1,261 results match your criteria POEMS Syndrome


Temporal dispersion in demyelination of POEMS syndrome and Castleman disease.

Clin Neurophysiol Pract 2020 31;5:112-117. Epub 2020 May 31.

Department of Neurology, Kuala Lumpur Hospital, Jalan Pahang, 50586, Wilayah Persekutuan, Kuala Lumpur, Malaysia.

Objective: We detailed the electrophysiological patterns of peripheral nerve temporal dispersion across spectrum of POEMS syndrome and Castleman disease (CD).

Methods: Compound muscle action potentials (CMAP) duration of 3 patients with POEMS syndrome and 2 with hyaline vascular type CD without clonal plasma cell dyscrasia were retrospectively analysed.

Results: Median and ulnar nerves distal CMAP duration were prolonged in all patients irrespective of plasma cell dyscrasia or M protein. Read More

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http://dx.doi.org/10.1016/j.cnp.2020.05.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322354PMC

Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study.

Neurology 2020 Jun 30. Epub 2020 Jun 30.

From the MRC Centre for Neuromuscular Disease, Neuroimmunology and CSF Laboratory (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), Institute of Neurology, Cancer Division (D.F., J.B., A.C., K.Y., S.D.), Department of Endocrinology (F.C., S.E.B., M.P.L.), and Division of Neuropathology, National Hospital for Neurology and Neurosurgery (Z.J., S.B.), University College London NHS Foundation Trust (S.K.), National Hospital for Neurology and Neurosurgery; Department of Neuromuscular Diseases (S.K., O.J.Z., H.M., A.C., M.M.R., M.P.L.), UCL Queen Square Institute of Neurology; and Department of Clinical Neurosciences (J.F., S.R.), John Radcliffe Hospital, Oxford, UK.

Objective: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin lesions) is a paraneoplastic disorder resulting in severe neurologic disability. Understanding the clinical, laboratory, neurophysiologic, and histopathologic features as well as treatment responses of POEMS will assist in more accurate and timely diagnosis, risk stratification, and effective management.

Methods: This was a retrospective longitudinal cohort study from 1998 to March 2019, with 7,184 person-months of follow-up time. Read More

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http://dx.doi.org/10.1212/WNL.0000000000009940DOI Listing

[Clinical and Pathological Characteristics of Related-Renal Damage in Patients with POEMS Syndrome].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2020 Jun;28(3):977-982

Department of Hematology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China,E-mail:

Objective: To investigated the clinical and pathological characteristics of related-renal damage in patients with POEMS syndrome.

Methods: Five patients diagnosed as POEMS syndrome in our hospital were selected. Their clinical manifestation, pathological characteristics of kidney and laboratory examination were analyzed retrospectively. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2020.03.043DOI Listing

Sclerodermalike syndromes: Great imitators.

Clin Dermatol 2020 Mar - Apr;38(2):235-249. Epub 2019 Oct 24.

Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. Electronic address:

Sclerodermalike syndromes (SLSs) comprise diseases with mucin deposition (eg, scleromyxedema, scleredema), with eosinophilia (eg, eosinophilic fasciitis), metabolic or biochemical abnormalities (eg, nephrogenic systemic fibrosis), or endocrine disorders (eg, POEMS syndrome, or polyneuropathy, organomegaly, endocrinopathy, monoclonal lymphoproliferative disorder, and hypothyroidism). Chronic graft-versus-host disease may also show sclerodermalike skin changes. Inherited progeria syndromes with early aging (eg, Werner syndrome) and a heterogeneous group of hereditary disorders with either skin thickening (eg, stiff skin syndrome) or atrophy and tightening (eg, acrogeria) can also imitate classic systemic sclerosis (SSc). Read More

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http://dx.doi.org/10.1016/j.clindermatol.2019.10.010DOI Listing
October 2019

Diagnosis of paraproteinemic neuropathy: Room for improvement.

J Neurol Sci 2020 May 18;415:116902. Epub 2020 May 18.

Department of Hematology and Oncology, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR OC14HO, USA.

Objective: To report our institutional experience with paraproteinemic neuropathy.

