1,172 results match your criteria POEMS Syndrome


Neurological impairment in a patient with concurrent cervical disc herniation and POEMS syndrome.

Eur Spine J 2019 Feb 13. Epub 2019 Feb 13.

Department of Orthopedics, West China Hospital of Sichuan University, Guoxuexiang No. 37, Wuhouqu, Chengdu, 610041, Sichuan, China.

Purpose: POEMS syndrome is a rare clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. We report a rare case of neurological impairment in patients with concurrent cervical disc herniation and POEMS syndrome.

Methods: A patient presented to a local hospital with C3/4 and C4/5 disc herniation, apparent spinal cord compression concomitant with neurological signs, and concurrent POEMS syndrome. Read More

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http://link.springer.com/10.1007/s00586-019-05914-5
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http://dx.doi.org/10.1007/s00586-019-05914-5DOI Listing
February 2019
2 Reads

Frequent central nervous system, pachymeningeal and plexus MRI changes in POEMS syndrome.

J Neurol 2019 Feb 12. Epub 2019 Feb 12.

MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK.

Objective: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare multisystem disease associated with a plasma-cell dyscrasia. Although pachymeningeal involvement has occasionally been described, MRI of the central nervous system (CNS) has not yet been extensively investigated.

Methods: We retrospectively evaluated CNS MRI in Europe's largest single-center cohort of POEMS syndrome. Read More

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http://link.springer.com/10.1007/s00415-019-09233-z
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http://dx.doi.org/10.1007/s00415-019-09233-zDOI Listing
February 2019
4 Reads

Positron Emission Tomography-Computed Tomography in a Case of POEMS Syndrome.

Indian J Nucl Med 2019 Jan-Mar;34(1):66-68

Department of Nuclear Medicine, Hospital Infanta Cristina, Badajoz, Spain.

The POEMS syndrome is a rare and multisystemic disease characterized by the presence of polyneuropathy, organomegaly, endocrinopathy, presence of M protein, and alterations in the skin. The existence of bone lesions is frequent, being generally blastic or mixed and rarely lytic. We present the case of a 54-year-old male patient diagnosed with POEMS syndrome, with atypical presence of lytic lesions, who was referred for an 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT) study for initial staging of the process. Read More

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http://dx.doi.org/10.4103/ijnm.IJNM_120_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352633PMC
February 2019

What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone?

Leukemia 2019 Jan 30. Epub 2019 Jan 30.

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. This study compared the responses to and survival of 347 POEMS syndrome patients given three first-line treatment regimens: autologous stem cell transplantation (ASCT, N = 165) and melphalan + dexamethasone (MDex, N = 79), or lenalidomide + dexamethasone (LDex, N = 103). After a median 45-month follow-up, overall hematologic complete remission (CR) was 46. Read More

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http://dx.doi.org/10.1038/s41375-019-0391-2DOI Listing
January 2019
3 Reads

Bortezomib-based regimens in patients with POEMS syndrome: a case series in newly diagnosed and relapsed patients.

Leuk Lymphoma 2019 Jan 22:1-4. Epub 2019 Jan 22.

b Neurology Unit, Department of Neuroscience , University of Padova , Padova , Italy.

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http://dx.doi.org/10.1080/10428194.2018.1564048DOI Listing
January 2019
1 Read

Glomeruloid Hemangioma as a Late Manifestation of POEMS Syndrome.

Acta Dermatovenerol Croat 2018 Dec;26(4):321-324

Professor Marcia Ramos-e-Silva, MD, PhD, Rua Dona Mariana 143 / C-32 , Rio de Janeiro 22280-020 , Brazil;

Glomeruloid hemangiomas were first described by Chan in 1990 as a cutaneous marker of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal proteinemia, and Skin changes). POEMS syndrome is a multisystem disease with a significant cutaneous involvement. Among its manifestations, hemangiomas are common, observed in up to one third of the patients, and, although specific, the glomeruloid subtype is very rare. Read More

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December 2018
1 Read

Genetic and transcriptional landscape of plasma cells in POEMS syndrome.

Leukemia 2019 Jan 11. Epub 2019 Jan 11.

Department of Hematology, Chiba University Hospital, Chiba, Japan.

