1,320 results match your criteria POEMS Syndrome


Pearls & Oy-sters: POEMS Syndrome: An Eloquent Acronym for a Rare Disease You Don't Want to Miss.

Neurology 2021 Jun 7. Epub 2021 Jun 7.

Jennifer Juhl Majersik, Department of Neurology, University of Utah, Salt Lake City, UT.

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Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.

JAMA Oncol 2021 Jun 3. Epub 2021 Jun 3.

Department of Medicine, University of California, San Francisco, San Francisco.

Importance: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare plasma cell disorder characterized by demyelinating peripheral neuropathy and clonal plasma cell proliferation. Clinical manifestations are believed to be associated with a surge of inflammatory and angiogenic mediators, including interleukins and vascular endothelial growth factor (VEGF), elicited by clonal and polyclonal plasma cells. The clinical manifestations of POEMS syndrome can be debilitating; therefore, early diagnosis is essential. Read More

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Skin Responses in Newly Diagnosed Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome After Therapy With Low-Dose Lenalidomide Plus Dexamethasone.

Front Immunol 2021 6;12:681360. Epub 2021 May 6.

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

Background: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare paraneoplastic disease with a high prevalence of skin symptoms. Few studies have focused on skin responses to systemic treatment of this disease.

Objective: To evaluate skin responses after treatment with low-dose lenalidomide plus dexamethasone and determine their relationship with vascular endothelial growth factor (VEGF) and hematological responses. Read More

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Successful second outpatient autologous hematopoietic cell transplant for relapsed POEMS syndrome in a patient with coexisting HIV, HBV and syphilis infections during the COVID-19 pandemic.

Transpl Immunol 2021 Aug 19;67:101412. Epub 2021 May 19.

Department of Hematology, Dr. José Eleuterio González University Hospital, School of Medicine, Universidad Autónoma de Nuevo León, Monterrey, Mexico.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a multisystem autoinflammatory disease due to an underlying plasma cell disorder that lacks a standard treatment strategy because of its rarity. We report a case of relapsed POEMS syndrome successfully treated with a second ambulatory autologous hematopoietic-cell transplantation (AHCT) after a daratumumab desensitization protocol performed during the coronavirus disease (COVID-19) pandemic in a patient with coexisting human immunodeficiency virus (HIV), hepatitis B virus (HBV) and syphilis infections. He is a 37-year old Latin-American male who had been treated with radiation, CyBorD regimen, AHCT and bortezomib therapy before being referred to our service. Read More

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POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management.

Am J Hematol 2021 07 31;96(7):872-888. Epub 2021 May 31.

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Disease Overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Read More

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POEMS syndrome: an uncommon cause of peritoneal effusion.

Rev Esp Enferm Dig 2021 May 5. Epub 2021 May 5.

Gastroenterology, Suzhou Ninth People's Hospital, China.

A 46-year-old female patient was admitted to our hospital with abdominal distension and fatigue for 1 months. Physical examination revealed several lymph nodes were palpable in bilateral axilla and groin, ranging from 0.5cm to 2. Read More

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Paraproteinemias and Peripheral Nerve Disease.

Clin Geriatr Med 2021 May;37(2):301-312

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA. Electronic address:

This article provides an overview of the clinical features, diagnosis, and treatment of the major paraprotein-related peripheral neuropathies, including monoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, POEMS syndrome, multiple myeloma, transthyretin amyloidosis, and light chain amyloidosis. For each paraprotein neuropathy, the epidemiology, demographics, systemic findings, and electrophysiologic features are presented. Pharmacologic treatment of transthyretin amyloid polyneuropathy also is reviewed. Read More

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Multicentric Castleman's disease in India - Does EBV rather than HHV8 play a role?

Indian J Pathol Microbiol 2021 Apr-Jun;64(2):302-309

Professor and Secretary Adult Hematolymphoid Disease Management Group, Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India.

Background And Aim: Multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder manifesting as multiple lymphadenopathy, multiorgan involvement, and inflammatory symptoms. This study aims at highlighting some unique features of MCD in Indian patients.

Materials And Methods: These 17 patients from review of 78 cases of Castleman's disease (CD) diagnosed. Read More

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TAFRO Syndrome With Kidney Involvement: A Case Series of Patients With Kidney Biopsies.

