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Case Report: POEMS Syndrome in Childhood

Sevketoglu, Esra MD*; Hatipoglu, Sami MD*; Ayan, Inci MD; Dogan, Oner MD; Salihoglu, Bahar MD*

Journal of Pediatric Hematology/Oncology: March 2008 - Volume 30 - Issue 3 - pp 235-238
doi: 10.1097/MPH.0b013e31815f88da
Original Articles

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.

*Department of Pediatrics, Bakirkoy Dr. Sadi Konuk Research and Training Hospital

Department of Pediatric Hematology Oncology, Istanbul University, Institute of Oncology

Department of Pathology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkiye

Reprints: Esra Sevketoglu, MD, Department of Pediatrics, Bakirkoy Dr Sadi Konuk Research and Training Hospital, Istanbul, Turkiye (e-mail: caglaes@yahoo.com).

Received for publication August 15, 2007; accepted October 20, 2007

© 2008 Lippincott Williams & Wilkins, Inc.