POEMS syndrome is a rare paraneoplastic disease associated with monoclonal plasma cells; however, the pathogenic importance of plasma cells remains unclear. We performed comprehensive genetic analyses of plasma cells in 20 patients with POEMS syndrome. Whole exome sequencing was performed in 11 cases and found a total of 308 somatic mutations in 285 genes. Read More

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http://www.nature.com/articles/s41375-018-0348-x
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http://dx.doi.org/10.1038/s41375-018-0348-xDOI Listing
January 2019
2 Reads

Tufted-angioma-like lesion associated with vascular endothelial growth factor and interleukin-6 in TAFRO syndrome: Is it a common histological feature of multicentric Castleman disease/POEMS syndrome?

J Cutan Pathol 2019 Jan 10. Epub 2019 Jan 10.

Department of General Medicine, Tenri Hospital, Nara, Japan.

The histology of skin lesions of TAFRO (thrombocytopenia, anasarca, reticulin fibrosis/renal failure, and organomegaly) syndrome has rarely been reported. We report herein two cases of TAFRO syndrome with characteristic vascular skin lesions. The lesions resembled a tufted angioma, although those of case 1 partially resembled a glomeruloid hemangioma, which was known as a specific lesion in POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes), a variant of multicentric Castleman disease (MCD). Read More

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http://dx.doi.org/10.1111/cup.13415DOI Listing
January 2019
1 Read

Clinical characteristics and the long-term outcome of patients with atypical POEMS syndrome variant with undetectable monoclonal gammopathy.

Ann Hematol 2019 Mar 5;98(3):735-743. Epub 2019 Jan 5.

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

The diagnosis of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome requires polyneuropathy and monoclonal plasma cell proliferation as two mandatory criteria. Our aim was to summarize clinical manifestations and treatment responses of POEMS variants with no evidence of monoclonal gammopathy. We queried all medical documentation of patients referred to Peking Union Medical College Hospital from August 2012 to July 2017, and reviewed the clinical and laboratory features of 13 patients with atypical POEMS syndrome with undetectable monoclonal gammopathy, and compared to prototypes published. Read More

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http://link.springer.com/10.1007/s00277-018-03589-4
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http://dx.doi.org/10.1007/s00277-018-03589-4DOI Listing
March 2019
8 Reads

[POEMS syndrome].

Ann Dermatol Venereol 2019 Jan 28;146(1):82-85. Epub 2018 Dec 28.

Service de dermatologie, hôpital Claude-Huriez, CHU de Lille, rue Michel-Polonowski, 59037 Lille cedex, France; Université de Lille, 59037 Lille, France.

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http://dx.doi.org/10.1016/j.annder.2018.11.003DOI Listing
January 2019
1 Read

[Video-assisted Thoracoscopic Pericardial Fenestration for Pericardial Effusion in a Patient with Crow-Fukase Syndrome].

Kyobu Geka 2018 12;71(13):1092-1095

Department of Cardiovascular Surgery, National Hospital Organization Yokohama Medical Center, Yokohama, Japan.

A 65-year-old woman was referred to our department with recurrent pericardial effusion. Her serum vascular endothelial growth factor was high, serum M-protein was positive, and nerve conduction velocity of extremities was decreased. Therefore, she was diagnosed with Crow-Fukase (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes:POEMS) syndrome, which is characterized by the presence of plasma cell dyscrasia with them. Read More

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December 2018
3 Reads

Glomeruloid haemangioma: a possible consequence of elevated VEGF in POEMS and Erdheim-Chester disease.

Eur J Dermatol 2018 Dec;28(6):784-789

Unité fonctionnelle de dermatologie, hôpitaux universitaires Pitié-Salpêtrière-C. Foix, AP-HP, Sorbonne Universités, UPMC université Paris 06, Paris, France.

Glomeruloid haemangioma (GH) is considered a specific marker of POEMS syndrome, despite some published GH cases unrelated to POEMS syndrome. To present two cases with GH and atypical presentations of Erdheim-Chester disease (ECD) or POEMS syndrome, as well as a retrospective monocentric study of histologically-confirmed GH. Clinical, biological and histological data of the patients is presented. Read More

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http://dx.doi.org/10.1684/ejd.2018.3451DOI Listing
December 2018
3 Reads
1.990 Impact Factor

Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome.

Can J Ophthalmol 2018 Dec 7;53(6):e233-e236. Epub 2018 Jul 7.

