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    Portal hypertension as the initial manifestation of POEMS syndrome: a case report.
    BMC Hematol 2017 11;17. Epub 2017 May 11.
    Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730 China.
    Background: Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. Read More

    Endocrine perturbations in POEMS syndrome: Misunderstood features of a rare paraneoplastic syndrome.
    Ann Endocrinol (Paris) 2017 May 8. Epub 2017 May 8.
    IE3M, endocrinologie et médecine de la reproduction, centre de référence des maladies endocriniennes rares de la croissance, hôpitaux universitaires La Pitié-Salpêtrière-Charles-Foix, AP-HP, 75013 Paris, France. Electronic address:

    Electrophysiological features of POEMS syndrome compared to MGUS-related neuropathy.
    Muscle Nerve 2017 May 4. Epub 2017 May 4.
    Department of Neurology, University of Ulsan College of Medicine, Seoul, Republic of Korea.
    Introduction: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome and monoclonal gammopathy of undetermined significance (MGUS) are paraproteinemic disorders that can cause demyelinating polyneuropathy. Here, we assessed the findings of nerve conduction studies (NCSs) in patients with POEMS syndrome and MGUS-related neuropathy to determine whether the NCS characteristics can help differentiate between the conditions.

    Methods: We enrolled 24 POEMS and 37 MGUS-related neuropathy patients. Read More

    An Unfortunate Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Change (POEMS).
    Cureus 2017 Mar 8;9(3):e1086. Epub 2017 Mar 8.
    Internal Medicine, University of Central Florida College of Medicine.
    POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, which is a rare paraneoplastic disease of monoclonal plasma cells. A mandatory criterion to diagnose POEMS syndrome is the presence of a monoclonal plasma cell dyscrasia in which plasma cell leukemia is the most aggressive form. Early identification of the features of the POEMS syndrome is critical for patients to identify an underlying plasma cell dyscrasias and to reduce the morbidity and mortality of the disease by providing early therapy. Read More

    Diagnosis and management of neuropathies associated with plasma cell dyscrasias.
    Hematol Oncol 2017 Apr 10. Epub 2017 Apr 10.
    Division of Hematology & Oncology, Columbia University Medical Center, New York, NY, USA.
    Neuropathies associated with plasma cell dyscrasias are a major cause of morbidity for patients managed by medical oncologists. Because of similarities in clinical presentation and on nerve conduction studies, identifying the underlying disease leading to a paraproteinemic neuropathy can often be difficult. In addition, the degree of neurologic deficit does not strictly correlate with the extent of abnormalities on common clinical laboratory testing. Read More

    Osteolytic-variant POEMS syndrome: an uncommon presentation of "osteosclerotic" myeloma.
    Skeletal Radiol 2017 Jun 25;46(6):817-823. Epub 2017 Mar 25.
    Department of Radiology, Mayo Clinic, Mayo Building, E2, 200 First Street SW, Rochester, MN, 55905, USA.
    Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome, a form of osteosclerotic myeloma, is a multisystem disease related to a monoclonal plasma cell proliferative disorder. Osseous lesions are most commonly sclerotic on radiographs and computed tomography (CT), demonstrate low T1 and T2 signal intensity on magnetic resonance imaging (MRI), and have variable degrees of avidity on positon emission tomography (PET) imaging using 18-fluorodeoxyglucose ((18)F-FDG). We present three cases of POEMS syndrome manifesting as osteolytic lesions with indolent features, including well-defined thin sclerotic rims, no cortical disruption or periosteal reaction, no associated soft-tissue mass, and a periarticular location, all features that could lead to misinterpretation as benign bone lesions. Read More

    POEMS Syndrome: an Enigma.
    Curr Hematol Malig Rep 2017 Apr;12(2):85-95
    Division of Hematology, Medicine and Oncology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
    POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis. Read More

    Immunotactoid glomerulopathy leading to the discovery of POEMS syndrome
    Clin Nephrol 2017 Jun;87(6):310-315
    Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Read More

    POEMS syndrome and idiopathic portal hypertension: a possible association.
    Rev Esp Enferm Dig 2017 May;109(5):393
    Pathology, Centro Hospitalar e Universitário de Coimbra.
    A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). Read More

    Acta Ortop Bras 2016 Jul-Aug;24(4):184-186
    . Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto. Ribeirão Preto, SP, Brazil.
    Objectives: Retrospective clinical evaluation of 31 patients who underwent ulnar nerve decompression at the elbow and subcutaneous anterior transposition.