Methods: We reviewed the charts of patients evaluated at our tertiary, academic neuromuscular clinic for neuropathy between 2017 and 2019 and selected those with a serum monoclonal protein. We collected patients' characteristics and reviewed their initial diagnoses and eventual outcomes. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116902DOI Listing

Ascites as a presenting sign of multicentric mixed-type Castleman disease variant of POEMS syndrome.

Hematol Transfus Cell Ther 2020 Mar 20. Epub 2020 Mar 20.

Ampang Hospital, Jalan Mewah Utara, Ampang, Selangor, Malaysia.

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http://dx.doi.org/10.1016/j.htct.2020.01.007DOI Listing

Reliability of optic disc edema area in estimating the severity of papilledema in patients with POEMS syndrome.

Orphanet J Rare Dis 2020 May 19;15(1):116. Epub 2020 May 19.

Department of Hematology, Peking Union Medical College Hospital , Chinese Academy of Medical Sciences, No.1 Shuaifuyuan Wangfujing Dongcheng District, Beijing, 100730, China.

Background: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome involving multisystem. Optic disc edema (ODE) is the most common ocular manifestation in patients with POEMS syndrome and serves as an independent prognostic factor. However, parameters previously used to estimate its severity were inconvenient and costly. Read More

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http://dx.doi.org/10.1186/s13023-020-01392-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7238582PMC

High rates of venous and arterial thrombotic events in patients with POEMS syndrome: results from the UCLH (UK) POEMS Registry.

Blood Adv 2020 May;4(10):2139-2142

Joint POEMS Clinic, Cancer Division, University College Hospital, London, United Kingdom.

Arterial and venous thromboses occur in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein level, and skin changes) syndrome at a previously reported rate of 20%. We reviewed the University College London Hospitals (UCLH) POEMS Registry to determine the rate of venous thromboembolism (VTE), arterial events, and risk factors. This registry, established in 1999 and comprising 103 patients at the time of this study, is the largest single-center cohort in Europe. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020001492DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7252560PMC

Caught in the act: Allergic-like reaction to gadolinium-based contrast agent in POEMS syndrome.

Radiol Case Rep 2020 Jul 1;15(7):887-890. Epub 2020 May 1.

Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.

POEMS syndrome is a rare paraneoplastic condition related to an abnormal proliferation of plasma cells. Allergic-type reactions to gadolinium-based MR contrast media are likewise uncommon adverse events. In this report, we present a highly unusual case involving the collision of these 2 entities. Read More

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http://dx.doi.org/10.1016/j.radcr.2020.04.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200610PMC

Skin and Nerve Neovascularization in POEMS Syndrome: Insights From a Small Cohort.

J Neuropathol Exp Neurol 2020 May;79(5):542-550

Department of Pathology, Limoges, France.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome is a rare systemic disorder linked to plasma cell dyscrasia and is related to elevation of vascular endothelial growth factor (VEGF). Diagnosis is still challenging and pathophysiology unclear. Because VEGF drives neovascularization, we investigated skin and nerve vascularization in 6 patients with POEMS syndrome compared with 5 control groups of polyneuropathies and healthy subjects (n = 104) from the University Hospital of Limoges between 2009 and 2018. Read More

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http://dx.doi.org/10.1093/jnen/nlaa021DOI Listing

[Pitfalls for Diagnosis of Polyneuropathy by Nerve Conduction Study].

Authors:
Sonoko Misawa

Brain Nerve 2020 Apr;72(4):417-423

Department of Neurology, Graduate School of Medicine, Chiba University.

Nerve conduction study is helpful and useful for diagnosing peripheral neuropathies. However, there can be some pitfalls in the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) and its mimics. POEMS syndrome, anti-MAG antibody neuropathy, and amyloid neuropathies can sometimes be diagnosed as CIDP, but their therapeutic strategies are evidently different from that for CIDP. Read More

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http://dx.doi.org/10.11477/mf.1416201540DOI Listing

Rapid growth of congenital angioma in an HIV patient with POEMS syndrome.

Eur J Dermatol 2020 Feb;30(1):66-67

Department of Dermatology, University Hospital Zurich, Zurich, Switzerland, Faculty of Medicine, University of Zurich, Zurich, Switzerland, Medical Campus Davos, Davos, Switzerland.