Chinese PLA General Hospital, Beijing, P. R. China. Electronic address:

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http://dx.doi.org/10.1016/j.jcjo.2018.02.015DOI Listing
December 2018
2 Reads

POEMS syndrome: clinical update.

J Neurol 2019 Jan 29;266(1):268-277. Epub 2018 Nov 29.

Department of Neurology, Royal Free Hospital, Pond Street, London, NW3 2QG, UK.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. Read More

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http://link.springer.com/10.1007/s00415-018-9110-6
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http://dx.doi.org/10.1007/s00415-018-9110-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342878PMC
January 2019
4 Reads

Improvement of thyroid function in POEMS syndrome after combination therapy of lenalidomide and dexamethasone.

Leuk Lymphoma 2018 Nov 8:1-5. Epub 2018 Nov 8.

b Department of Hematology , Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College , Beijing , People's Republic of China.

Hypothyroidism is the most common endocrine disorder in POEMS syndrome. In this single-center prospective study, 36 patients with newly diagnosed POEMS syndrome were treated with lenalidomide (10 mg daily for 21 days) and dexamethasone (40mg once per week) for 12 cycles. Hypothyroidism was demonstrated in 58. Read More

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http://dx.doi.org/10.1080/10428194.2018.1485909DOI Listing
November 2018
3 Reads

Anti-BCMA CAR-T cells for treatment of plasma cell dyscrasia: case report on POEMS syndrome and multiple myeloma.

J Hematol Oncol 2018 Oct 22;11(1):128. Epub 2018 Oct 22.

Department of Hematology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, 1095 Jie-Fang Avenue, Wuhan, 430030, Hubei, People's Republic of China.

Background: POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome still has no standard treatment. On the basis that both POEMS syndrome and myeloma have an underlying plasma cell dyscrasia, anti-myeloma therapy can be expected to be useful for POEMS syndrome. Chimeric antigen receptor T (CAR-T) cells targeting B cell maturation antigen (BCMA) has been used in the treatment of relapsed and refractory multiple myeloma (RRMM). Read More

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https://jhoonline.biomedcentral.com/articles/10.1186/s13045-
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http://dx.doi.org/10.1186/s13045-018-0672-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198365PMC
October 2018
9 Reads
4.812 Impact Factor

POEMS syndrome complicated with multiple ischemic vascular events: case report and review of literature.

Onco Targets Ther 2018 27;11:6271-6276. Epub 2018 Sep 27.

Department of Hematology, University Emergency Hospital Bucharest, Bucharest, Romania,

POEMS syndrome (acronym consisting of: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is an uncommon disorder associated with an underlying plasma cell dyscrasia. There is no single specific test for POEMS, and due to its rarity and heterogeneity, patients are often mis- or underdiagnosed. Castleman disease (CD) is a rare lymphoproliferative disorder, closely related to POEMS syndrome; ~11%-30% of POEMS patients are associated with concomitant CD. Read More

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https://www.dovepress.com/poems-syndrome-complicated-with-mu
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http://dx.doi.org/10.2147/OTT.S146221DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6166759PMC
September 2018
3 Reads

Castleman disease of the hyaline vascular variant transforming to POEMS syndrome as endpoint: a case report.

BMC Neurol 2018 Oct 9;18(1):169. Epub 2018 Oct 9.

Oncology Department, the 1st Affiliated Hospital of Anhui Medical University, Jixi Road 218, Hefei, 230022, China.

Background: POEMS syndrome is a rare neoplastic syndrome reflected by plasma cell disorder. It is composed by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. It is also reported to associate with Castleman disease. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-018-1172-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6176504PMC
October 2018
1 Read

Proposal of new clinical diagnostic criteria for POEMS syndrome.

J Neurol Neurosurg Psychiatry 2019 Feb 2;90(2):133-137. Epub 2018 Oct 2.

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

Objective: To propose the optimal diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome using appropriate statistical methods and disease controls.

