Case report: POEMS syndrome in childhood

J Pediatr Hematol Oncol. 2008 Mar;30(3):235-8. doi: 10.1097/MPH.0b013e31815f88da.

Abstract

POEMS syndrome is a rare multisystem disorder, which is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We report a 15-year-old girl with POEMS syndrome, who developed growth retardation, delayed puberty, gradually increasing abdominal distention, brown skin pigmentation, hypogonadism, hepatosplenomegaly, lympadenomegaly, monoclonal gammopathy, and anemia. The patient dramatically responded to pulse steroid therapy. To our knowledge, this patient is one of the youngest reported cases of POEMS syndrome.

Publication types

  • Case Reports

MeSH terms

  • Abdomen / pathology
  • Adolescent
  • Anemia / diagnosis
  • Diagnosis, Differential
  • Female
  • Growth Disorders / diagnosis
  • Hematopoietic Stem Cell Transplantation*
  • Hepatomegaly / diagnosis
  • Humans
  • Hypogonadism / diagnosis
  • POEMS Syndrome / diagnosis*
  • POEMS Syndrome / therapy*
  • Paraproteinemias / diagnosis
  • Pigmentation Disorders / diagnosis
  • Pigmentation Disorders / therapy
  • Predictive Value of Tests
  • Puberty, Delayed / diagnosis
  • Steroids / therapeutic use*
  • Treatment Outcome

Substances

  • Steroids