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http://dx.doi.org/10.1684/ejd.2020.3706DOI Listing
February 2020

Immunoglobulin variable domain high-throughput sequencing reveals specific novel mutational patterns in POEMS syndrome.

Blood 2020 May;135(20):1750-1758

Centre National de la Recherche Scientifique (CNRS) Unité Mixte de Recherche (UMR) 7276/INSERM U1262, Université de Limoges, Limoges, France.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystem disease resulting from an underlying plasma cell (PC) dyscrasia. The pathophysiology of the disease remains unclear, but the role of the monoclonal immunoglobulin (Ig) light chain (LC) is strongly suspected because of the highly restrictive usage of 2 λ variable (V) domains (IGLV1-40 and IGLV1-44) and the general improvement of clinical manifestations after PC clone-targeted treatment. However, the diagnostic value of Ig LC sequencing, especially in the case of incomplete forms of the disease, remains to be determined. Read More

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http://dx.doi.org/10.1182/blood.2019004197DOI Listing

[Effective treatment of POEMS syndrome accompanied by plasmacytoma with lenalidomide, dexamethasone, and local irradiation].

Rinsho Ketsueki 2020 ;61(3):262-267

Department of Hematology and Oncology, Dokkyo Medical University Hospital.

A 70-year-old woman experienced pain in both gastrocnemius muscles, numbness in the toes, and muscle weakness in both the legs that lasted for two months. After getting admitted to our hospital, the muscle weakness extended to both her arms, and nerve conduction studies revealed decreased nerve conduction velocity, which was more prominent in the elbow and the axilla than in the wrist. A magnetic resonance imaging revealed a tumor in the right femoral neck, which was histologically diagnosed as plasmacytoma. Read More

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http://dx.doi.org/10.11406/rinketsu.61.262DOI Listing

Treatment response and prognosis of POEMS syndrome coexisting with Castleman disease.

J Neurol Sci 2020 Jun 6;413:116771. Epub 2020 Mar 6.

Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.

POEMS (polyneuropathy, organomegaly, endocrinopathy monoclonal gammopathy, and skin changes) syndrome is occasionally associated with Castleman disease (CD) and their prognosis is considered as poorer than that in POEMS alone patients. To elucidate recent prognosis of POEMS syndrome coexisting with CD, we reviewed clinical data of 102 patients with POEMS syndrome treated at our institution between 2000 and 2018 and compared clinical characteristics, response to treatment, and prognosis between POEMS patients with biopsy-proven CD (POEMS-CD) and those without it. Fourteen POEMS-CD patients and 56 POEMS alone patients were identified, and the remaining 32 patients with unbiopsied lymphadenopathy were excluded. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116771DOI Listing

Somatic Mutations Confer Severe Peripheral Neuropathy in POEMS Syndrome-Associated Multicentric Castleman Disease.

Neurosci Bull 2020 Jun 12;36(6):664-666. Epub 2020 Mar 12.

Department of Hematology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, 310003, China.

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http://dx.doi.org/10.1007/s12264-020-00481-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7270242PMC

Thrombosis revealing POEMS syndrome. About a case.

Ann Biol Clin (Paris) 2020 Apr;78(2):195-197

Service de médecine interne, Groupement hospitalier Nord, Hôpital Croix-Rousse, Hospices civils de Lyon, France, Université Claude Bernard Lyon 1, Hesper EA 7425, Lyon, France.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare paraneoplastic disorder due to an underlying plasma cell dyscrasia. The diagnosis of POEMS syndrome requires a chronic polyneuropathy and a monoclonal lambda plasma cell-proliferative disorder (mandatory criteria), and various systematic symptoms such as sclerotic bone lesions, Castleman's disease, organomegaly, endocrinopathy, skin changes, papilloedema and biological abnormalities such as elevated vascular endothelial growth factor (VEGF), thrombocytosis or polycythaemia. We describe an observation of a patient with recurrent thrombosis with thrombocytosis that, after excluding a myeloproliferative neoplasm, proved to be due to POEMS syndrome. Read More

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http://dx.doi.org/10.1684/abc.2020.1534DOI Listing

Mass cytometry in POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome: looking for a needle in a haystack.