Methods: This retrospective cohort study included 104 consecutive patients with suspected POEMS syndrome, among whom a gold standard group of 60 patients with definitive POEMS syndrome diagnosis were followed for at least 12 months to strictly exclude other disorders mimicking POEMS syndrome and to confirm response to POEMS syndrome-specific treatment. Thirty patients with chronic inflammatory demyelinating polyradiculoneuropathy (demyelinating polyradiculoneuropathy controls) and 30 with multiple myeloma or immunoglobulin light chain amyloidosis (monoclonal plasma cell proliferation controls) were also included. Read More

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http://jnnp.bmj.com/lookup/doi/10.1136/jnnp-2018-318514
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http://dx.doi.org/10.1136/jnnp-2018-318514DOI Listing
February 2019
4 Reads

Increased Risk of Ischemic Stroke in Multiple Myeloma Associated With Lenalidomide Treatment: A Case Report and Review of the Literature.

Clin Neuropharmacol 2018 Nov/Dec;41(6):232-235

Department of Neurology and Stroke Center, La Paz University Hospital, Autonomous University of Madrid, La Paz University Hospital Health Research Institute (IdiPAZ), Madrid, Spain.

Objective: The aim of the study was to illustrate the increased risk of ischemic stroke in the context of multiple myeloma (MM) under treatment with lenalidomide combined with dexamethasone.

Methods: This is a case report and literature review.

Results: A 62-year-old woman diagnosed with relapsed MM under treatment with lenalidomide and dexamethasone presented with acute onset disorientation, disturbed behavior, and aphasia. Read More

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http://dx.doi.org/10.1097/WNF.0000000000000310DOI Listing
December 2018
20 Reads

An update on the diagnosis and management of the polyneuropathy of POEMS syndrome.

J Neurol 2019 Jan 27;266(1):258-267. Epub 2018 Sep 27.

Neuropathology Unit, Division of Neuroscience and Department of Neurology, Institute of Experimental Neurology, San Raffaele Scientific Institute, Via Olgettina 48, 20132, Milan, Italy.

POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Read More

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http://link.springer.com/10.1007/s00415-018-9068-4
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http://dx.doi.org/10.1007/s00415-018-9068-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343023PMC
January 2019
6 Reads

Prevalence and course of endocrinopathy in POEMS syndrome.

J Clin Endocrinol Metab 2018 Sep 17. Epub 2018 Sep 17.

Department of Endocrinology, University College London Hospitals NHS Foundation Trust, London, UK.

Context: POEMS syndrome is a rare multisystem disorder characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder and skin changes among other features.

Objective: To describe the prevalence and course of the endocrine dysfunction in the context of POEMS.

Design: Cohort study with systematic review of the endocrinopathy in POEMS. Read More

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http://dx.doi.org/10.1210/jc.2018-01516DOI Listing
September 2018
2 Reads

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome complicated by ischemic stroke: A case report.

Iran J Neurol 2018 Apr;17(2):95-96

Department of Neurology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131334PMC

Diagnostic framing of IgM monoclonal gammopathy: Focus on Waldenström macroglobulinemia.

Hematol Oncol 2018 Sep 7. Epub 2018 Sep 7.

Division of Hematology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy.

The finding of an IgM monoclonal gammopathy often represents a diagnostic challenge. In fact, there are many pathological disorders associated with this condition, each of which has distinctive characteristics and requires specific clinical, instrumental, and laboratory assessments to set the appropriate treatment. This review has two aims. Read More

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http://dx.doi.org/10.1002/hon.2539DOI Listing
September 2018
7 Reads

Raised VEGF: High sensitivity and specificity in the diagnosis of POEMS syndrome.

Neurol Neuroimmunol Neuroinflamm 2018 Sep 15;5(5):e486. Epub 2018 Aug 15.

MRC Centre for Neuromuscular Diseases (M.P., S.K., M.M.R., M.P.L.), National Hospital for Neurology and Neurosurgery, Queen Square, London, UK; Cancer Division (S.D., K.L.Y.), University College London Hospitals NHS Foundation Trust, London, UK; and Department of Neuroimmunology (A.J.C., M.P.L.), Institute of Neurology, Queen Square, London, UK.

Objective: To investigate the sensitivity and the specificity of serum vascular endothelial growth factor (sVEGF) for the diagnosis of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome in patients with a neuropathy (NP) and to identify confounding causes of raised vascular endothelial growth factor (VEGF) in this context to improve accuracy.

