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    Autologous peripheral blood stem cell transplantation and the role of lenalidomide in patients affected by poems syndrome.
    Hematol Oncol 2017 Sep 15. Epub 2017 Sep 15.
    Department of Hematology, Catholic University of Sacred Heart, Rome, Italy.
    POEMS syndrome is a rare paraneoplastic condition, with a poorly understood pathogenesis. High dose chemotherapy followed by autologous stem cell transplantation (ASCT) has been reported to be an effective therapeutic option for patients with good performance status. Here, we review the role of ASCT for POEMS syndrome and discuss indications together with advantages and disadvantages, and related issues such lenalidomide given before or after ASCT, VEGF levels as a marker of disease, and different regimens for stem cell mobilization. Read More

    [Incomplete POEMS syndrome with multicentric Castleman's disease].
    An Sist Sanit Navar 2017 Sep 14;0(0):52410. Epub 2017 Sep 14.
    .
    Castleman's disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats. Read More

    POEMS SYNDROME: an Update.
    Mediterr J Hematol Infect Dis 2017 1;9(1):e2017051. Epub 2017 Sep 1.
    Department of Medical Oncology and Hematology, Humanitas Cancer Center, Humanitas Clinical and Research Hospital IRCCS, Rozzano, Milan, Italy.
    POEMS syndrome is a rare, chronic and disabling condition. The causes of this condition remain unknown; however, chronic overproduction of proinflammatory cytokines appears to be a major contributor. Early diagnosis is essential to start treatment before the clinical state of the patient becomes compromised. Read More

    POEMS Syndrome with Biclonal Gammopathy: A Rare Association.
    J Clin Diagn Res 2017 Jul 1;11(7):XD12-XD13. Epub 2017 Jul 1.
    Consultant, Department of Transfusion Medicine, The Mission Hospital, Durgapur, West Bengal, India.
    Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes (POEMS) syndrome is rare plasma cell dyscrasia with multisystem involvement. The name comes from the five characteristic features: Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The presence of biclonal M band is a rare manifestation. Read More

    Plexogenic pulmonary hypertension associated with POEMS syndrome.
    Respir Med Case Rep 2017 12;22:168-170. Epub 2017 Aug 12.
    Mayo Clinic, Rochester MN, United States.
    Pulmonary hypertension is one of the well-known clinical manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, occurring in approximately 25-30% of the affected individuals. However, the histopathologic spectrum of pulmonary hypertension associated with POEMS syndrome has not been fully documented in the literature. Herein, we report an autopsy case of POEMS syndrome in a patient whose lung tissues showed histopathology indistinguishable from that of idiopathic pulmonary arterial hypertension with abundant plexiform lesions in the small pulmonary arteries. Read More

    Eosinophilia secondary to lenalidomide therapy.
    J Clin Pharm Ther 2017 Aug 17. Epub 2017 Aug 17.
    Pharmacy Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
    What Is Known And Objective: Limited data are available on eosinophilia as a drug adverse event. We describe a case of eosinophilia from lenalidomide therapy.

    Case Description: A 50-year-old woman received lenalidomide, dexamethasone and cyclophosphamide as POEMS syndrome treatment. Read More

    POEMS syndrome in a patient with rheumatoid arthritis.
    BMJ Case Rep 2017 Aug 3;2017. Epub 2017 Aug 3.
    Department of Rheumatology, Kawasaki Medical School, Kurashiki, Japan.
    Multiple myeloma has been reported to be associated with rheumatoid arthritis (RA). POEMS syndrome is a rare variant of multiple myeloma and is characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report the case of a 67-year-old patient with RA who developed numbness and tingling in both legs due to polyneuropathy. Read More

    POEMS syndrome: 2017 Update on diagnosis, risk stratification, and management.
    Am J Hematol 2017 Aug;92(8):814-829
    Mayo Clinic, Professor of Medicine and Laboratory Medicine, Rochester, Minnesota, 55905.
    Disease Overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Read More

    Paraneoplastic neuropathies.
    Curr Opin Neurol 2017 Oct;30(5):513-520
    Department of Neurology, University Hospital of Saint-Etienne, Saint-Etienne, France.
    Purpose Of Review: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development.

