Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is associated with increased risk for ischemic stroke (IS). Because POEMS syndrome is rare, little is known regarding the underlying mechanism and prognosis for IS in patients in whom it occurs. The medical records of patients with POEMS syndrome were screened between January 2018 and January 2000 at Peking Union Medical College Hospital to identify those with IS. The baseline characteristics, IS features, and patient outcomes were analyzed. Forty-one (8.0%) of 510 POEMS patients were documented to have IS. Patients with IS were older, had a higher percentage of Overall Neuropathy Limitation Scale score >4, and had a higher level of fibrinogen compared with those who did not have IS. Ninety-three percent of IS events occurred before or within 3 months after a diagnosis of POEMS. Of 41 occurrences of IS, 29 (46.3%) were multifocal. Recurrent IS was observed in 13 (31.7%) of 41 patients, but not in patients with successful anti-plasma cell therapy. The 3-year overall survival rate in patients with IS was 71.0% and for those without IS, it was 88.5% (P = .002). We showed that 8.0% of POEMS patients had IS, and most IS events occurred in POEMS patients not being treated effectively. Having IS was a predictor of unfavorable prognosis. Early diagnosis, immediate initiation of treatment for POEMS, and control of POEMS syndrome is key to reducing the occurrence of IS, improving survival, and preventing recurrence of IS.
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http://dx.doi.org/10.1182/bloodadvances.2020001865 | DOI Listing |
Front Oncol
December 2024
Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is a paraneoplastic syndrome associated with an underlying plasma cell neoplasm. According to the current diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy and clonal plasma cell disorder are required for diagnosis. We report a case of a Castleman disease variant of POEMS syndrome without monoclonal protein (M protein) expression, which presented with polyneuropathy, organomegaly, endocrinopathy, skin lesions, and sclerotic bone lesions.
View Article and Find Full Text PDFLeuk Res Rep
November 2024
Hematology, Department of Translational and Precision Medicine, Sapienza University of Rome, Azienda Policlinico Umberto I, Rome, Italy.
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome) is a rare condition due to an underlying plasma cell neoplasm whose clinical presentation can be various so it could lead to delayed diagnosis and treatment. The pathogenesis of the syndrome is not well understood, and its therapy is adapted from other plasma cell disorders with the aim of alleviating symptoms, decreasing end-organ damage, improving quality of life and prolonging overall survival. We report a case of a 71 years-old woman who has been treated with continuous DRd (daratumumab, lenalidomide and dexamethasone) scheme.
View Article and Find Full Text PDFLeukemia
December 2024
Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Cause of death (COD) in POEMS (polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and skin changes) syndrome is not well described. We investigated COD in patients with POEMS syndrome treated at Mayo Clinic between 2000 and 2022. Of the 89 deaths, 49 patients had known COD and were the subject of this study.
View Article and Find Full Text PDFAnn Diagn Pathol
February 2025
Pathology Department and Translational Hematopathology Lab, Hospital Universitario Marqués de Valdecilla/IDIVAL, UNICAN, Santander, Spain. Electronic address:
Plasma cell neoplasia and POEMS syndrome patients may present Castleman disease (CD)-type features in lymph nodes. Our aim was to better characterize the histopathological patterns found in plasma cell neoplasia associated CD and to improve the detection of clonal plasma cell populations in the lymph node biopsies of these patients. Lymph node and bone marrow samples from six cases with plasma cell neoplasia associated CD, including POEMS syndrome and multiple myeloma were analyzed.
View Article and Find Full Text PDFAsian J Surg
November 2024
Department of Neurology, The First Affiliated Hospital of Soochow University, Suzhou, 215000, China. Electronic address:
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