Authors:
Jo Caers

Br J Haematol 2020 Jul 3;190(1):16-17. Epub 2020 Mar 3.

Laboratory of Hematology, GIGA I3, University of Liège, Liège, Belgium.

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http://dx.doi.org/10.1111/bjh.16554DOI Listing

Recurrent stroke due to quasi-moyamoya disease associated with POEMS syndrome: An autopsy case.

J Neurol Sci 2020 May 15;412:116738. Epub 2020 Feb 15.

Department of Neurology and Neurological Science, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, Japan. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2020.116738DOI Listing
May 2020
2.474 Impact Factor

Mass cytometry identifies expansion of double positive and exhausted T cell subsets in the tumour microenvironment of patients with POEMS syndrome.

Br J Haematol 2020 Jul 20;190(1):79-83. Epub 2020 Feb 20.

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

We sought to dissect the tumour microenvironment in a small cohort (N = 10) of patients with POEMS at diagnosis and after therapy using mass cytometry. We included 10 MGUS patients as controls. We identified 29 immune cell subsets in the CD45 and CD3 compartments. Read More

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http://dx.doi.org/10.1111/bjh.16522DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7329569PMC

Lymphadenopathy in POEMS syndrome: a correlation between clinical features and imaging findings.

Int J Clin Exp Pathol 2020 1;13(1):21-25. Epub 2020 Jan 1.

Ruijin Hospital, Shanghai Jiaotong University School of Medicine Shanghai, PR China.

Lymphadenopathy is an important characteristic of POEMS syndrome, and a Castleman disease (CD)-like pathologic change in the lymph nodes is one of the major diagnostic criteria. However, the characteristics of lymphadenopathy in POEMS still have not been completely elucidated. The lymph node biopsies are available only for a small proportion of patients. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7013372PMC
January 2020

POEMS Syndrome: an Atypical Presentation with Chronic Diarrhoea and Asthenia.

Eur J Case Rep Intern Med 2020 16;7(1):001241. Epub 2019 Dec 16.

Department of Internal Medicine, Egas Moniz Hospital, Lisbon, Portugal.

POEMS syndrome is a rare paraneoplastic condition associated with polyneuropathy, organomegaly, monoclonal gammopathy, endocrine and skin changes. We report a case of a man with Castleman disease and monoclonal gammopathy, with a history of chronic diarrhoea and asthenia. Gastrointestinal involvement in POEMS syndrome is not frequently referred to in the literature and its physiopathology is not fully understood. Read More

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http://dx.doi.org/10.12890/2019_001241DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6993909PMC
December 2019

Lenalidomide Treatment for Thalidomide-refractory POEMS Syndrome: A Prospective Single-arm Clinical Trial.

Intern Med 2020 May 1;59(9):1149-1153. Epub 2020 Feb 1.

Department of Neurology, Graduate School of Medicine, Chiba University, Japan.

Objective A randomized controlled trial has shown the efficacy of thalidomide against polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome; however, there are still refractory patients. We studied the effects of lenalidomide, a derivative of thalidomide, on patients refractory to thalidomide. Methods This prospective single-arm trial evaluated the safety and efficacy of lenalidomide plus dexamethasone in refractory or recurrent patients with POEMS syndrome. Read More

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http://dx.doi.org/10.2169/internalmedicine.3800-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7270764PMC

Posttreated POEMS Syndrome With Concurrent Follicular Lymphoma Revealed by 18F-FDG and 68Ga-Pentixafor PET/CT.

Clin Nucl Med 2020 Mar;45(3):220-222

From the Department of Nuclear Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital.

A 59-year-old man with history of POEMS syndrome was found with a painless mass in the right neck for 9 months. In F-FDG and Ga-pentixafor PET/CT, there were multiple neck nodes and left inguinal nodes that showed intense F-FDG and Ga-pentixafor uptake. In addition, osteoblastic bone lesions with mild F-FDG and Ga-pentixafor uptake were noted in the pelvis, which was consistent with posttreated osseous involvement of POEMS syndrome. Read More

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http://dx.doi.org/10.1097/RLU.0000000000002923DOI Listing

POEMS syndrome: a rare cause of adrenal insufficiency in a young male.

