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.
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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.
