• haematological diseases
• medical research

• Humans
• POEMS Syndrome/mortality
• POEMS Syndrome/complications
• POEMS Syndrome/pathology
• Male
• Female
• Middle Aged
• Capillary Leak Syndrome/etiology
• Capillary Leak Syndrome/pathology
• Capillary Leak Syndrome/mortality
• Phenotype
• Aged
• Adult
• Cause of Death
• Prognosis
• Survival Rate

• Autologous stem cell transplantation
• Castleman's disease
• MGCS
• POEMS syndrome
• Radiation
• Treatment
• VEGF

• POEMS syndrome
• heart failure

• M protein
• POEMS syndrome
• Polyserositis
• Renal damage
• VEGF

• POEMS Syndrome/complications
• POEMS Syndrome/diagnosis
• POEMS Syndrome/blood
• Humans
• Glomerulonephritis, Membranoproliferative/complications
• Vascular Endothelial Growth Factor A/blood
• Male
• Middle Aged

• Humans
• Monoclonal Gammopathy of Undetermined Significance/diagnosis
• Monoclonal Gammopathy of Undetermined Significance/complications
• Monoclonal Gammopathy of Undetermined Significance/therapy
• Male
• Female
• Aged
• Middle Aged
• Nervous System Diseases/etiology

• Castleman disease
• POEMS syndrome
• case report
• diagnostic criteria
• monoclonal protein

• Cytokines
• Differential diagnosis
• Prognosis
• Rare diseases
• Therapy

• Castleman Disease/diagnosis
• Castleman Disease/pathology
• Castleman Disease/therapy
• Humans
• Prognosis

• POEMS syndrome
• carfilzomib
• daratumumab
• dexamethasone
• lenalidomide
• treatment

• POEMS syndrome
• ascites
• case report
• hydrothorax
• portal hypertension

• Humans
• POEMS Syndrome/diagnosis
• POEMS Syndrome/pathology
• Dermoscopy
• Hemangioma/diagnostic imaging
• Hemangioma/pathology
• Hemangioma/diagnosis
• Male
• Skin Neoplasms/pathology
• Skin Neoplasms/diagnosis
• Skin Neoplasms/diagnostic imaging
• Female
• Middle Aged

• Humans
• POEMS Syndrome/blood
• POEMS Syndrome/complications
• POEMS Syndrome/diagnosis
• POEMS Syndrome/immunology
• Immunoglobulin M/blood
• Paraproteins
• Male
• Immunoglobulin lambda-Chains/blood
• Middle Aged

• EuroFlow
• Immunophenotype
• Multiparameter flow cytometry
• POEMS syndrome

• POEMS Syndrome/diagnosis
• Humans
• Flow Cytometry/methods
• Middle Aged
• Male
• Female
• Aged
• Plasma Cells/metabolism
• Plasma Cells/pathology
• Adult
• Immunophenotyping/methods
• Bone Marrow/pathology

• Cancer
• Immune checkpoint inhibitors
• Immune-related adverse events
• Inflammatory arthritis
• Mimickers
• Paraneoplastic syndromes

• Humans
• Arthritis/chemically induced
• Arthritis/diagnosis
• Arthritis/immunology
• Crystal Arthropathies/diagnosis
• Crystal Arthropathies/immunology
• Diagnosis, Differential
• Immune Checkpoint Inhibitors/adverse effects
• Neoplasms/drug therapy
• Neoplasms/immunology
• Osteoarthritis/diagnosis
• Osteoarthritis/immunology
• Sarcoidosis/diagnosis
• Sarcoidosis/immunology

• Case report
• Dexamethasone
• Dilated cardiomyopathy
• Lenalidomide
• M-protein
• Polyneuropathy
• endocrinopathy
• organomegaly
• skin changes syndrome

• Autologous Stem Cell Transplantation
• Long-Term Outcome
• POEMS Syndrome

• Humans
• Male
• Adult
• Middle Aged
• Aged
• Female
• POEMS Syndrome/therapy
• POEMS Syndrome/drug therapy
• Hematopoietic Stem Cell Transplantation
• Vascular Endothelial Growth Factor A
• Retrospective Studies
• Transplantation, Autologous
• Republic of Korea/epidemiology

• Humans
• POEMS Syndrome/diagnosis
• POEMS Syndrome/therapy
• POEMS Syndrome/pathology
• Vascular Endothelial Growth Factor A/metabolism
• Risk Factors
• Diagnosis, Differential
• Polyradiculoneuropathy/diagnosis

• Robert A. Kyle Hematologic Malignancies Fund
• Predolin Foundation
• JABBS Foundation
• Andrew & Lillian A. Posey Foundation

• POEMS syndrome
• Plasma cell disorder
• Pleural effusion
• Pulmonary function test
• Pulmonary hypertension

• Humans
• Male
• Middle Aged
• Female
• POEMS Syndrome/complications
• POEMS Syndrome/diagnosis
• Retrospective Studies
• Lung Diseases
• Hypertension, Pulmonary
• Lung/diagnostic imaging