Kidney Med 2021 Mar-Apr;3(2):286-293. Epub 2021 Jan 19.

Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan.

TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal insufficiency, and organomegaly) syndrome is a systemic inflammatory disease sharing some features with Castleman disease and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome in relation to abnormal secretions of interleukin 6 and vascular endothelial growth factor. The kidney is a main target organ of TAFRO syndrome but the kidney histopathology associated with TAFRO syndrome is yet to be completely defined. We report 3 TAFRO syndrome cases with different clinical courses in which kidney biopsies were performed. Read More

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January 2021

Cereblon expression is a prognostic marker in newly diagnosed POEMS syndrome treated with lenalidomide plus dexamethasone.

Ann Hematol 2021 Jun 10;100(6):1547-1552. Epub 2021 Apr 10.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.

POEMS syndrome is a rare plasma cell disorder. Lenalidomide has recently emerged as a therapeutic option for POEMS syndrome. Cereblon has been identified as the direct target of lenalidomide, and high cereblon expression is associated with better response and outcome to lenalidomide therapy in multiple myeloma patients. Read More

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Two cases of special POEMS syndrome without monoclonal protein expression: a case report and literature review.

J Int Med Res 2021 Apr;49(4):300060521990967

Gastrointestinal Department, Zhejiang University School of Medicine, Sir Run Run Shaw Hospital, Hangzhou, Zhejiang, China.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is rare, with polyneuropathy and monoclonal plasma cell disorder generally considered as essential diagnostic symptoms. We report two cases of POEMS syndrome without monoclonal protein expression. The first case was a 72-year-old man who had experienced recurrent edema of the lower limbs for 2 years and abdominal distention for 2 months. Read More

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A Review of Genetic Abnormalities in Unicentric and Multicentric Castleman Disease.

Biology (Basel) 2021 Mar 24;10(4). Epub 2021 Mar 24.

Department of Pathology, University of California, San Francisco, CA 94143, USA.

Castleman disease (CD) is a rare lymphoproliferative disorder known to represent at least four distinct clinicopathologic subtypes. Large advancements in our clinical and histopathologic description of these diverse diseases have been made, resulting in subtyping based on number of enlarged lymph nodes (unicentric versus multicentric), according to viral infection by human herpes virus 8 (HHV-8) and human immunodeficiency virus (HIV), and with relation to clonal plasma cells (POEMS). In recent years, significant molecular and genetic abnormalities associated with CD have been described. Read More

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Asymmetric optic disc edema in a young patient with POEMS: A rare presentation of a rare disease.

Am J Ophthalmol Case Rep 2021 Jun 16;22:101064. Epub 2021 Mar 16.

Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, 635 West 165th Street, New York, NY, USA.

Purpose: To describe a case of asymmetric optic disc edema presenting as the initial ocular feature of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes) syndrome.

Observations: A 29-year-old female patient presented with 3 weeks history of blurred vision, proptosis, and peripheral neuropathy as well as hypothyroidism. Fundoscopy revealed optic disc edema associated with visual loss in the left eye. Read More

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Revealing serum lipidomic characteristics and potential lipid biomarkers in patients with POEMS syndrome.

J Cell Mol Med 2021 May 28;25(9):4307-4315. Epub 2021 Mar 28.

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

POEMS syndrome is a rare plasma cell dyscrasia with distinct lipid metabolism abnormalities at disease onset. However, the serum lipidomic characteristics in patients with POEMS syndrome were not investigated. The study performed an untargeted lipidome screening by liquid chromatography-tandem mass spectrometry (LS-MS/MS) in the pre- and post-treatment serum of 24 patients with POEMS syndrome, together with the serum of 24 paired healthy controls. Read More

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Patients who meet electrodiagnostic criteria for CIDP rarely present with a sensory predominant DSP phenotype.

Muscle Nerve 2021 Jun 8;63(6):881-884. Epub 2021 Apr 8.

Department of Neurology, Division of Neuromuscular Medicine, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.

Introduction: It is unknown how often patients with electrodiagnostic evidence of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a potentially treatable condition, present with a distal symmetric polyneuropathy (DSP) phenotype.

Methods: We reviewed the records of patients who presented to our electrodiagnostic laboratory between January 1, 2011, to December 31, 2019, and fulfilled electrodiagnostic criteria for CIDP to identify those who presented with a sensory predominant DSP phenotype.

