Multiparameter Flow Cytometry for the Identification of Neoplastic Plasma Cells in POEMS Syndrome with IgG-kappa Gammopathy: Successful Treatment Using Lenalidomide and Dexamethasone

Ikeda, Sho; Kobayashi, Takahiro; Saito, Masaya; Komatsuda, Atsushi; Ubukawa, Kumi; Kameoka, Yoshihiro; Takahashi, Naoto

doi:10.2169/internalmedicine.2882-19

Cited by 7 publications

(6 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…CD19 expression in PCs of monoclonal gammopathy of undetermined significance is lower than in normal PCs but higher than in MM PCs, and loss of CD19 has been associated with tumor progression 23 . Several reports referring to the immunophenotype of POEMS syndrome also reported that CD19 was negative 8 , 11 , 24 , 25 , which is consistent with this study, indicating that the monoclonal PCs in POEMS syndrome are immunophenotypically distinct from normal polytypic PCs. On the other hand, one case with CD19 + monoclonal PCs was detected in this study (Fig.…”

Section: Discussionsupporting

confidence: 91%

Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry

Ishii,

Tsukamoto,

Mimura

et al. 2024

Sci Rep

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 91%

Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry

Ishii,

Tsukamoto,

Mimura

et al. 2024

Sci Rep

View full text Add to dashboard Cite

show abstract

“… 1 , 4 These isolated cases have demonstrated either demyelinating polyneuropathy alone or with axonal degeneration, except for 1 case in which the neuropathy was not characterized. 4 , 5 , 6 , 7 However, there was no evidence of demyelinating polyneuropathy in this case. Additionally, all previously reported kappa restricted cases showed evidence of endocrinopathy, which is absent here.…”

Section: Discussionmentioning

confidence: 56%

Plasma cell and neutrophil enriched neovascularization with granulomatous lymphangitis in POEMS syndrome

Adeuyan,

Magro,

Trager

et al. 2024

JAAD Case Reports

View full text Add to dashboard Cite

“…This suggests that the M-protein might be one of the driving factors of the disease. The plasma cell clone producing the M protein is usually λ light chain restricted, but atypically can also present with Kappa light chain restriction [ 10 , 11 ]. Clonal immunoglobulin λ light variable chain (IGLV) was strictly derived from IVLG-1–40 and IVLG-1–44 genes.…”

Section: Pathogenesis Of the Diseasementioning

confidence: 99%

Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

Zerdan

George

Bunting

et al. 2022

JCM

View full text Add to dashboard Cite

POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.

show abstract

Multiparameter Flow Cytometry for the Identification of Neoplastic Plasma Cells in POEMS Syndrome with IgG-kappa Gammopathy: Successful Treatment Using Lenalidomide and Dexamethasone

Cited by 7 publications

References 30 publications

Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry

Detection of clonal plasma cells in POEMS syndrome using multiparameter flow cytometry

Plasma cell and neutrophil enriched neovascularization with granulomatous lymphangitis in POEMS syndrome

Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

Contact Info

Product

Resources

About