Methods: We studied the specificity and sensitivity of sVEGF for the diagnosis of POEMS syndrome in a cohort of 195 consecutive patients with an NP in serum samples from June 2009 to November 2013, including 27 untreated patients with POEMS syndrome. We then studied VEGF in other neuropathies and analyzed causes of elevated VEGF in a multiple logistic regression analysis in a larger cohort of 236 patients including 168 with a non-POEMS NP and 68 without NP. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000486DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117193PMC
September 2018
17 Reads

Impact of lenalidomide exposure on blood cell collection for autotransplants in persons with POEMS syndrome.

Arch Med Sci 2018 Aug 2;14(5):1048-1054. Epub 2016 Nov 2.

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

Introduction: Lenalidomide is an effective therapy of POEMS syndrome. However, there is concern that exposure to lenalidomide may reduce the efficiency of blood cell collection in persons who may eventually receive an autotransplant. We studied the impact of lenalidomide therapy on subsequent blood cell mobilization and collection including frequency of blood CD34+ cells and CXCR4 expression before and after mobilization with cyclophosphamide and granulocyte-colony stimulating factor (G-CSF). Read More

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http://dx.doi.org/10.5114/aoms.2016.63353DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6111355PMC
August 2018
12 Reads
1.890 Impact Factor

POEMS syndrome.

Curr Opin Neurol 2018 Oct;31(5):551-558

Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.

Purpose Of Review: To provide an overview of polyneuropathy organomegaly endocrinopathy M-protein and skin changes (POEMS) syndrome, detailing new insights into pathogenesis, prognostic factors, treatments, and outcome scores.

Recent Findings: With the development of large multicentre national cohorts of patients, POEMS syndrome is evolving into a well characterized multisystem hematoneurological syndrome. Without early diagnosis significant disability results from the neuropathy. Read More

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http://dx.doi.org/10.1097/WCO.0000000000000610DOI Listing
October 2018
18 Reads

[Progress in Treatment of POEMS Syndrome--Review].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Aug;26(4):1225-1229

Department of Hematology, Center for Hematologic Diseases of Chinese PLA, Lanzhou Military Command General Hospital, Lanzhou 730050, Gansu Proince, China.E-mail:

POEMS syndrome is a rare multiple organ involvement of the parasympathetic syndrome associated with abnormal plasma cells, mostly with high-dose chemotherapy and stem cell transplantation for the treatment. Recently, more treatment attempts to treat POEMS syndrome have been utilized so as to improve the efficacy and safety for the patients with POEMS syndrome, such as immunomodulator, alkylating agent, cytokine-induced killer cells and so on. Lenalidomide has a significant effect on relapse/refractory POEMS syndrome and patients with endocrinopathy. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2018.04.047DOI Listing
August 2018
1 Read

Serum vascular endothelial growth factor (VEGF) level can be used as a diagnostic marker in POEMS syndrome.

Pathology 2018 Oct 3;50(6):674-676. Epub 2018 Aug 3.

Department of Clinical Immunology and Allergy, Royal North Shore Hospital, Sydney, NSW, Australia; Immunorheumatology Laboratory, New South Wales Health Pathology, Sydney, NSW, Australia; The University of Sydney, Sydney, NSW, Australia.

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http://dx.doi.org/10.1016/j.pathol.2018.03.010DOI Listing
October 2018
1 Read

POEMS syndrome: diagnostic delay and successful treatment with lenalidomide, cyclophosphamide and prednisone followed by autologous peripheral stem cell transplantation.

BMJ Case Rep 2018 Jul 26;2018. Epub 2018 Jul 26.

Internal Medicine, Walter Reed National Military Medical Center, Bethesda, Maryland, USA.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare systemic disease, often unrecognised in the primary care setting. POEMS syndrome is associated with plasma cell dyscrasias and upregulation of vascular endothelial growth factor leading to systemic oedema, papilloedema and pulmonary hypertension. A wide constellation of presenting symptoms often leads to late diagnosis. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2017-22316
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http://dx.doi.org/10.1136/bcr-2017-223168DOI Listing
July 2018
16 Reads

European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias.

Leukemia 2018 Sep 23;32(9):1883-1898. Epub 2018 Jul 23.

Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.