• M-protein
• POEMS syndrome
• VEGF
• endocrinopathy
• polyneuropathy

• 230 Ministero della Salute

• autologous stem cell transplantation
• castleman variant of poems syndrome
• claw hand
• hdt
• polyneuropathy

• Bibliometric analysis
• Diagnosis
• POEMS syndrome
• Plasma cell neoplasm
• VEGF

• CORNEA / EXTERNAL DISEASE
• GLAUCOMA
• RETINA
• Retinal degenerations associated with systemic disease
• corneal degenerations

• Humans
• Paraproteinemias/complications
• Paraproteinemias/diagnosis
• Eye
• Eye Diseases/diagnosis
• Eye Diseases/etiology
• Treatment Outcome

• Case reports
• Multiple myeloma
• Paraneoplastic symptoms
• Polycythemia vera
• Sweet syndrome

• Clinical picture
• POEMS syndrome
• Plasma cell dyscrasia
• Therapy

• Idiopathic non cirrhotic portal hypertension
• POEMS syndrome
• PSVD
• Porto-Sinusoidal Vascular Disorder

• light chain-restricted plasmacytic differentiation
• mediastinal malt lymphoma
• multiple myeloma
• treatment
• case report

• Humans
• Lymphoma, B-Cell, Marginal Zone/complications
• Lymphoma, B-Cell, Marginal Zone/drug therapy
• Lymphoma, B-Cell, Marginal Zone/diagnosis
• Rituximab/therapeutic use
• Multiple Myeloma/complications
• Multiple Myeloma/diagnosis
• Lenalidomide/therapeutic use
• POEMS Syndrome/complications
• POEMS Syndrome/diagnosis
• Bortezomib/therapeutic use
• Thymus Neoplasms

• Ascites
• POEMS syndrome
• peripheral neuropathy
• plasma cell disease
• sclerotic bone lesions

• clinical aspects; clinical haematology; multiple myeloma; tumour immunotheraphy

To determine the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with PH as well as precapillary PH.

Olmsted County residents with PH, diagnosed between 1/1/1995 and 9/30/2017, were identified, and age and sex were matched to a normal control group. The PH group and normal control group were then cross-referenced with the Mayo Clinic MGUS database. Charts were reviewed to verify MGUS and PH. Heart catheterization data were then analyzed in these patients for reference to the gold standard for diagnosis.

There were 3419 patients diagnosed with PH by echocardiography between 1995 and 2017 in Olmsted County that met the criteria of our study. When the PH group (N = 3313) was matched to a normal control group (3313), a diagnosis of MGUS was significantly associated with PH 10.2% (OR = l.84 [95% CI 1.5–2.2], p < 0.001), compared with controls 5.8% based on echo diagnosis. Using heart catheterization data (484 patients), a diagnosis of MGUS was associated with PH 13.0% (OR = 3.94 [95% CI 2.28–6.82], p < 0.001). For pulmonary artery hypertension (N = 222), a diagnosis of MGUS was associated with PH at similar 12.2% (OR = 4.50 [95%CI 1.86–10.90], p < 0.001.

There is a higher prevalence of MGUS in patients with PH and precapillary PH compared with normal controls. This association cannot be explained fully by other underlying diagnoses associated with PH. Assessing for this in patients with PH of unclear etiology may be reasonable in the workup of patients found to have PH.

• POEMS syndrome
• ascites
• case report
• hepatosplenomegaly
• pelvic effusion
• polyneuropathy

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare multisystem disorder that occurs due to an underlying plasma cell dyscrasia. A diagnosis is made with the presence of two mandatory criteria and at least one major and one minor criterion. We present a case of a 28-year-old patient who presented with weakness of bilateral arms and legs, thinning of hands, and swelling of bilateral lower limbs and abdomen. The patient also reported weight loss and loss of appetite. Examination revealed areflexic quadriparesis with sensory loss, diffuse lymphadenopathy, pleural effusion, ascites, and pulmonary hypertension. Investigations showed elevated erythrocyte sedimentation rate (ESR). Nerve conduction studies revealed severe axonal polyneuropathy of all nerves. Lymph node biopsy showed Castleman disease. A diagnosis of POEMS syndrome was made and he was sent for a stem cell transplant, which is the definitive treatment in patients eligible for stem cell transplant.