    Methods: From January 2000 to December 2013, 71 patients underwent subcutaneous anterior transposition of the ulnar nerve. Thirty-one patients returned for evaluation. Read More

    [Sarcoidosis flare after autologous stem cell transplantation: An immune paradox?]
    Rev Med Interne 2017 Feb 10. Epub 2017 Feb 10.
    Service de médecine interne, institut E3M, centre national de référence maladies auto-immunes systémiques rares, hôpital Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France; Université Pierre-et-Marie-Curie, université Paris VI, Sorbonne Université, Paris 75013, France. Electronic address:
    Introduction: Sarcoidosis is a systemic granulomatous disorder of unknown cause. Apparition or flare of previously diagnosed sarcoidosis following hematopoietic stem cell transplantation (HSCT) has rarely been reported.

    Observation: We report a 62-year-old woman who presented a radiological flare of sarcoidosis post-autologous stem cell transplantation for a POEMS syndrome. Read More

    Polyneuropathy as Novel Initial Manifestation in a Case of "Nonsecretory" POEMS Syndrome with Sjögren's Syndrome.
    Case Rep Med 2017 4;2017:1276759. Epub 2017 Jan 4.
    Nursing Department, Huashan Hospital, Fudan University, Shanghai 200040, China.
    POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome driven by plasma cell dyscrasias. We report a patient with novel initial manifestation of polyneuropathy, which was considered due to Sjögren's syndrome but with poor response to methylprednisolone (120 mg/d) and intravenous immunoglobulin (IVIg). Further investigation by imaging tests and following biopsy eventually confirmed the diagnosis of POEMS syndrome secondary to solitary plasmocytoma. Read More

    Impact of pretransplant induction therapy on autologous stem cell transplantation for patients with newly diagnosed POEMS syndrome.
    Leukemia 2017 Feb 14. Epub 2017 Feb 14.
    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
    POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a rare plasma cell dyscrasia sometimes treated with a haematopoietic cell autotransplant. We analyzed data from 138 subjects with newly diagnosed POEMS syndrome receiving a autotransplant at our center. Thirty-two subjects with severe end-organ dysfunction ineligible for immediate autotransplant received pretransplant therapy, which made a subsequent autotransplant feasible. Read More

    Cranial Pachymeningeal Involvement in POEMS Syndrome: Evaluation by Pre- and Post-contrast FLAIR and T1-weighted Imaging.
    Magn Reson Med Sci 2016 Dec 22. Epub 2016 Dec 22.
    Department of Advanced Medical Imaging, Graduate School of Medical Science, Kanazawa University.
    Purpose: To evaluate the cranial pachymeningeal involvement of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome using pre- and post-contrast fluid-attenuated inversion recovery (FLAIR) and T1-weighted imaging (T1WI).

    Methods: The appearance of pachymeningeal involvement in nine cases of POEMS syndrome was evaluated using pre- and post-contrast FLAIR and T1WI. The degree of pachymeningeal thickening was graded as normal or abnormal using pre-contrast FLAIR. Read More

    Thrombotic microangiopathy associated with monoclonal gammopathy.
    Kidney Int 2017 Mar 18;91(3):691-698. Epub 2016 Dec 18.
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Electronic address:
    Thrombotic microangiopathy (TMA) is a rare disease comprising of a diverse set of disorders linked by a common histologic finding of endothelial injury. Monoclonal immunoglobulins may act as a potential trigger in the pathogenesis of TMA. To determine the prevalence of monoclonal gammopathy and clinicopathological features of TMA associated with monoclonal immunoglobulin, we performed a retrospective study in adults (18 and older) with a clinical diagnosis of TMA. Read More

    Retina 2016 Dec 7. Epub 2016 Dec 7.
    Departments of *Ophthalmology, and †Hematology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
    Purpose: To evaluate the relationship of serum vascular endothelial growth factor (VEGF) levels and ocular manifestations in Chinese patients with POEMS syndrome.

