1,390 results match your criteria POEMS Syndrome


Biclonal Gammopathy as a Misleading Indicator to Diagnose POEMS Syndrome: An Autopsy Case Report and a Review of the Literature.

Cureus 2022 May 20;14(5):e25153. Epub 2022 May 20.

Department of General Internal Medicine, Kobe University Hospital, Kobe, JPN.

A 76-year-old man presented with a four-month history of progressive bilateral lower limb muscle weakness and dysesthesia. The patient had extravascular volume overload, and laboratory findings confirmed hypothyroidism, renal dysfunction, and chronic inflammation. Serum protein and immunofixation electrophoresis revealed biclonality of immunoglobulin A (IgA)-kappa and IgA-lambda, which was attributed to chronic inflammation. Read More

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A case of POEMS syndrome with severe kidney damage: lessons for the clinical nephrologist.

J Nephrol 2022 Jun 23. Epub 2022 Jun 23.

Department of Chemotherapy of Paraproteinemic Blood Malignancies, National Medical Research Centre of Hematology, Moscow, Russian Federation.

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POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes): A Case Report of a Rare Paraneoplastic Syndrome.

Cureus 2022 May 13;14(5):e24980. Epub 2022 May 13.

Department of Hematology and Oncology, Mount Sinai Hospital, Chicago, USA.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome is a multisystem disorder. Peripheral neuropathy and monoclonal plasma cell disorder are the most common manifestations of POEMS. Although osteosclerotic or mixed sclerotic-lytic lesions are typical, osteolytic lesions are rarely encountered. Read More

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Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes (POEMS) Syndrome and Idiopathic Portal Hypertension: A Rare Association.

Cureus 2022 May 11;14(5):e24923. Epub 2022 May 11.

Hematology, Faculty of Medicine, Mohammed VI University of Health Sciences (UM6SS), Casablanca, MAR.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma (PCD), and at least one of the minor criteria. The most frequent liver manifestation is hepatomegaly. Read More

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[Ixazomib-Based Regimen Followed by Autologous Hematopoietic Stem Cell Transplantation in the Treatment of POEMS Syndrome].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2022 Jun;30(3):937-942

Department of Hematology, Wuhan No.1 Hospital, Wuhan 430022, Hubei Province, China,E-mail:

Objective: To investigate the safety and efficacy of a new proteasome inhibitor Ixazomib followed by autologous hematopoietic stem cell transplantation (AHSCT) in the treatment of POEMS syndrome.

Methods: The clinical manifestations, diagnosis and treatment process and follow-up results of 4 patients with POEMS syndrome who were treated with Ixazomib-based regimen combined with AHSCT in Wuhan No.1 Hospital from February 2018 to July 2020 were analyzed retrospectively. Read More

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Osteosclerotic Multiple Myeloma without POEMS Syndrome - A Rare Case Report.

J Orthop Case Rep 2022 Jan;12(1):18-21

Department of Spine, Sancheti Institute for Orthopaedics and Rehabilitation, Pune, Maharashtra, India.

Introduction: Multiple myeloma is a plasma cell neoplasm that is commonly associated with lytic bone lesions; however, osteosclerotic multiple myeloma is a rare entity. Osteosclerotic multiple myeloma has been reported in association with POEMS syndrome.

Case Presentation: A 60-year-old female patient presented to us with low back pain for 2 months. Read More

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January 2022

TAFRO Syndrome: A Disease Requiring Immediate Medical Attention.

Intern Med 2022 May 21. Epub 2022 May 21.

Department of Hematology, National Hospital Organization Kyoto Medical Center, Japan.

TAFRO syndrome was first described in 2010, standing for thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly. Because the lymph node histopathology of TAFRO syndrome mimics idiopathic multicentric Castleman disease (iMCD), some researchers consider TAFRO syndrome to be a subtype of iMCD. However, the clinical features of TAFRO syndrome considerably differ from those of iMCD without TAFRO. Read More

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Raynaud's phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report.

