology, Hacettepe University Faculty of Medicine, Ankara, Türkiye DOI : 10.24953/turkjped.2022.254 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. Keywords : Klippel-Trenaunay syndrome, chronic myeloid leukemia, overgrowth syndrome, PIK3CA gene, vascular malformation, CVLM syndrome

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