    Methods: This is a prospective study. Forty-one treatment-naive patients were enrolled from April 2014 to November 2014. Read More

    What is new in 2015 in dysimmune neuropathies?
    Rev Neurol (Paris) 2016 Dec 17;172(12):779-784. Epub 2016 Nov 17.
    Department of Clinical Neurosciences, Nerve-Muscle Unit, Neurology Service, Lausanne University Hospital (CHUV), 46, rue du Bugnon, 1011 Lausanne, Switzerland. Electronic address:
    This review discusses and summarizes the concept of nodopathies, the diagnostic features, investigations, pathophysiology, and treatment options of chronic inflammatory demyelinating polyradiculoneuropathy, and gives updates on other inflammatory and dysimmune neuropathies such as Guillain-Barré syndrome, sensory neuronopathies, small-fiber-predominant ganglionitis, POEMS syndrome, neuropathies associated with IgM monoclonal gammopathy and multifocal motor neuropathy. This field of research has contributed to the antigenic characterization of the peripheral motor and sensory functional systems, as well as helping to define immune neuropathic syndromes with widely different clinical presentation, prognosis and response to therapy. Read More

    Safety and Efficacy of Granulocyte Colony-Stimulating Factor Monotherapy for Peripheral Blood Stem Cell Collection in POEMS Syndrome.
    Biol Blood Marrow Transplant 2017 Feb 10;23(2):361-363. Epub 2016 Nov 10.
    Department of Hematology, Chiba University Hospital, Chiba, Japan.
    Although autologous stem cell transplantation can achieve excellent responses in patients with POEMS syndrome, the optimal regimen for peripheral blood stem cell (PBSC) collection is still controversial. We retrospectively investigated the safety and efficacy of 41 PBSC collecting procedures in 37 patients with POEMS syndrome. PBSC mobilization was performed using cyclophosphamide + granulocyte colony-stimulating factor (G-CSF) (CG, n = 14) or G-CSF alone (G, n = 27). Read More

    Measurement of β-isomerized C-terminal telopeptide of type I collagen in patients with POEMS syndrome: diagnostic, prognostic, and follow-up utilities.
    Blood Cancer J 2016 Nov 11;6(11):e495. Epub 2016 Nov 11.
    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

    The Danish National Multiple Myeloma Registry.
    Clin Epidemiol 2016 25;8:583-587. Epub 2016 Oct 25.
    Department of Hematology, Odense University Hospital, Odense, Denmark.
    Aim: The Danish National Multiple Myeloma Registry (DMMR) is a population-based clinical quality database established in January 2005. The primary aim of the database is to ensure that diagnosis and treatment of plasma cell dyscrasia are of uniform quality throughout the country. Another aim is to support research. Read More

    Clinical spectrum of Castleman disease-associated neuropathy.
    Neurology 2016 Dec 2;87(23):2457-2462. Epub 2016 Nov 2.
    From the Departments of Neurology (E.N., M.L.M.), Internal Medicine (A.D.), and Medicine (J.M.), Mayo Clinic, Rochester, MN.
    Objective: To define the peripheral neuropathy phenotypes associated with Castleman disease.

