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Clinical and Experimental Immunology May 2024Serum B-cell maturation antigen (sBCMA) levels can serve as a sensitive biomarker in multiple myeloma (MM). In the research setting, sBCMA levels can be accurately...
Serum B-cell maturation antigen (sBCMA) levels can serve as a sensitive biomarker in multiple myeloma (MM). In the research setting, sBCMA levels can be accurately detected by enzyme-linked immunosorbent assay (ELISA), but the approach has not been approved for clinical use. Here, we used a novel chemiluminescence method to assess sBCMA levels in 759 serum samples from 17 healthy donors and 443 patients with plasma cell (PC) diseases including AL amyloidosis, POEMS syndrome and MM. Serum BCMA levels were elevated 16.1-fold in patients with newly diagnosed MM compared to healthy donors and rare PC diseases patients. Specifically, the sBCMA levels in patients with progressive disease were 64.6-fold higher than those who showed partial response or above to treatment. The sBCMA level also correlated negatively with the response depth of MM patients. In newly diagnosed and relapsed MM patients, survival was significantly longer among those subjects whose sBCMA levels are below the median levels compared with those above the median value. We optimized the accuracy of the survival prediction further by integrating sBCMA level into the Second Revised International Staging System (R2-ISS). Our findings provide evidence that the novel chemiluminescence method is sensitive and practical for measuring sBCMA levels in clinical samples and confirm that sBCMA might serve as an independent prognostic biomarker for MM.
PubMed: 38743453
DOI: 10.1093/cei/uxae043 -
Brain and Nerve = Shinkei Kenkyu No... May 2024POEMS syndrome is a multisystem disorder associated with monoclonal plasma cell proliferation and the overproduction of vascular endothelial growth factors. The... (Review)
Review
POEMS syndrome is a multisystem disorder associated with monoclonal plasma cell proliferation and the overproduction of vascular endothelial growth factors. The prognosis of POEMS syndrome has significantly improved owing to anti-myeloma treatments such as thalidomide and autologous stem cell transplantation. Therefore, early diagnosis and appropriate treatment are becoming increasingly important. A thorough and comprehensive evaluation of both systemic symptoms and laboratory abnormalities associated with the disease is essential for early diagnosis. The collaboration between neurology and hematology is indispensable to ensure proper treatment.
Topics: POEMS Syndrome; Humans; Prognosis
PubMed: 38741495
DOI: 10.11477/mf.1416202642 -
Scientific Reports May 2024POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein [M-protein], and skin changes) is a rare systemic disorder characterized by various...
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein [M-protein], and skin changes) is a rare systemic disorder characterized by various symptoms caused by underlying plasma cell (PC) dyscrasia. Detection of monoclonal PCs is mandatory for the diagnosis of POEMS syndrome; however, the usefulness of EuroFlow-based next-generation flow cytometry (EuroFlow-NGF) in POEMS syndrome for detecting monoclonal PCs in bone marrow (BM) and the gating strategy suitable for flow cytometry study of POEMS syndrome remain unknown. We employed EuroFlow-NGF-based single-tube eight-color multiparameter flow cytometry (MM-flow) and established a new gating strategy (POEMS-flow) to detect the monoclonal PCs in POEMS syndrome, gating CD38 broadly from dim to bright and CD45 narrowly from negative to dim compared to MM-flow. MM-flow detected monoclonal PCs in 9/25 (36.0%) cases, including 2/2 immunofixation electrophoresis (IFE)-negative cases (100%). However, POEMS-flow detected monoclonal PCs in 18/25 cases (72.0%), including 2/2 IFE-negative cases (100%). POEMS-flow detected monoclonal PCs with immunophenotypes of CD19 in 17/18 (94.4%). In six cases where post-treatment samples were available, the size of the clones was significantly reduced after the treatment (P = 0.031). POEMS-flow can enhance the identification rate of monoclonal PCs in POEMS syndrome and become a valuable tool for the diagnosis of POEMS syndrome.
