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Unraveling Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome: Diagnostic Challenges and Therapeutic Strategies at a National Tertiary Care Center.
Cureus
December 2024
Internal Medicine, National Medical Center November 20, Mexico City, MEX.
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) syndrome is a rare paraneoplastic disorder caused by plasma cell proliferation and overproduction of cytokines, particularly vascular endothelial growth factor (VEGF). This complex syndrome affects multiple organ systems and presents with a broad range of clinical and laboratory manifestations, which can complicate both diagnosis and management. Not all components of the acronym are observed in every patient, highlighting the clinical heterogeneity of the condition.
View Article and Find Full Text PDFCase report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome.
Front Med (Lausanne)
December 2024
Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China.
POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy.
View Article and Find Full Text PDFA case report of an M protein-negative patient with POEMS syndrome associated with renal involvement.
BMC Nephrol
December 2024
Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China.
Background: POEMS syndrome with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes is an uncommon plasma cell paraneoplastic syndrome involving multiple system. It is relatively rare in clinical practice, and renal involvement is a usual yet easily overlooked symptom.
Case Presentation: We successfully treated a patient with M protein-negative POEMS syndrome with membranoproliferative glomerulonephritis (MPGN) findings and thrombotic microangiopathic changes by comparing the level of Vascular endothelial growth factor (VEGF) in the serum and the changes in polyserositis before and after the patient's treatment.
Castleman disease variant of POEMS syndrome without M protein: a case report.
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 PDFFirst-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report.
Leuk 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.
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