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

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COŞKUN Ç., AKSU T., GÜMRÜK F., ÜNAL Ş.

Turkish Journal of Pediatrics, vol.65, no.1, pp.124-128, 2023 (SCI-Expanded) identifier identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 65 Issue: 1
  • Publication Date: 2023
  • Doi Number: 10.24953/turkjped.2022.254
  • Journal Name: Turkish Journal of Pediatrics
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.124-128
  • Keywords: Klippel-Trenaunay syndrome, chronic myeloid leukemia, overgrowth syndrome, PIK3CA gene, vascular malformation, CVLM syndrome
  • Hacettepe University Affiliated: Yes

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.