The introduction of novel agents in the management of multiple myeloma and related plasma cell dyscrasias has changed our treatment approaches and subsequently the outcome of patients. Due to current advances, the European Myeloma Network updated the diagnostic and therapeutic recommendations for patients with Waldenström's macroglobulinemia (WM), AL-amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), POEMS syndrome, and primary plasma cell leukemia. For patients with WM, the combination of rituximab with chemotherapy remains the treatment cornerstone, while the Bruton-tyrosine kinase inhibitor ibrutinib has been introduced and approved for relapsed/refractory disease. Read More

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http://www.nature.com/articles/s41375-018-0209-7
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http://dx.doi.org/10.1038/s41375-018-0209-7DOI Listing
September 2018
12 Reads

Clonal immunoglobulin λ light-chain gene rearrangements detected by next generation sequencing in POEMS syndrome.

Am J Hematol 2018 Sep 31;93(9):1161-1168. Epub 2018 Aug 31.

Department of Hematology, Chiba University Hospital, Chiba, Japan.

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, extravascular fluid overload, M protein, and a myriad of skin changes. The pathogenesis is poorly understood, but monoclonal plasma cells are λ-restricted and these immunoglobulin λ light chain variable (IGLV) region genes are derived from only two germlines, either IGLV1-44 or 1-40. Here we analyzed the clonal IGLV gene rearrangements of genomic DNA samples of bone marrow mononuclear cells using next-generation sequencing (NGS) to understand the clonal composition of IGLV genes in patients with POEMS syndrome (n = 30). Read More

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http://dx.doi.org/10.1002/ajh.25213DOI Listing
September 2018
10 Reads
3.800 Impact Factor

Role of F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Scan in Castleman's Disease.

Indian J Nucl Med 2018 Jul-Sep;33(3):224-226

Department of Nuclear Medicine, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana, India.

Castleman's disease (CD) is a rare benign lymphoproliferative disorder. We are presenting three cases of CD of which one is unicentric CD, and the other two are an idiopathic multicentric CD. One of the two multicentric cases is associated with POEMS syndrome. Read More

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http://dx.doi.org/10.4103/ijnm.IJNM_26_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6011568PMC
July 2018
1 Read

Clinical, electrophysiological and VEGF 2-year response after lenalidomide or stem cell transplantation in patients with POEMS syndrome.

J Neurol Neurosurg Psychiatry 2019 Mar 26;90(3):367-368. Epub 2018 Jun 26.

Department of Medical Biotechnology and Translational Medicine, Milan University, Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Center, Rozzano, Italy

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http://dx.doi.org/10.1136/jnnp-2018-317949DOI Listing
March 2019
1 Read

Cutaneous manifestations as a clue to the diagnosis of POEMS syndrome.

Postgrad Med J 2018 Nov 19;94(1117):668-669. Epub 2018 Jun 19.

Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1136/postgradmedj-2018-135860DOI Listing
November 2018
4 Reads

Transthyretin amyloid polyneuropathies mimicking a demyelinating polyneuropathy.

Neurology 2018 Jul 15;91(2):e143-e152. Epub 2018 Jun 15.

From Service de Physiologie Clinique-Explorations Fonctionnelles (P.L.), AP-HP, Hôpital Lariboisière, Paris; INSERM UMR965 (P.L.), Paris; Université Paris Diderot Sorbonne Paris Cité (P.L., B.A.), Paris; French National Reference Center for FAP (NNERF) (L.-L.M., P.D., G.B., M.T., C.A., D.A.), Le Kremlin-Bicêtre; Service de Neurologie (L.-L.M., P.D., M.T., D.A.) and Service d'anatomopathologie (C.A.), APHP, Hôpital Bicêtre, Le Kremlin-Bicêtre; Unité de Neurophysiologie Clinique et d'épileptologie (G.B.), Hôpital Bicêtre, Le Kremlin-Bicêtre; Immuno-Hematology Department (B.A.), Saint-Louis Hospital, Paris; Université Paris 11 (D.A.); and INSERM UMR1195 (D.A.), Le Kremlin-Bicêtre, France.

Objective: To clearly define transthyretin familial amyloid polyneuropathies (TTR-FAPs) fulfilling definite clinical and electrophysiologic European Federation of Neurological Societies/Peripheral Nerve Society criteria for chronic inflammatory demyelinating polyneuropathy (CIDP).

Methods: From a cohort of 194 patients with FAP, 13 of 84 patients (15%) of French ancestry had late-onset demyelinating TTR-FAP. We compared clinical presentation and electrophysiology to a cohort with CIDP and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome. Read More

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http://dx.doi.org/10.1212/WNL.0000000000005777DOI Listing

An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature.