Topics: POEMS Syndrome; Humans; Flow Cytometry; Middle Aged; Male; Female; Aged; Plasma Cells; Adult; Immunophenotyping; Bone Marrow
PubMed: 38710832
DOI: 10.1038/s41598-024-61034-1 -
Journal of the Neurological Sciences May 2024
Review
Topics: Humans; POEMS Syndrome; Immunoglobulin M; Paraproteins; Male; Immunoglobulin lambda-Chains; Middle Aged
PubMed: 38704324
DOI: 10.1016/j.jns.2024.123024 -
Muscle & Nerve Jul 2024Whole-body magnetic resonance neurography (MRN) is an imaging modality that shows peripheral nerve signal change in patients with chronic inflammatory demyelinating...
INTRODUCTION/AIMS
Whole-body magnetic resonance neurography (MRN) is an imaging modality that shows peripheral nerve signal change in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We aimed to explore the diagnostic potential of whole-body MRN and its potential as a monitoring tool after immunotherapy in treatment-naïve CIDP patients.
METHODS
Whole-body MRN using coronal 3-dimensional short tau inversion recovery (STIR) sampling perfection with application-optimized contrasts by using different flip angle evolution (SPACE) techniques was performed in patients being investigated for CIDP and in healthy controls. Baseline clinical neuropathy scales and electrophysiologic parameters were collected, and MRN findings were compared before and after CIDP treatment.
RESULTS
We found highly concordant symmetrical thickening and increased T2 signal intensities in the brachial/lumbosacral plexus, femoral, or sciatic nerves in five of the eight patients with a final diagnosis of CIDP and none of the healthy controls. There were no treatment-related imaging changes in five patients with CIDP who completed a follow-up study. Diffuse, symmetrical thickening, and increased T2 signal in root, plexus, and peripheral nerves were found in two patients ultimately excluded due to a diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome in addition to signal changes in the muscles, bony lesions, organomegaly, and lymphadenopathy.
DISCUSSION
Whole-body MRN imaging shows promise in detecting abnormalities in proximal nerve segments in patients with CIDP. Future studies evaluating the role of MRN in assessing treatment response should consider follow-up scans after treatment durations of more than 4 months.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Male; Female; Middle Aged; Magnetic Resonance Imaging; Aged; Whole Body Imaging; Adult; Peripheral Nerves; Neural Conduction
PubMed: 38698725
DOI: 10.1002/mus.28098 -
Medicina Clinica May 2024
PubMed: 38697895
DOI: 10.1016/j.medcli.2024.02.022 -
Indian Journal of Gastroenterology :... Apr 2024
PubMed: 38676909
DOI: 10.1007/s12664-024-01581-7 -
EJHaem Apr 2024
Intravitreal aflibercept confounds interpretation of plasma VEGF (vascular endothelial growth factor) levels in POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome.
PubMed: 38633109
DOI: 10.1002/jha2.883 -
Cureus Mar 2024POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes) refers to a rare paraneoplastic entity linked to a plasma...
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes) refers to a rare paraneoplastic entity linked to a plasma cell disorder, characterized by multiple systemic manifestations that must be studied together to establish a timely diagnosis. We report a case of a 60-year-old female who had been initially classified to have Guillain Barré syndrome for one year and seven months, receiving three cycles of immunoglobulin without a positive response. The clinical picture was characterized by painful paresthesias in four limbs and paraparesis, with the patient also developing distal cutaneous hyperpigmentation and multiple adenopathies. Neuroconduction studies revealed chronic sensorimotor axonal polyneuropathy and albumin-cytological dissociation was evidenced in the study of cerebrospinal fluid (CSF). In the subsequent evaluation, Lambda light chains and a lymph node biopsy compatible with Castleman's disease were found, and hence it was determined that the patient met the criteria for POEMS syndrome. This case report highlights the importance of incorporating other diagnostic perspectives when encountering patients with polyneuropathy of immunological origin who fail to respond to conventional therapies.
PubMed: 38623123
DOI: 10.7759/cureus.56229 -
Hong Kong Medical Journal = Xianggang... Apr 2024
PubMed: 38618913
DOI: 10.12809/hkmj2210439