BMC Rheumatol 2022 May 19;6(1):26. Epub 2022 May 19.

Internal Medicine, Universidad Nacional de Colombia, Bogotá, Colombia.

Background: POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis. Read More

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Update on the POEMS syndrome.

Authors:
Yu Ri Kim

Blood Res 2022 Apr;57(S1):27-31

Division of Hematology, Department of Internal Medicine, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.

POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. It is a rare paraneoplastic disorder related to plasma cell neoplasm. However, its pathophysiology has not yet been clearly elucidated. Read More

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Historical and pathological overview of Castleman disease.

J Clin Exp Hematop 2022 Apr 27. Epub 2022 Apr 27.

Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.

Castleman disease consists of several lymphoproliferative subtypes that share some histological features in the lymph nodes. On the other hand, numerous clinical findings and etiologies make the disease challenging to understand. The origin of the disease is the hyaline vascular-type unicentric Castleman disease (UCD), first reported by Benjamin Castleman et al. Read More

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POEMS Syndrome: Presented as Idiopathic Multicentric Castleman Disease of Plasma Cell Variant for Eight Years and Dramatic Treatment with Siltuximab Followed by Autologous Peripheral Blood Stem Cell Transplantation.

Diagnostics (Basel) 2022 Apr 15;12(4). Epub 2022 Apr 15.

Department of Pathology, College of Medicine, Chungnam National University, 266 Munwha-ro, Jung-gu, Daejeon 35015, Korea.

Background: POEMS syndrome (POEMS) is a rare plasma cell clonal paraneoplastic syndrome consisting of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes presenting with idiopathic multicentric Castleman disease (iMCD) histology, the treatment of which has not yet been well established. iMCD is also a distinctive rare non-clonal lymphoproliferative disorder, of which dramatic response to Siltuximab, a monoclonal anti-IL-6 antibody, has been reported recently.

Methods: the differential diagnosis between POEMS and iMCD can be very challenging because of the identical histology, overlapping similar symptoms such as polyneuropathy, and vital signs insidiously presented to diagnose POEMS. Read More

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POEMS Syndrome: Skin Lesions Bringing the Jigsaw Puzzle Together.

Skinmed 2022 28;20(1):66-68. Epub 2022 Feb 28.

Department of Pathology and Laboratory Medicine, AIIMS, Raipur, India.

A 47-year-old man presented to the outpatient department with multiple asymptomatic, erythematous and violaceous, soft, dome-shaped papulonodular lesions that had been present for 3 years. The lesions were vascular and were about 1 cm in diameter. They were present over the face, trunk, arms and legs (Figures 1 and 2). Read More

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Vitamin B6 deficiency as a cause of polyneuropathy in POEMS syndrome: rapid recovery with supplementation in two cases.

Hematology 2022 Dec;27(1):463-468

Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.

Background: The etiology of POEMS syndrome and its associated polyneuropathy have not been fully elucidated. The clinical picture of POEMS-associated polyneuropathy and nutritional polyneuropathy due to vitamin B6 (VB6) deficiency are strikingly similar, both being typically sensorimotor, symmetrical, stocking and glove distribution, and more severe in the lower extremities.

Case Presentation: We report two consecutive POEMS patients with VB6 deficiency who showed unusual rapid and drastic recovery of polyneuropathies within 6-8 weeks after oral VB6 supplementation. Read More

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December 2022

Cutaneous manifestations of monoclonal gammopathy.

Blood Cancer J 2022 04 11;12(4):58. Epub 2022 Apr 11.

Division of Hematology, Mayo Clinic Rochester, Mayo Clinic, MN, USA.

Monoclonal gammopathy associated with dermatological manifestations are a well-recognized complication. These skin disorders can be associated with infiltration and proliferation of a malignant plasma cells or by a deposition of the monoclonal immunoglobulin in a nonmalignant monoclonal gammopathy. These disorders include POEMS syndrome, light chain amyloidosis, Schnitzler syndrome, scleromyxedema and TEMPI syndrome. Read More

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Imaging for Plasma Cell Dyscrasias: What, When, and How?

