Case presentation
A 50-year-old woman presented with a 5-month history of abdominal distension, lower limb edema and dyspnea, accompanied by numbness and weakness of hands and feet. She had no history of hepatitis, tuberculosis and diabetes mellitus. Skin pigmentation was observed, especially for the hand (Figure 1A). Radiograph of bilateral femur (Figure 1B-C) demonstrated extensive cortical thickening and periosteal proliferation (arrows). Auxiliary examinations revealed cervical lymphadenopathy (Castleman disease comfirmed by biopsy), hepatomegaly, splenomegaly, ascites, pulmonary hypertension and embolism. Laboratory findings indicated monoclonal plasma cell-proliferative disorder (IgA-λ). The diagnosis of POEMS syndrome was made. The patient received treatment with bortezomib and corticosteroid, with symptoms improved significantly including abdominal distension and suffocation, but the skin changes and bone lesions had not regressed.
POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, monoclonal plasma cells disorder, the presence of Castleman disease, organomegaly, sclerotic bone lesions and skin changes.
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POEMS syndrome is associated with predominantly osteosclerotic bone lesions, which can have mixed sclerotic-lytic components, and are also referred to as osteosclerotic myeloma.2
Lesions are often small and multiple, which are commonly found in the pelvis, thoracic and lumbar vertebrae, and ribs, and also occur in the scapula, clavicle, sternum, skull and long bones.3
Unlike the previous studies, symmetrical solid periosteal reactions were revealed in POEMS syndrome. Periosteum contains mesenchymal progenitor cells differentiating to osteoblasts. POEMS syndrome is associated with plasma cell dyscrasia and elevated serum vascular endothelial growth factor (VEGF).3
VEGF might enhance bone morphogenetic protein-2 (BMP-2)-induced bone formation through modulation of angiogenesis.Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Declaration of Competing Interest
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this manuscript.
References
- POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management.Am J Hematol. 2021; 96: 872-888
- POEMS syndrome: clinical update.J Neurol. 2019; 266: 268-277
- Detection of bone lesions by CT in POEMS syndrome.Intern Med. 2011; 50: 1393-1396
Article info
Publication history
Published online: September 06, 2022
Accepted:
August 31,
2022
Received:
March 16,
2022
Identification
Copyright
© 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
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