Results: One hundred sixty-two patients had a chronic acquired demyelinating neuropathy, of whom 138 met criteria for typical or atypical CIDP. Read More

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[An illustrative case of the POEMS syndrome].

Rev Med Liege 2021 Mar;76(3):156-159

Service d'Hématologie, CHU Liège, Belgique.

POEMS syndrome is a rare and invalidating entity characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and dermatoses. The diagnosis of this condition is often late and challenging due to the heterogeneity of clinical forms. The light chains secreted by the clonal plasmocytes cause overproduction of VEGF (Vascular Endothelial Growth Factor) responsible for the appearance of the clinical manifestations of POEMS. Read More

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[POEMS syndrome: Diagnosis, stratification, treatments].

Rev Med Interne 2021 May 5;42(5):320-329. Epub 2021 Mar 5.

Service d'immuno-hématologie, Hôpital Saint-Louis, AP-HP, Paris, France, Université de Paris, Paris, France; Inserm, UMR 976, Institut de Recherche Saint-Louis, Université de Paris, Paris, France.

POEMS syndrome is a rare form of B-cell dyscrasia with multiple clinical signs including the acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. It is a paraneoplastic syndrome due to an underlying plasma cell disorder belonging to the monoclonal gammopathies of clinical significance (MGCS). The major criteria for this syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor (VEGF), and the presence of Castleman's disease. Read More

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[Ascites as the first manifestation of POEMS syndrome: a case report].

Zhonghua Nei Ke Za Zhi 2021 Mar;60(3):249-250

Department of Gastroenterology, Shandong Provincial Hospital, Jinan 250021, China.

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CIDP mimics: a case series.

BMC Neurol 2021 Feb 28;21(1):94. Epub 2021 Feb 28.

Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce St., 3 West Gates, Philadelphia, PA, 19104, USA.

Background: To report our experience with a group of patients referred for refractory CIDP who fulfilled "definite" electrodiagnostic EFNS criteria for CIDP but were found to have an alternate diagnosis.

Methods: Patients who were seen between 2017 and 2019 for refractory CIDP that fulfilled "definite" electrodiagnostic ENFS criteria for CIDP, but had an alternate diagnosis, were included. Patients who correctly had CIDP, anti MAG neuropathy, or MMN with conduction block, were excluded from the study. Read More

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February 2021

Reply to "POEMS syndrome remains a mystery after 40 years" (Wang Chen. 20-LEU-1856).

Authors:
Jian Li

Leukemia 2021 04 25;35(4):1222. Epub 2021 Feb 25.

Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College, Beijing, China.

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Nerve ultrasound studies in POEMS syndrome.

Muscle Nerve 2021 05 6;63(5):758-764. Epub 2021 Mar 6.

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

Introduction: The aim of our study was to assess the ultrasonographic features of peripheral nerves in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome.

Method: 34 POEMS syndrome patients and 26 healthy control (HC) participants were recruited prospectively. Cross-sectional area (CSA) was measured in nerves of limbs, trunks of brachial plexus, and cervical nerve roots RESULTS: The CSAs were mildly enlarged at the arm segment of median nerve, elbow segment of ulnar nerve and upper trunk, moderately enlarged at the forearm segment of both median and ulnar nerve, upper trunk of brachial plexus, and C6, C7 cervical nerve roots, and markedly enlarged at the arm segment of ulnar nerve, middle and lower trunk of brachial plexus, as well as C5 cervical root. Read More

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Left ventricular systolic dysfunction with concomitant bradyarrhythmia in a patient with POEMS syndrome: a case report.

Eur Heart J Case Rep 2021 Feb 4;5(2):ytaa510. Epub 2021 Jan 4.

Department of Cardiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng Hospital, Singapore 308433, Singapore.

Background: POEMS syndrome (PS) is a paraneoplastic disorder from plasma cell dyscrasia, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Vascular endothelial growth factors (VEGFs)-driven fluid extracellular matrix expansion plays a key role in this condition. Associated cardiac involvement has been sparsely reported thus far. Read More

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February 2021

POEMS syndrome remains a mystery after 40 years.

Authors:
Chen Wang

Leukemia 2021 04 15;35(4):1220-1221. Epub 2021 Feb 15.

