Abstract
Objective. Bone lesion is crucial for diagnosing and management of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome, a rare plasma cell disorder. This study is to compare the effectiveness of X-ray skeletal survey (SS) and computed tomography (CT) for detecting bone lesions in Chinese POEMS syndrome patients, and to investigate the relationship between bone lesion features and serum markers.Methods. SS and chest/abdomen/pelvic CT images of 38 Chinese patients (26 males, 12 females, aged 21–70 years) with POEMS syndrome recruited at our medical center between January 2013 and January 2015 were retrospectively analyzed. Bone lesions identified by CT were further categorized according to the size (<5 mm, 5–10 mm, >10 mm) and appearance (osteosclerotic, lytic, mixed). The percentage of plasma cells in bone marrow smears, type of immunoglobulin, platelet (Plt), and levels of serum bone metabolic markers and inflammatory factors including alkaline phosphatase (ALP), calcium, phosphate, parathyroid hormone (PTH), beta-isomerized C-telopeptide (β-CTx), vascular endothelial growth factor (VEGF), and interleukin (IL)-6 levels were also recorded.Results. Of the 38 POEMS syndrome patients, the immunoglobulin heavy chain isotypes were IgA in 25 patients (65.8%; 25/38) and IgG in 13 patients (34.2%; 13/38), and the light chain isotypes were λ in 35 patients (92.1%; 35/38) and κ in 3 patients (7.9%; 3/38). There were 23 patients with thrombocytosis. More patients with bone lesions were detected by CT than by SS (97.4% vs. 86.8%). The most commonly affected location was the pelvis (89.5%), followed by the spine, clavicle/scapula/sternum/ribs, skull, and long bones. Of the 38 POEMS syndrome patients, 35 (94.6%) had osteosclerotic and 32 (86.5%) had mixed lesions. Osteosclerotic lesions were typically scattered, variable in size, and plaque-like, whereas mixed lesions were pouch-shaped or soup bubble-like with a clear sclerotic margin and were generally larger. Although the majority of bone lesions were small in size, 23 (62.2%) had at least one lesion >10 mm. There was no correlation between serum marker levels and bone lesion patterns after Bonferroni correction (all P > 0.001).Conclusions. CT is more sensitive and accurate than SS in detecting bone lesions in POEMS syndrome.
Highlights
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome is a rare plasma cell disorder
Both mandatory major criteria, one of the three major criteria (Castleman disease, osterosclerotic lesions, elevated serum levels of plasma vascular endothelial growth factor (VEGF)), and at least one of the six minor criteria are required for a definitive diagnosis
Inclusion criteria were as follows: (a) patients met the 2007 diagnostic criteria for POEMS syndrome; (b) patients underwent an X-ray skeletal survey (SS) for bone lesions; (c) a chest/abdomen/pelvic computed tomography (CT) scan was performed within 1 month of SS; and (d) all SS and CT examinations were completed before treatment
Summary
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome is a rare plasma cell disorder. Diagnosis is challenging since the syndrome is rare and can be mistaken for other disorders. Both mandatory major criteria (polyneuropathy and monoclonal plasma cell proliferative disorder), one of the three major criteria (Castleman disease, osterosclerotic lesions, elevated serum levels of plasma vascular endothelial growth factor (VEGF)), and at least one of the six minor criteria are required for a definitive diagnosis. Bone lesions are important for diagnosing and evaluating POEMS syndrome, but are crucial for administering appropriate treatments (Dispenzieri, 2014). Imaging is indispensable for accurately diagnosing POEMS syndrome and determining the treatment approach that is required. Most studies are case reports or have examined a small patient sample (Glazebrook et al, 2015; Shi et al, 2015; Shibuya et al, 2011), in part due to the rarity and complexity of this disorder
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