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POEMS syndrome with renal plasmacytoma and classic polyarteritis nodosa: a case report.

ABSTRACT: POEMS syndrome is a rare conglomeration of disorders associated with plasma cell dyscrasia. The acronym POEMS is derived from main features of the syndrome namely 'polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions'. Other clinical features include presence of sclerotic bone lesions, Castleman's disease, papilledema, pleural effusion, edema, ascites, erythrocytosis and thrombocytosis. Myeloma is the most common plasma cell dyscrasia associated with POEMS syndrome. Renal involvement is rare and renal biopsy is characterized by glomerular involvement with membranoproliferative glomerulonephritis and endothelial injury. We report a case of a 67-year-old male who presented with clinical features satisfying the diagnostic criteria of POEMS syndrome and had rapidly progressive renal failure. Renal biopsy showed extensive interstitial infiltration by plasma cells and concomitant presence of classic polyarteritis nodosa. Although association with small-vessel vasculitis has been reported in patients with POEMS syndrome, to the best of our knowledge, this is the first report of POEMS syndrome associated with medium-sized vessel vasculitis.

SUBMITTER: Jha PK

PROVIDER: S-EPMC4421663 | biostudies-literature | 2011 Dec

REPOSITORIES: biostudies-literature

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POEMS syndrome with renal plasmacytoma and classic polyarteritis nodosa: a case report.

Jha Pranaw Kumar PK Vankalakunti Mahesha M Bonu Ravishankar R Babu Kishore K Ballal H Sudarshan HS

NDT plus 20111201 6

POEMS syndrome is a rare conglomeration of disorders associated with plasma cell dyscrasia. The acronym POEMS is derived from main features of the syndrome namely 'polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions'. Other clinical features include presence of sclerotic bone lesions, Castleman's disease, papilledema, pleural effusion, edema, ascites, erythrocytosis and thrombocytosis. Myeloma is the most common plasma cell dyscrasia associated with POEMS syndrom ...[more]

PMID: 25984208

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Project description:Background and Purpose: The management of unruptured intracranial aneurysms remains controversial. The decisions to treat are heavily informed by estimated risk of bleeding. However, these estimates are imprecise, and better methods for stratifying the risk or tailoring treatment strategy are badly needed. Here, we demonstrate an initial proof-of-principle concept for endovascular biopsy to identify the key molecular pathways and gene expression changes associated with aneurysm formation. We couple this technique with single cell RNA sequencing (scRNAseq) to develop a roadmap of the pathogenic changes of a dolichoectatic vertebrobasilar aneurysm in a patient with polyarteritis nodosa. Methods: Endovascular biopsy and fluorescence activated cell sorting was used to isolate the viable endothelial cells (ECs) using the established techniques. A single cell RNA sequencing (scRNAseq) was then performed on 24 aneurysmal ECs and 23 patient-matched non-aneurysmal ECs. An integrated panel of bioinformatic tools was applied to determine the differential gene expression, enriched signaling pathways, and cell subpopulations hypothesized to drive disease pathogenesis. Results: We identify a subset of 7 (29%) aneurysm-specific ECs with a distinct gene expression signature not found in the patient-matched control ECs. A gene set enrichment analysis identified these ECs to have increased the expression of genes regulating the leukocyte-endothelial cell adhesion, major histocompatibility complex (MHC) class I, T cell receptor recycling, tumor necrosis factor alpha (TNFα) response, and interferon gamma signaling. A histopathologic analysis of a different intracranial aneurysm that was later resected yielded a diagnosis of polyarteritis nodosa and positive staining for TNFα. Conclusions: We demonstrate feasibility of applying scRNAseq to the endovascular biopsy samples and identify a subpopulation of ECs associated with cerebral aneurysm in polyarteritis nodosa. Endovascular biopsy may be a safe method for deriving insight into the disease pathogenesis and tailoring the personalized treatment approaches to intracranial aneurysms.

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Dataset Information

POEMS syndrome with renal plasmacytoma and classic polyarteritis nodosa: a case report.

Publications

POEMS syndrome with renal plasmacytoma and classic polyarteritis nodosa: a case report.

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