J Cancer Res Ther 2018 Apr-Jun;14(3):679-681

Department of Internal Medicine, Health East Care System, Saint Paul, Minnesota, 55102, USA.

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We present an atypical case of POEMS syndrome with subclinical polyneuropathy, splenomegaly, hypogonadism, and myeloma of immunoglobulin G kappa type. The typical osteosclerotic lesions and skin lesions were absent, and vascular endothelial growth factor (VEGF) level was normal. Read More

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http://dx.doi.org/10.4103/0973-1482.172123DOI Listing
November 2018
11 Reads

[Successful treatment of POEMS syndrome-associated pulmonary hypertension with lenalidomide and dexamethasone therapy].

Rinsho Ketsueki 2018;59(5):489-491

Department of Hematology and Immunology, Kagoshima University Hospital.

POEMS syndrome is often complicated by pulmonary hypertension. The standard therapy for patients with POEMS syndrome is high-dose chemotherapy followed by autologous stem cell transplantation. However, the safety of high-dose chemotherapy for patients complicated with pulmonary hypertension remains unclear, and the optimal therapy for these patients is yet to be establishment. Read More

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http://dx.doi.org/10.11406/rinketsu.59.489DOI Listing
January 2018

Cutaneous Mycobacterium chelonae infection following autologous peripheral blood stem cell transplantation for POEMS syndrome.

J Infect Chemother 2018 Dec 31;24(12):983-986. Epub 2018 May 31.

Department of Hematology, Tottori Prefectural Central Hospital, Tottori, Japan.

Although hematopoietic stem cell transplantation (HSCT) may increase the curability of refractory hematologic diseases, it requires complication management due to a long-term immunocompromised state. We experienced a case who received an autologous peripheral blood stem cell transplantation (Auto-PBSCT) for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) and developed cutaneous Mycobacterium chelonae infection. It is clear that attention needs to be paid to prevent bacterial, fungal and viral infection after HSCT. Read More

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http://dx.doi.org/10.1016/j.jiac.2018.05.004DOI Listing
December 2018
4 Reads

Atypical central retinal artery occlusion as the first presentation of POEMS syndrome: a case report.

BMC Neurol 2018 May 8;18(1):64. Epub 2018 May 8.

Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Bangkok, 10400, Thailand.

Background: POEMS syndrome is a plasma cell disorder, which clinically manifests from paraneoplastic syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. The most common ocular manifestation is optic disc swelling, whereas other ocular manifestations; cystoid macular edema, serous macular detachment, venous sinus thrombosis, infiltrative orbitopathy, uveitis, neovascularization of the disc, peripapillary choroidal neovascularization and optic disc drusen, had also been reported.

Case Presentation: A 52-year-old Thai man presented with 5-day sudden painless visual loss in the left eye. Read More

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http://dx.doi.org/10.1186/s12883-018-1071-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5938810PMC
May 2018
11 Reads

Effectiveness of rituximab-containing treatment regimens in idiopathic multicentric Castleman disease.

Ann Hematol 2018 Sep 7;97(9):1641-1647. Epub 2018 May 7.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 1# Shuai Fu Yuan, Dongcheng District, Beijing, 100730, China.

Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. Read More

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http://dx.doi.org/10.1007/s00277-018-3347-0DOI Listing
September 2018
57 Reads
2.634 Impact Factor

Glomeruloid hemangioma associated with TAFRO syndrome.

Hum Pathol 2018 Dec 25;82:172-176. Epub 2018 Apr 25.

Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo 113-0033, Japan. Electronic address:

Glomeruloid hemangioma is a rare cutaneous lesion that has been considered as a specific cutaneous marker of POEMS syndrome. Herein, we present the first case of glomeruloid hemangioma associated with TAFRO syndrome, a unique variant of idiopathic multicentric Castleman disease. The patient is a 74-year-old woman presented with fever, cervical lymphadenopathy, thrombocytopenia, bilateral pleural effusions and ascites. Read More

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http://dx.doi.org/10.1016/j.humpath.2018.04.011DOI Listing
December 2018
4 Reads

Hepatobiliary and Pancreatic: Obliterative portal venopathy found in Castleman disease variant of POEMS syndrome.

J Gastroenterol Hepatol 2018 Jul 24;33(7):1311. Epub 2018 Apr 24.

Department of Rheumatology and Clinical Immunology, Peking University International Hospital, Beijing, China.