    Methods: We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). Read More

    Nerve pathologic features differentiate POEMS syndrome from CIDP.
    Acta Neuropathol Commun 2016 Oct 31;4(1):116. Epub 2016 Oct 31.
    Mayo Clinic, Department of Neurology, 200 First Street SW, Rochester, MN, 55905, USA.
    The objective of this study is to determine if the nerve pathology in patients with POEMS syndrome is different from CIDP. We hypothesized that nerve biopsies from patients with POEMS syndrome would have more small vessels and axonal degeneration but less inflammation than CIDP.We performed a retrospective analysis of nerve biopsies performed on "classic" CIDP and POEMS cases. Read More

    TAFRO syndrome with primary Sjogren's syndrome.
    Nihon Rinsho Meneki Gakkai Kaishi 2016 ;39(5):478-484
    Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center.
      A 25-year-old woman diagnosed 1 year earlier with Primary Sjogren's syndrome was admitted to a nearby hospital with fever of unknown origin. Examination revealed anasarca, systemic lymphadenopathy, hepatosplenomegaly and high C-reactive protein level. The patient's symptoms were initially suspected to be caused by severe bacterial infection with Sjogren's syndrome flare. Read More

    An Occult Malignancy Behind a Demyelinating Disease: POEMS Syndrome.
    J Investig Med High Impact Case Rep 2016 Oct-Dec;4(4):2324709616673389. Epub 2016 Oct 18.
    Mount Sinai Medical Center, Miami Beach, FL, USA.
    We report a case of a 38-year-old man presenting with bilateral lower extremity weakness and paresthesias that progressed during a 4-month period to severe polyneuropathy forcing the patient to be bed bound. Throughout his multiple hospitalizations, he was treated erroneously for chronic inflammatory demyelinating polyneuropathy, without significant improvement in his symptoms. In addition, he developed hepatosplenomegaly (organomegaly); endocrinopathies such as diabetes mellitus, central hypogonadism, and hypothyroidism; monoclonal spike evidenced in the serum electrophoresis; and hyperpigmentation of skin, altogether consistent with POEMS syndrome. Read More

    Remarkable expression of vascular endothelial growth factor in bone marrow plasma cells of patients with POEMS syndrome.
    Leuk Res 2016 Nov 26;50:78-84. Epub 2016 Sep 26.
    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address:
    Vascular endothelial growth factor (VEGF) is pathognomonically elevated in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome. However, its source of overproduction is unclear. As clinical improvement is almost always associated with VEGF reduction after anti-plasma cell therapy, its increase at diagnosis has been attributed to the underlying monoclonal gammopathy, although direct evidence is still lacking. Read More

    Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome.
    Oncotarget 2016 11;7(45):74410-74414
    Department of Endocrinology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
    POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Arterial thrombosis is a distinctively unusual feature in patients with POEMS syndrome. We report a 33-year-old man with intermittent amaurosis of left eye and skin changes as the onset manifestations, who was finally confirmed as having POEMS syndrome. Read More

    Temporal and Spatial Correlation Between Choroidal Thickness and Visual Function in a Case of POEMS Syndrome.
    Ocul Immunol Inflamm 2016 Oct 11:1-4. Epub 2016 Oct 11.
    a Ophthalmology Department , University Hospital of Henares , Madrid , Spain.
    Purpose: To report the correlation between visual function and subfoveal choroidal thickness (SChT) in a case of POEMS syndrome.

    Case Report: A 53 year old man diagnosed with POEMS syndrome was referred due to blurred vision. Best corrected visual acuity (BCVA) was 0. Read More

    Changes in subfoveal choroidal thickness and reduction of serum levels of vascular endothelial growth factor in patients with POEMS syndrome.
    Br J Ophthalmol 2016 Oct 4. Epub 2016 Oct 4.
    Department of Ophthalmology and Visual Science, Graduate School of Medicine, Chiba University, Chiba, Japan.
    Aims: To determine the changes in the subfoveal choroidal thickness (CT), the foveal thickness (FT) and the serum level of vascular endothelial growth factor (VEGF) after thalidomide treatment in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome.