Front Oncol 2022 24;12:825394. Epub 2022 Mar 24.

Homi Bhabha National Institute, Training School Complex, Mumbai, India.

Imaging plays a vital role in the diagnosis, response assessment, and follow-up of patients with plasma cell bone disease. The radiologic diagnostic paradigm has thus far evolved with developing technology and availability of better imaging platforms; however, the skewed availability of these imaging modalities in developed vis-à-vis the developing countries along with the lack of uniformity in reporting has led to a consensus on the imaging criteria for diagnosing and response assessment in plasma cell dyscrasia. Therefore, it is imperative for not only the radiologists but also the treating oncologist to be aware of the criteria and appropriate imaging modality to be used in accordance with the clinical question. Read More

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From Biology to Treatment of Monoclonal Gammopathies of Neurological Significance.

Cancers (Basel) 2022 Mar 18;14(6). Epub 2022 Mar 18.

Neurology Unit, Department of Neurosciences, University of Padova, 35128 Padova, Italy.

Monoclonal gammopathy and peripheral neuropathy are common diseases of elderly patients, and almost 10% of patients with neuropathy of unknown cause have paraprotein. However, growing evidence suggests that several hematological malignancies synthesize and release monoclonal proteins that damage the peripheral nervous system through different mechanisms. The spectrum of the disease varies from mild to rapidly progressive symptoms, sometimes affecting not only sensory nerve fibers, but also motor and autonomic fibers. Read More

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A Case of POEMS Syndrome with Biclonal Gammopathy and Renal Involvement.

Intern Med 2022 Mar 12. Epub 2022 Mar 12.

Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan.

POEMS syndrome manifests as elevated levels of vascular endothelial growth factor (VEGF) and monoclonal gammopathy. We treated a case of POEMS syndrome showing monoclonality in both IgA-λ and IgG-κ. Serial renal biopsies before treatment and after normalization of the VEGF levels suggested that glomerular microangiopathy had developed due to VEGF, while biclonal gammopathy was not eliminated. Read More

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Clinical characteristics and prognosis of patients with Castleman disease in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor.

Am J Transl Res 2022 15;14(2):1051-1059. Epub 2022 Feb 15.

Department of Urology, The First Affiliated Hospital of Nanjing Medical University Nanjing 210029, Jiangsu Province, China.

Objective: Castleman disease (CD) is a rare lymphoproliferative disorder with limited clinical research data. This study aimed to investigate the clinical manifestations, pathologic features, and prognostic factors of CD.

Methods: The clinicopathological data of 54 patients with CD hospitalized in the First Affiliated Hospital of Nanjing Medical University were retrospectively analyzed. Read More

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February 2022

Nerve Ultrasound Performances in Differentiating POEMS Syndrome from CIDP.

Neurotherapeutics 2022 Mar 7;19(2):455-463. Epub 2022 Mar 7.

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Chronic inflammatory demyelinating polyneuropathy (CIDP) and polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome are both acquired demyelinating polyneuropathies. We aim to explore the different features of ultrasonographic changes between CIDP and POEMS syndrome. Nerve ultrasonographic studies were performed in 120 patients with CIDP and 34 patients with POEMS syndrome. Read More

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POEMS Syndrome Diagnosis in a Patient with Mixed Polyneuropathy: Case Report.

Prague Med Rep 2022 ;123(1):27-34

Department of Neuroscience, Hospital Universitario San Rafael de Tunja, Boyacá, Colombia.