Endocrinol Diabetes Metab Case Rep 2019 Sep 17;2019. Epub 2019 Sep 17.

Departments of Endocrinology, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal.

Summary: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a rare multisystemic disease. Clinical presentation is variable, the only mandatory criteria being polyneuropathy and monoclonal gammapathy in association with one major and one minor criterion. Primary adrenal insufficiency is rarely reported. Read More

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http://dx.doi.org/10.1530/EDM-19-0010DOI Listing
September 2019

Effect of UVA1 on hypertrophic scarring in the rabbit ear model.

Biosci Rep 2020 Jan;40(1)

Department of Orthopaedics, Gongli Hospital of Shanghai Pudong New Area, Shanghai 200135, China.

Hypertrophic scars (HTSs) are common and cause functional and psychological morbidity. UVA1 (340-400 nm) phototherapy has been previously shown to be effective in the treatment of localized scleroderma, systemic sclerosis, and POEMS syndrome with minimal side effects, all of which are presented as collagen fibrils hyperplasia that is common with scarring in skin histology. In the present study, we aimed to investigate the impact of UVA1 on the protein expression of TGF-β signal pathway and myofibroblasts in a rabbit model of cutaneous scarring. Read More

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http://dx.doi.org/10.1042/BSR20190007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6974420PMC
January 2020

Polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin (POEMS) changes syndrome presenting with a pseudosensory level: a case report.

J Med Case Rep 2019 Dec 27;13(1):384. Epub 2019 Dec 27.

University Medical Unit, National Hospital of Sri Lanka, Colombo, Sri Lanka.

Introduction: Polyneuropathy is a key feature of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome, which is a paraneoplastic manifestation of an underlying lymphoproliferative neoplasm. We report the first case of polyneuropathy, organomegaly, endocrinopathy, monoclonal band, and skin changes syndrome presenting with a pseudosensory level.

Case Presentation: A 59-year-old Tamil woman with long-standing diabetes mellitus and hypertension developed painless, progressive inguinal lymphadenopathy. Read More

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http://dx.doi.org/10.1186/s13256-019-2309-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6933708PMC
December 2019

Living with Polyneuropathy Organomegaly Endocrinopathy Monoclonal gammopathy Skin changes (POEMS) syndrome: a case study of healthcare experiences and quality of life.

Disabil Rehabil 2019 Dec 14:1-9. Epub 2019 Dec 14.

The Department of Clinical Neuropsychology, Salford Royal NHS Foundation Trust, Northern Care Alliance, Salford, UK.

Polyneuropathy Organomegaly Endocrinopathy Monoclonal gammopathy Skin changes (POEMS) Syndrome is a rare condition with an estimated prevalence rate of 0.3 per 100,000 people. Patient perspectives on healthcare experiences and quality of life have not yet been studied in depth. Read More

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http://dx.doi.org/10.1080/09638288.2019.1700563DOI Listing
December 2019

POEMS syndrome: a unique presentation and a diagnostic challenge.

BMJ Case Rep 2019 Dec 3;12(12). Epub 2019 Dec 3.

Rheumatology, Mater Dei Hospital, Msida, Malta.

We present the case of a previously well, 70-year-old lady who presented with gait deterioration and a clinicoradiological picture of myositis with T2-enhancement on MRI of the distal lower limb muscles. A muscle biopsy pointed towards neurogenic amyotrophy and a mixed demyelinating and axonal sensorimotor polyneuropathy was confirmed on nerve conduction studies. This was initially thought to be secondary to a myeloproliferative disorder but a positron emission tomography scan showed uptake in the left hip which corresponded to what was previously reported as a subchondral cyst on CT. Read More

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http://dx.doi.org/10.1136/bcr-2019-230284DOI Listing
December 2019

[A Case of POEMS Syndrome Treated by Ld Prior to HDCTx Followed by aPBSCT].

Gan To Kagaku Ryoho 2019 Nov;46(11):1799-1802

Dept. of Hematology, Kanto Rosai Hospital.

A man in his late 40s was presented to a hospital with complaints of peripheral numbness and fatigue. Systemic edema, pleural effusion and ascites, pigmentation, splenomegaly, and CT findings of osteoplastic changes suggested POEMS syndrome. He was referred to our division, and a bone marrow examination indicated MGUS. Read More

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November 2019

Distinctive patterns of peripheral neuropathy across the spectrum of plasma cell disorders.