    Recent Findings: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. Read More

    Coexistence of Quasi-moyamoya Disease and POEMS Syndrome in a Patient with Intracranial Hemorrhage: A Case Report and Literature Review.
    NMC Case Rep J 2017 Jan 8;4(1):5-9. Epub 2016 Dec 8.
    Department of Neurosurgery, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.
    POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare paraneoplastic syndrome elicited by plasma cell dyscrasia. Its clinical manifestations are multiple and stroke is not a recognized feature. A 44-year-old woman with a 3-month history of bilateral lower extremity sensorimotor disturbance was admitted to our hospital. Read More

    POEMS syndrome: a rare cause of exudative ascites and chronic peripheral neuropathy.
    BMJ Case Rep 2017 Jun 20;2017. Epub 2017 Jun 20.
    Neurology Department, Rashid Hospital, Dubai, UAE.
    POEMS syndrome (Peripheral neuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes) is a rare plasma cell disorder with multisystem involvementA 40-year-old man with a chronic history of unexplained peripheral neuropathy , presented with exudative ascites.He was found to have an incidental osteolytic lesion of the sacrum proven to be a plasmacytoma. Immunoelectrophoresis for monoclonal protein was negative. Read More

    Neurologic Complications of Lymphoma, Leukemia, and Paraproteinemias.
    Continuum (Minneap Minn) 2017 Jun;23(3, Neurology of Systemic Disease):669-690
    Purpose Of Review: This article reviews the spectrum of neurologic complications associated with lymphoma, leukemia, and paraproteinemic disorders. While leptomeningeal metastasis is the most common complication of lymphoma and leukemia and peripheral neuropathy is the most common complication of paraproteinemic disorders, clinicians need to be familiar with the diverse neurologic complications of these disorders.

    Recent Findings: Lymphomatous nervous system involvement can be difficult to diagnose, especially when it is the presenting symptom. Read More

    Diagnostic challenges in POEMS syndrome presenting with polyneuropathy: A case series.
    J Neurol Sci 2017 Jul 11;378:170-174. Epub 2017 May 11.
    Department of Neurology, University Hospitals Cleveland Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA.
    Objective: To analyze the clinical characteristics of patients with POEMS presenting with polyneuropathy and discuss associated diagnostic challenges.

    Method: Retrospective analysis of 6 patients from 2 tertiary-care institutions.

    Results: Six patients presented with progressive sensorimotor deficits predominantly or exclusively in the lower extremities and were diagnosed with POEMS syndrome. Read More

    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 Jul 8;78(3):190-194. 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:

    Electrophysiologic features of POEMS syndrome compared with MGUS-related neuropathy.
    Muscle Nerve 2017 May 4. Epub 2017 May 4.
    Department of Neurology, Asan Medical Center, 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. Herein we assessed the findings of nerve conduction studies (NCS) in patients with POEMS syndrome and MGUS-related neuropathy to determine whether the NCS characteristics can help differentiate between these 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
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    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

    Two patients with TAFRO syndrome exhibiting strikingly similar anterior mediastinal lesions with predominantly fat attenuation on chest computed tomography.
    Respir Investig 2017 Mar 23;55(2):176-180. Epub 2016 Nov 23.
    First Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan. Electronic address:
    We herein report on two middle-aged men with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis or renal failure, and organomegaly) syndrome, a unique clinicopathological variant of multicentric Castleman׳s disease recently proposed in Japan. Strikingly similar anterior mediastinal fat swellings with soft tissue density were observed in the patients on chest computed tomography. In TAFRO syndrome, bilateral pleural effusion and slight lymph node swelling are common in the thoracic region; however, anterior mediastinal lesions have not been previously observed. 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

    ELBOW ULNAR NEUROPATHY: TREATMENT BY ANTERIOR TRANSPOSITION OF THE ULNAR NERVE.
    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 Sep 10;38(9):619-622. 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 Jun 19;31(6):1375-1381. Epub 2017 Jan 19.
    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 2017 Jul 22;16(3):231-237. Epub 2016 Dec 22.
    Department of Radiology, Kanazawa University Hospital.
    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

    OCULAR MANIFESTATIONS AND TREATMENT OUTCOMES IN CHINESE PATIENTS WITH POEMS SYNDROME.
    Retina 2017 Sep;37(9):1784-1791
    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

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