    Methods: We studied 13 left eyes of 13 treatment-naïve patients with POEMS syndrome. The subfoveal CT and FT were determined by enhanced depth imaging optical coherence tomography, and the serum level of VEGF was determined by ELISA at the baseline and at 6 months after thalidomide treatment. Read More

    A case of POEMS mimicking a "Guillain-Barré like" syndrome.
    J Neurol Sci 2016 Oct 13;369:268-70. Epub 2016 Aug 13.
    Department of Neurophysiology, Queen Elizabeth Hospital, University Hospitals of Birmingham, Birmingham, UK; Department of Neurology, Queen Elizabeth Hospital, University Hospitals of Birmingham, Birmingham, UK; Aston Brain Centre, Aston University, Birmingham, UK. Electronic address:

    High-dose therapy and autologous stem cell transplantation in patients with POEMS syndrome: a retrospective study of the Plasma Cell Disorder sub-committee of the Chronic Malignancy Working Party of the European Society for Blood & Marrow Transplantation.
    Haematologica 2017 Jan 15;102(1):160-167. Epub 2016 Sep 15.
    Hôpital Saint Antoine, Paris, France.
    POEMS syndrome is a rare para-neoplastic syndrome secondary to a plasma cell dyscrasia. Effective treatment can control the disease-related symptom complex. We describe the clinical outcome of autologous stem cell transplantation for patients with POEMS syndrome, determining the impact of patient- and disease-specific factors on prognosis. Read More

    POEMS syndrome: a rare case report.
    G Ital Dermatol Venereol 2016 Sep 15. Epub 2016 Sep 15.
    Dermatologic Clinic "Maggiore della Carità" Hospital and UPO University of Eastern Piedmont, Novara, Italy -
    The authors report a case of sensorimotor polyneuropathy, diffuse hemangiomas and monoclonal gammopathy. Besides weight loss, there were diabetes mellitus and severe hypothyroidism. These alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Read More

    Bone lesions in Chinese POEMS syndrome patients: imaging characteristics and clinical implications.
    PeerJ 2016 2;4:e2294. Epub 2016 Aug 2.
    Department of Radiology, Peking Union Medical College Hospital , Beijing , China.
    Objective. Bone lesion is crucial for diagnosing and management of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome, a rare plasma cell disorder. This study is to compare the effectiveness of X-ray skeletal survey (SS) and computed tomography (CT) for detecting bone lesions in Chinese POEMS syndrome patients, and to investigate the relationship between bone lesion features and serum markers. Read More

    Skin manifestations of POEMS and AESOP syndrome in the same patient revealing plasma cell dyscrasia.
    J Cutan Pathol 2016 Dec 15;43(12):1167-1171. Epub 2016 Sep 15.
    AOU, S. Giovanni di Dio Hospital, Mario Aresu Department of Medical Science, Section of Dermatology, University of Cagliari, Cagliari, Italy.
    POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin signs) and AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndromes are rare paraneoplastic conditions due to an underlying plasma cell dyscrasia. We report a 70-year-old patient with the rare coexistence of POEMS and AESOP syndromes and in whom skin signs, that differ both clinically and histologically, were the clues to the diagnosis of a plasma cell disorder. Vascular endothelial growth factor-A overexpression seems to be the common pathogenetic link of the different clinicopathological presentations of the skin lesions. Read More

    Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial.
    Lancet Neurol 2016 Oct 3;15(11):1129-37. Epub 2016 Aug 3.
    Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address:
    Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare cause of demyelinating neuropathy, with multi-organ involvement characterised by plasma cell dyscrasia and VEGF overproduction. No treatments have been established for patients with POEMS syndrome who are not eligible for stem-cell transplantation. Thalidomide suppresses VEGF and plasma cell proliferation. Read More

    A Rare Case of Polyneuropathy and Monoclonalgammopathy with Recurrent Acute Kidney Injury.
    Electrolyte Blood Press 2016 Jun 30;14(1):5-10. Epub 2016 Jun 30.
    Department of Internal Medicine, Kangdong Sacred Heart Hospital, Hallym Kidney Research Institute, Seoul, Korea.
    POEMS syndrome is a rare paraneoplastic syndrome and there are few reports of polyneuropathy and monoclonal gammopathy associated with kidney dysfunction. Here, we report a case of POEMS syndrome with recurrent acute kidney injury (AKI). A 52-year-old man presented with bilateral aggravating paresthesia and latermotor weakness of the lower extremities accompanied by repeated elevation of serum creatinine. Read More

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