POEMS syndrome is a rare condition of paraneoplasic origin characterized by the presence of a sensorimotor polyneuropathy associated with the presence of a proliferative disorder of plasmatic monoclonal cells and overproduction of vascular endothelial growth factor. The acronym "POEMS" represents multisystem findings including polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes; nevertheless, clinical presentation is heterogeneous. We describe a clinical case, the diagnostic and therapeutic approach in a patient with sensorimotor polyneuropathy in whom POEMS syndrome was diagnosed; to understand this pathology, its clinical and paraclinical manifestations in order to make a diagnosis or to avoid a delayed one and to provide an adequate treatment. Read More

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POEMS syndrome causing left ventricular hypertrophy, myocardial dysfunction, and pericardial effusion: a case report.

Eur Heart J Case Rep 2022 Jan 16;6(1):ytab504. Epub 2021 Dec 16.

Department of Cardiology, The Second Affiliated Hospital of Soochow University, Suzhou, China.

Background: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a paraneoplastic syndrome caused by a plasma cell proliferative disorder. Characteristics of POEMS syndrome include elevated pro-inflammatory and angiogenic cytokine levels that lead to multi-organ dysfunction. Patients have a variety of initial symptoms, but cardiac involvement is not common. Read More

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January 2022

A Case of Oral Glomeruloid Hemangioma Without Systemic Conditions.

Cureus 2022 Jan 29;14(1):e21705. Epub 2022 Jan 29.

Hard Tissue Pathology Unit, Graduate School of Oral Medicine, Matsumoto Dental University, Shiojiri, JPN.

Glomeruloid hemangioma is a rare variant of hemangioma that is accompanied by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities (POEMS) syndrome and, rarely, by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome. This report presents the case of a 78-year-old male who presented with a hemorrhagic nodule on the tongue without any other systemic diseases. Microscopically, the lesion was a lobular proliferation extending from the lamina propria to muscular tissue. Read More

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January 2022

POEMS Syndrome: an uncommon cause of pleural effusion.

J Bras Pneumol 2022 02 2;48(1):e20210409. Epub 2022 Feb 2.

. Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brasil.

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February 2022

Efficacy of novel agents in patients with nephropathy associated with POEMS syndrome.

Int Urol Nephrol 2022 Feb 8. Epub 2022 Feb 8.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, #305 East Zhongshan Road, Nanjing, 210016, China.

Objective: To evaluate the clinical characteristics and outcomes of patients with nephropathy associated with POEMS syndrome who received novel agents in combination with dexamethasone therapy, and renal pathological changes based on repeat biopsy in some patients after these novel-agent-based therapies.

Methods: The records of patients with nephropathy associated with POEMS syndrome in a single hospital from May 2017 to February 2021 were retrieved and studied in detail. All the patients received four cycles of initial novel-agent-based regimens such as bortezomib and dexamethasone (BD) or thalidomide plus dexamethasone (TD) or lenalidomide plus dexamethasone (RD) treatment. Read More

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February 2022

A case of systemic mastocytosis mimicking POEMS syndrome: A case report.

Medicine (Baltimore) 2022 Feb;101(5):e28651

Second Affiliated Hospital of Nanjing Medical University, Nanjing, China.

Rationale: POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare and complicated disease related to multiple organs and systems. Here, we report a case of systemic mastocytosis (SM) that was misdiagnosed as a POEMS syndrome.

Patient Concerns: A 42-year-old man presented with skin changes, diarrhea, and limb numbness. Read More

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February 2022

Glomeruloid Hemangioma in a Patient with TAFRO Syndrome.

Intern Med 2022 Feb 1. Epub 2022 Feb 1.

Department of Nephrology, University of Yamanashi Hospital, Japan.

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February 2022

Clinical and neurophysiological description of patients with POEMS syndrome.

Gac Med Mex 2021 ;157(5):466-472

Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Introduction: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a monoclonal gammopathy with polyneuropathy as a mandatory criterion.

Objective: To describe potential associations between clinical expression and electrodiagnostic patterns in POEMS syndrome.

Method: Observational, retrospective, cross-sectional study of cases cared for in a referral center, diagnosed with POEMS syndrome from 2009 to 2019. Read More

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February 2022