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http://dx.doi.org/10.1111/jgh.14084DOI Listing
July 2018
8 Reads

[Research and Applications Progress of Lenalidomide in Relapsed / Refractory Blood System Diseases -Review].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Apr;26(2):595-599

Center of Hematologic Diseases of Chinese PLA, Lanzhou Military Command General Hospital, Lanzhou 730050, Gansu Province, China.

Lenalidomide, a novel immunomodulatory agent, is a kind of thalidomide derivatives, which shows a good efficacy and safety for hematological system diseases. This review is aimed to evaluate the efficacy and safety of lenalidomide in treatment of patients with multiple myeloma, chronic lymphocytic leukemia, acute myeloid leukemia, non-Hodgkin's lymphoma, classical Hodgkin's lymphoma and POEMS syndrome at their replased or refractory state. At the same time, this review focuses on the newest clinical research and the latest application progress of lenalidomide for relapsed or refractory hematological system diseases. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2018.02.048DOI Listing
April 2018
4 Reads

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila.

Clin Med Res 2018 Jun 12;16(1-2):16-28. Epub 2018 Apr 12.

University of Central Florida College of Medicine/HCA Consortium Graduate Medical Education, North Florida Regional Medical Center, 6500 W Newberry Rd, Gainesville, FL 32605.

Flushing is the subjective sensation of warmth accompanied by visible cutaneous erythema occurring throughout the body with a predilection for the face, neck, pinnae, and upper trunk where the skin is thinnest and cutaneous vessels are superficially located and in greatest numbers. Flushing can be present in either a wet or dry form depending upon whether neural-mediated mechanisms are involved. Activation of the sympathetic nervous system results in wet flushing, accompanied by diaphoresis, due to concomitant stimulation of eccrine sweat glands. Read More

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http://dx.doi.org/10.3121/cmr.2017.1379aDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6108509PMC
June 2018
7 Reads

Autologous Stem Cell Transplantation for Multiple Myeloma: Single Centre Experience from North India.

Indian J Hematol Blood Transfus 2018 Apr 19;34(2):261-267. Epub 2017 Sep 19.

1Clinical Haematology Division, Department of Internal Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012 India.

Autologous stem cell transplantation (ASCT) is considered as standard of care in patients with multiple myeloma (MM) patients aged 65 years or younger. We analyzed data of 94 patients of plasma cell dyscrasias who underwent 95 autologous transplants at our institute from October 2003 to Aug 2016. Other than 76 patients of newly diagnosed multiple myeloma, we also transplanted two patients of POEMS syndrome, two patients of plasma cell leukemia, three patients of concurrent light chain deposition disease, three patients of multifocal plasmacytomas, and eight patients of isolated light chain myeloma. Read More

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http://dx.doi.org/10.1007/s12288-017-0876-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5884990PMC
April 2018
6 Reads
1 Citation
0.230 Impact Factor

Daratumumab for POEMS Syndrome.

Mayo Clin Proc 2018 04;93(4):542-544

University of Arkansas for Medical Sciences, Little Rock, AR.

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https://linkinghub.elsevier.com/retrieve/pii/S00256196183009
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http://dx.doi.org/10.1016/j.mayocp.2018.02.001DOI Listing
April 2018
26 Reads

Prognostic value of serum vascular endothelial growth factor and hematological responses in patients with newly-diagnosed POEMS syndrome.

Blood Cancer J 2018 04 4;8(4):37. Epub 2018 Apr 4.

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

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http://dx.doi.org/10.1038/s41408-018-0073-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5884844PMC
April 2018
4 Reads

Problems in Comparing Jitter Values Obtained with Voluntary Activation and Electrical Stimulation.

J Neuromuscul Dis 2018 ;5(2):225-230

Department of Neurology, Duke University Medical Center, Durham, NC, USA.

Comparing results from jitter studies performed with voluntary and electrical activation is difficult to perform quantitatively, particularly in complex signals as seen in reinnervation. High jitter values in individual spikes in these multispike signals can be missed with both activation methods, which introduces a bias towards more normal values.With voluntary activation, triggering on a spike from an abnormal end-plate in multispike potentials will overestimate individual jitter values and the number of abnormal jitter values. Read More

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http://dx.doi.org/10.3233/JND-170289DOI Listing
November 2018
3 Reads