2008•Journal of Pediatric Hematology/OncologyRequires access

Case Report: POEMS Syndrome in Childhood

Esra Şevketoğlu, Sami Hatipoğlu, İnci Ayan, Öner Doğan, Bahar Salihoğlu

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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.

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What this paper is about

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.

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OpenAlex reports 10 citations for this work. Citation counts describe recorded attention and do not establish research quality.

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Available 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.

Key concepts: POEMS syndrome, Organomegaly, Medicine, Hepatosplenomegaly, Polyneuropathy, Monoclonal gammopathy, Dermatology, Anemia

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