Department of Internal Medicine, University of South Florida, Morsani College of Medicine, Tampa, FL, USA.

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Cerebrovascular manifestations in hematological diseases: an update.

J Neurol 2021 Feb 13. Epub 2021 Feb 13.

Serviço de Hematologia e Transplantação de Medula, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.

Patients with hematological diseases often experience cerebrovascular complications including ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds, posterior reversible encephalopathy syndrome, and dural sinus and cerebral vein thrombosis (CVT). In this update, we will review recent advances in the management of cerebrovascular diseases in the context of myeloproliferative neoplasms, leukemias, lymphomas, multiple myeloma, POEMS, paroxysmal nocturnal hemoglobinuria (PNH), thrombotic thrombocytopenic purpura (TTP), and sickle-cell disease. In acute ischemic stroke associated with hematological diseases, thrombectomy can in general be applied if there is a large vessel occlusion. Read More

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February 2021

High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome-A Comparative Study.

Diagnostics (Basel) 2021 Feb 9;11(2). Epub 2021 Feb 9.

Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany.

Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is an important differential diagnosis of CIDP. Until now, there have been no studies that could identify specific HRUS abnormalities in POEMS syndrome patients. Read More

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February 2021

Missed diagnosis of POEMS syndrome with onset of progressive fatigue and numbness: a case report.

J Int Med Res 2021 Feb;49(2):300060520986706

Department of Endocrinology, Third Affiliated Hospital, Soochow University, Changzhou, Jiangsu, China.

POEMS syndrome is a rare multisystem disease associated with an underlying plasma cell disorder. Its name is an acronym for peripheral neuropathy (P), endocrinopathy (E), organomegaly (O), monoclonal plasma cell proliferative disorder (M), and skin changes (S). This case report describes a patient with POEMS syndrome who presented with progressive fatigue and numbness in the lower extremities. Read More

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February 2021

POEMS Syndrome with Extensive Calciphylaxis: A Case Report and Literature Review.

Indian J Dermatol 2020 Nov-Dec;65(6):516-518

Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.

POEMS syndrome, a paraneoplastic disorder, caused by plasma cell dyscrasia, is characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Calciphylaxis is a vasculopathy of subcutaneous arteries with calcification and skin ulceration, most commonly associated with end-stage renal disease. Calciphylaxis occurring in patients with POEMS syndrome is very rare; only 8 cases have been reported to date. Read More

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January 2021

Peripheral retinal leakage in POEMS syndrome.

Int J Retina Vitreous 2021 Jan 12;7(1). Epub 2021 Jan 12.

Clinical Neuro-Ophthalmology Fellow, Wilmer Eye Institute, 600 N Wolfe Street, Baltimore, MD, 21287, USA.

Background: POEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, skin changes) syndrome is a rare blood disorder with multi-system involvement. The cause is unknown. It is marked by elevated plasma cells, platelets, & VEGF (vascular endothelial growth factor) levels. Read More

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January 2021

Monoclonal Gammopathies of 'Neurological Significance': Paraproteinemic Neuropathies.

Can J Neurol Sci 2021 Jan 5:1-10. Epub 2021 Jan 5.

The Brunei Neuroscience Stroke and Rehabilitation Centre, Brunei Darussalam.

Objectives: To study the clinical profile and outcomes of patients with paraproteinemic neuropathy (PPN) and to explore the utility of nerve conduction studies (NCSs) to differentiate between the demyelinating subtypes.

Methods: We did a retrospective analysis of patients diagnosed with PPN between January 2010 and December 2019 in an inpatient setting. The study population consisted of patients above 16 years of age presenting with clinical features suggestive of chronic peripheral neuropathy and on evaluation was found to have PPN. Read More

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January 2021

Neuroretinitis: A Rare Coinfection in POEMS Syndrome.

Turk J Ophthalmol 2020 12;50(6):371-376

University Sains Malaysia School of Medical Sciences, Department of Internal Medicine, Kelantan, Malaysia.

is a recognized cause of neuroretinitis in cat scratch disease. Meanwhile, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome with Castleman disease (evidence of lymph node hyperplasia), is a chronic debilitating condition that predisposes to various superimposed infections. neuroretinitis implicated in POEMS syndrome has not been reported previously. Read More

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December 2020