Sci Rep 2019 11 14;9(1):16769. Epub 2019 Nov 14.

Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, South Korea.

Many patients with plasma cell disorders suffer from peripheral neuropathy, but differential diagnosis with chronic inflammatory demyelinating polyneuropathy (CIDP) is difficult. We aimed to (1) identify factors useful for differential diagnosis between peripheral neuropathy associated with plasma cell disorders versus CIDP and (2) determine whether neuropathy presentations and severity varied across the spectrum of different plasma cell disorders. A retrospective chart review of 18 monoclonal gammopathy of unknown significance (MGUS) patients, 15 POEMS syndrome patients and 34 CIDP patients between January 2005 and December 2016 was conducted. Read More

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http://dx.doi.org/10.1038/s41598-019-53289-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6856063PMC
November 2019

Evaluating heart function in patients with POEMS syndrome.

Echocardiography 2019 11 6;36(11):1997-2003. Epub 2019 Nov 6.

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

Aims: Our aim is to investigate the characterized echocardiographic cardiac measurements of POEMS syndrome and determine its relationship with clinical manifestations.

Methods And Results: The cross-sectional study included 27 treatment-naïve patients with newly diagnosed POEMS syndrome and 26 age- and sex-matched healthy volunteers. Information of clinical manifestations, serological tests, pulmonary function tests, and both conventional echocardiograph and tissue Doppler imaging (TDI) were collected and analyzed. Read More

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http://dx.doi.org/10.1111/echo.14516DOI Listing
November 2019

Outpatient Autologous Stem Cell Transplants for Multiple Myeloma: Analysis of Safety and Outcomes in a Tertiary Care Center.

Clin Lymphoma Myeloma Leuk 2019 12 9;19(12):784-790. Epub 2019 Oct 9.

Leukemia/Bone Marrow Transplant Program of British Columbia, Vancouver General Hospital, BC Cancer, Vancouver, BC, Canada. Electronic address:

Background: Autologous stem cell transplant (ASCT) is the preferred consolidation strategy to treat eligible patients with multiple myeloma (MM) and related plasma cell dyscrasias. Given the increasing volume of patients and longer wait time, outpatient ASCT for MM is the standard of care at the Vancouver General Hospital.

Patients And Methods: Patients with MM, POEMS syndrome, and amyloidosis undergoing ASCT were included in this analysis. Read More

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http://dx.doi.org/10.1016/j.clml.2019.09.619DOI Listing
December 2019

An atypical presentation of POEMS syndrome.

Presse Med 2019 Oct 23;48(10):1190-1193. Epub 2019 Oct 23.

CHU de MohammedVI, service de neurologie, Marrakech, Morocco.

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http://dx.doi.org/10.1016/j.lpm.2019.08.022DOI Listing
October 2019
1.173 Impact Factor

Reversal of skin changes in smoldering myeloma with clinical presentation of POEMS syndrome with a lenalidomide-based regimen.

Ann Hematol 2019 Nov 25;98(11):2625-2626. Epub 2019 Oct 25.

Department of Hematology, University of Patras Medical School, Rion, Greece.

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http://dx.doi.org/10.1007/s00277-019-03818-4DOI Listing
November 2019

Papillary Hemangioma: An Under-Recognized Entity Not to Be Confused With Glomeruloid Hemangioma.

Am J Dermatopathol 2020 Mar;42(3):211-214

Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Lebanon, NH.

Papillary hemangioma is a recently described benign hemangioma that typically presents on the head and neck of otherwise healthy individuals. It comprises branching papillary structures that protrude into the lumen of ectatic thin-walled vessels. Glomeruloid hemangioma (GH) is a similar, but unique, entity that occurs in patients with POEMS syndrome. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001546DOI Listing

Paraneoplastic Syndromes in Neuro-ophthalmology.

Continuum (Minneap Minn) 2019 Oct;25(5):1401-1421

Purpose Of Review: This article discusses the varied types of paraneoplastic syndromes that commonly have neuro-ophthalmologic manifestations. Diagnostic considerations and therapeutic options for individual diseases are also discussed.