• CLINICAL NEUROLOGY
• NEUROPATHY
• NEUROPHYSIOLOGY
• NEURORADIOLOGY

• POEMS syndrome
• VEGF
• biclonal gammopathy
• polyneuropathy
• proteinuria

• Activities of Daily Living
• Humans
• Monoclonal Gammopathy of Undetermined Significance
• POEMS Syndrome/complications
• POEMS Syndrome/diagnosis
• Paraproteinemias/complications
• Vascular Endothelial Growth Factor A

• Castleman disease
• TAFRO syndrome
• idiopathic multicentric Castleman disease
• idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia

• Castleman Disease/diagnosis
• Herpesviridae Infections
• Humans
• Lymph Nodes/pathology
• Skin/pathology

A 76-year-old man presented with a four-month history of progressive bilateral lower limb muscle weakness and dysesthesia. The patient had extravascular volume overload, and laboratory findings confirmed hypothyroidism, renal dysfunction, and chronic inflammation. Serum protein and immunofixation electrophoresis revealed biclonality of immunoglobulin A (IgA)-kappa and IgA-lambda, which was attributed to chronic inflammation. Subsequently, we detected the proliferation of monoclonal plasma cells in the bone marrow, which led to a diagnosis of POEMS syndrome. Despite the initiation of chemotherapy, the patient died of aspiration pneumonia. In this case, biclonal gammopathy in peripheral blood delayed a diagnosis of POEMS syndrome.

POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud’s phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis.

A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud’s phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain–Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud’s phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud’s phenomenon, acrocyanosis, and white nails, but the neurological response was partial.

POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud’s phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud’s phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation.

• Autoimmune disease
• Case report
• POEMS syndrome
• Paraneoplastic polyneuropathy
• Raynaud disease

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome is a multisystem disorder. Peripheral neuropathy and monoclonal plasma cell disorder are the most common manifestations of POEMS. Although osteosclerotic or mixed sclerotic-lytic lesions are typical, osteolytic lesions are rarely encountered. We present a case of a 39-year-old male with a history of multiple endocrine disorders who presented with paresthesia, edema, and hyperpigmentation and was eventually diagnosed with POEMS syndrome. Patients with unexplained neuropathy should be evaluated for POEMS syndrome, especially when it is associated with other findings like endocrinopathy, organomegaly, skin changes, or edema.

POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. It is a rare paraneoplastic disorder related to plasma cell neoplasm. However, its pathophysiology has not yet been clearly elucidated. The production of pro-inflammatory cytokines is thought to play an important role in the pathogenesis of POEMS syndrome. Vascular endothelial growth factor level reflects disease activity, which is helpful for the diagnosis and evaluation of treatment response. Conventional agents such as corticosteroids and melphalan are effective and safe combination regimens. Autologous hematopoietic stem cell transplantation is another option for high-risk transplant-eligible patients. Radiotherapy is effective in patients with localized lesions. The anti-myeloma agents lenalidomide, thalidomide, and bortezomib have shown good treatment outcomes for POEMS syndrome; however, large-scale studies with long-term follow-up are required. Early identification and active treatment can improve the outcomes of POEMS syndrome patients.

• POEMS syndrome
• Polyneuropathy
• Transplantation
• Vascular endothelial growth factor

Background: POEMS syndrome (POEMS) is a rare plasma cell clonal paraneoplastic syndrome consisting of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes presenting with idiopathic multicentric Castleman disease (iMCD) histology, the treatment of which has not yet been well established. iMCD is also a distinctive rare non-clonal lymphoproliferative disorder, of which dramatic response to Siltuximab, a monoclonal anti-IL-6 antibody, has been reported recently. Methods: the differential diagnosis between POEMS and iMCD can be very challenging because of the identical histology, overlapping similar symptoms such as polyneuropathy, and vital signs insidiously presented to diagnose POEMS. Results: here, we report the case of a 53-year-old man with iMCD treated for 8 years developing sequential polyneuropathy, monoclonal gammopathy, and bone lytic lesions, all of which were confirmed after his iMCD achieved complete remission resulting from siltuximab administration and finally confirmed as POEMS. Conclusions: we describe the clinical ambiguity of disease presenting that we can face in the real world between iMCD and POEMS and emphasise careful, enduring observation lasting several years.

Monoclonal gammopathy associated with dermatological manifestations are a well-recognized complication. These skin disorders can be associated with infiltration and proliferation of a malignant plasma cells or by a deposition of the monoclonal immunoglobulin in a nonmalignant monoclonal gammopathy. These disorders include POEMS syndrome, light chain amyloidosis, Schnitzler syndrome, scleromyxedema and TEMPI syndrome. This article provides a review of clinical manifestations, diagnostics criteria, natural evolution, pathogenesis, and treatment of these cutaneous manifestations.

Cranial solitary plasmacytomas are uncommon lesions, and localization to the skull base is rare. Here we present a case in a 36-year-old woman who complained of dizziness and mild headaches. Radiographic imaging indicated the presence of a solitary skull base lesion in the posterior cranial fossa. Laboratory tests and imaging excluded systemic disease. A biopsy of the lesion confirmed the diagnosis of plasmacytoma. The patient was treated with proton-beam radiation and had a complete clinical and radiographic resolution, demonstrating the previously unreported utility of monotherapy with proton-beam radiation in such cases.

• POEMS syndrome
• Paraproteinemia
• Plasma cells
• Plasmocytoma
• Polyneuropathy