Recent Findings: Paraneoplastic syndromes can affect the afferent and efferent visual systems. Read More

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http://dx.doi.org/10.1212/CON.0000000000000788DOI Listing
October 2019
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Anaesthetic Challenges in a Rare Syndrome: Perioperative Management of a Patient with POEMS Syndrome Who Underwent Umbilical Hernioplasty.

Turk J Anaesthesiol Reanim 2019 Oct 15;47(5):420-422. Epub 2019 May 15.

Sree Chitra Tirunal Institute For Medical Sciences, Kerala, India.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome also known as 'Crow Fukase syndrome' is a rare paraneoplastic disorder, first described by Crow and Fukase with distinctive features of polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. There is a paucity of literature about anaesthetic management of patients with POEMS syndrome with isolated case reports of surgery under general anaesthesia and central neuraxial blockade. We present here the anaesthetic management of a patient with POEMS syndrome posted for umbilical hernia repair, which was successfully managed with a transverse abdominis plane (TAP) block. Read More

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http://dx.doi.org/10.5152/TJAR.2019.53824DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6756301PMC
October 2019
2 Reads

[POEMS syndrome: advances in molecular pathophysiology and treatment].

Authors:
Chiaki Nakaseko

Rinsho Ketsueki 2019 ;60(8):979-987

Department of Hematology, International University of Health and Welfare School of Medicine.

POEMS syndrome is a rare paraneoplastic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, λ-type monoclonal protein derived from only two germlines (IGLV1-40 or IGLV1-44), skin changes, extravascular volume overload, and serum vascular endothelial growth factor elevation. To understand the molecular pathophysiology of the disease, comprehensive genetic analyses of bone marrow plasma cells was performed in 20 patients with the syndrome. Although a median of 14. Read More

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http://dx.doi.org/10.11406/rinketsu.60.979DOI Listing
September 2019
3 Reads

Dermatology in the Diagnosis of Noncutaneous Malignancy: Paraneoplastic Diseases.

Dermatol Clin 2019 Oct 10;37(4):537-544. Epub 2019 Jul 10.

Oregon Health & Science University, 3303 Southwest Bond Avenue CH16D, Portland, OR 97239, USA.

It is important to recognize paraneoplastic dermatoses because they allow the practitioner to begin an early, directed workup to detect an underlying malignant neoplasm. In this review, several paraneoplastic dermatoses are outlined using existing data to detail each one's association with underlying malignancy, demographics, prognosis, and treatment considerations. Read More

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http://dx.doi.org/10.1016/j.det.2019.05.011DOI Listing
October 2019
5 Reads

TAFRO Syndrome: A Case of Significant Endocrinopathy in a Caucasian Patient.

Cureus 2019 Jun 19;11(6):e4946. Epub 2019 Jun 19.

Hematology & Oncology, Baton Rouge General Medical Center, Baton Rouge, USA.

Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disorder that manifests as multiorgan dysfunction secondary to widespread inflammation. The underlying pathogenesis is driven by an excessive and inappropriate cytokine storm. TAFRO syndrome is a rare subtype of iMCD, characterized by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Read More

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http://dx.doi.org/10.7759/cureus.4946DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6701897PMC
June 2019
4 Reads

Peripheral Blood Hemopoietic Stem Cell Mobilization Regimens in POEMS Syndrome: A Retrospective Study at 2 Hematologic Italian Centers.

Biol Blood Marrow Transplant 2019 12 21;25(12):2514-2516. Epub 2019 Aug 21.

Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Istituto di Ematologia, Università Cattolica del Sacro Cuore, Rome, Italy.

Autologous peripheral blood stem cell transplantation should be considered first-line therapy in young patients with POEMS. The best protocol to collect peripheral blood stem cells remains to be defined, because of the disease rarity and the heterogeneity of published case series. We collected clinical and laboratory data from 25 patients undergoing mobilization, of whom 11 were mobilized using cyclophosphamide (CY) followed by granulocyte colony-stimulating factor (G-CSF) and 14 patients using G-CSF. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.08.011DOI Listing
December 2019
6 Reads

POEMS syndrome: A rare paraneoplastic presentation of spinal plasmacytoma.

Med J Malaysia 2019 08;74(4):335-337

Hospital Tengku Ampuan Afzan Kuantan, Department of Medicine, Pahang, Malaysia.

The acronym POEMS syndrome was coined for a unique multisystem disorder characterised by peripheral neuropathy, organomegaly, endocrinopathies, monoclonal gammopathy and skin changes. We report a male patient presenting to us with spinal plasmacytoma complicated with paraplegia. He was subsequently diagnosed to have POEMS syndrome and successfully treated with thalidomide and dexamethasone. Read More

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August 2019
2 Reads

A relapse of POEMS syndrome presenting as acute kidney injury.

BMJ Case Rep 2019 Aug 15;12(8). Epub 2019 Aug 15.

Nephrology and Transplant Department, University Hospital of Wales, Cardiff, UK.

Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes (POEMS) syndrome is a rare disease, and only in a minority of cases, causes an impairment of kidney function. Here, we describe a case of a 55-year-old man with a history of POEMS syndrome who presented with acute kidney injury following a routine blood test. On further investigation, a relapse in POEMS syndrome was diagnosed, uniquely isolated to renal involvement. Read More

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http://dx.doi.org/10.1136/bcr-2018-228894DOI Listing
August 2019
2 Reads

Osteosclerotic myeloma without features of POEMS syndrome.

Int J Hematol 2019 Nov 12;110(5):517-518. Epub 2019 Aug 12.

Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8602, Japan.

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http://dx.doi.org/10.1007/s12185-019-02718-7DOI Listing
November 2019
2 Reads

Multiparameter Flow Cytometry for the Identification of Neoplastic Plasma Cells in POEMS Syndrome with IgG-kappa Gammopathy: Successful Treatment Using Lenalidomide and Dexamethasone.

Intern Med 2019 Dec 6;58(23):3461-3468. Epub 2019 Aug 6.

Department of Hematology, Nephrology and Rheumatology, Akita University Graduate School of Medicine, Japan.

A 72-year-old man presented with a 6-month history of systemic edema. Hyperpigmentation, hemangioma, pleural effusion, IgG-kappa-type monoclonal protein, high vascular endothelial growth factor values, renal failure, and nerve conduction study abnormalities were also present. Multiparameter flow cytometry (MFC) showed 0. Read More

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http://dx.doi.org/10.2169/internalmedicine.2882-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6928515PMC
December 2019
4 Reads

Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey.

Neurology 2019 09 1;93(10):e975-e983. Epub 2019 Aug 1.

From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.

Objective: To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome.

Methods: We conducted a nationwide survey in 2015 using an established epidemiologic method. Data processing sheets were sent to all neurology and hematology specialist departments throughout Japan to identify patients with POEMS who were seen between April 2012 and March 2015. Read More

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http://dx.doi.org/10.1212/WNL.0000000000008062DOI Listing
September 2019
2 Reads

Irreversible Vasculopathy Proceeds Rapidly in POEMS Syndrome.

Intern Med 2019 Dec 31;58(24):3573-3575. Epub 2019 Jul 31.

Department of Neurology, Kashiwa Hospital, The Jikei University School of Medicine, Japan.

A 48-year-old woman with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change (POEMS) syndrome suddenly presented with numbness of her right upper limb. Magnetic resonance imaging showed multiple acute infarctions in her left cerebrum, and magnetic resonance angiography (MRA) showed multiple intra-cranial vascular lesions, which contrasted with previously normal MRA results obtained eight months prior to the stroke. After completing successful treatment for POEMS syndrome, there were no recurrent stroke episodes. Read More

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http://dx.doi.org/10.2169/internalmedicine.3279-19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6949451PMC
December 2019
3 Reads

Castleman Disease.

Surg Pathol Clin 2019 Sep 23;12(3):849-863. Epub 2019 May 23.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA. Electronic address:

Castleman disease (CD) is divided clinically into unicentric or multicentric type. Pathologically, CD is divided into hyaline-vascular and plasma cell variants. Unicentric CD is most common, about 75% of these cases are hyaline-vascular variant, and surgical excision is often curative. Read More

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http://dx.doi.org/10.1016/j.path.2019.03.003DOI Listing
September 2019
4 Reads