The patient died due to multisystem complications nine months after presenting with acute on chronic renal failure. [cancerjournal.net]

The median age at presentation was 51 years; 63% were men. Median survival was 165 months. [ncbi.nlm.nih.gov]

In the present series, median survival was 165 months. [doi.org]

• Fever

  The multicentric form usually presents with generalized lymphadenopathy, fever, weight loss, and other systemic manifestations and it can be associated with malignancy, especially POEMS syndrome. [seer.ufrgs.br]

  A 53-year-old female patient was admitted to our hospital with limb weakness, numbness, edema, abdominal distention, and fever. [oaepublish.com]

  […] peripheral motor and sensory manifestations) Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis) Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever [msdmanuals.com]

  Five patients had serious adverse events: three in the thalidomide group (transient cardiac arrest, heart failure, and dehydration) and two in the placebo group (ileus and fever). No deaths occurred during the randomised study. [ncbi.nlm.nih.gov]

• Fatigue

  Five months later, the patient started to have lower extremity numbness and fatigue. Initial laboratory tests did not reveal positive monoclonal immunoglobulin. [ncbi.nlm.nih.gov]

  Other symptoms Visual disturbance, headache, and nausea Pericardial effusion Fluid in arms and legs Weight loss Fatigue Causes The real cause of POEMS syndrome remains unknown. [anavara.com]

  Common Symptoms Enlarged liver, spleen and/ or lymph nodes, progressive polyneuropathy, hyperpigmentation, weight loss, fatigue, blood clots, endocrine gland disorders, monoclonal plasma cell proliferative disorders, restrictive lung disease, pulmonary [autoimmuneinstitute.org]

  We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019 [patientslikeme.com]

• Progressive Polyneuropathy

  Before the implementation of aPBSCT, the clinical course of POEMS syndrome was characterized by progressive polyneuropathy potentially leading to death for respiratory failure. [ncbi.nlm.nih.gov]

  Common Symptoms Enlarged liver, spleen and/ or lymph nodes, progressive polyneuropathy, hyperpigmentation, weight loss, fatigue, blood clots, endocrine gland disorders, monoclonal plasma cell proliferative disorders, restrictive lung disease, pulmonary [autoimmuneinstitute.org]

  The major clinical feature of this syndrome is a chronic progressive polyneuropathy with a predominant motorial disability [1]. In our study, the initial symptoms of most patients were sensory and also motorial symptoms (Table 1). [hindawi.com]

• Pleural Effusion

  Her chest X-ray and computed tomographic imaging showed bilateral pleural effusion, and her chest drainage revealed chylothorax. Detailed examination failed to reveal the definitive cause of the chylothorax. [ncbi.nlm.nih.gov]

  Bone pain Demonstration of clonal plasma cells Neuropathy Pleural effusion Renal failure Board review answer #1 C and D. Neuropathy and pleural effusion; volume overload and neuropathy are characteristic features of POEMS syndrome. [pathologyoutlines.com]

  effusion, papilledema, and fever. [msdmanuals.com]

• Clubbed Finger

  […] of fingers Clubbed fingers Clubbing (hands) Finger clubbing [ more ] 0100759 Diabetes mellitus 0000819 Erectile abnormalities Abnormal erection 0100639 Fatigue Tired Tiredness [ more ] 0012378 Gynecomastia Enlarged male breast 0000771 Hemangioma Strawberry [rarediseases.info.nih.gov]

• Hepatomegaly

  Hepatomegaly was significantly reduced after the completion of BorCyDex. Our case eventually received autologous hematopoietic stem cell transplantation with high dose melphalan. [ncbi.nlm.nih.gov]

  A diagnosis of POEMS syndrome was reached based on the presence of polyneuropathy, hepatomegaly, endocrinopathy (diabetes mellitus), monoclonal gammopathy, and skin hyperpigmentation. [pmj.bmj.com]

• Hypertension

  But she died after two years because of severe pulmonary hypertension. Pulmonary hypertension can be a clinical manifestation of POEMS syndrome in this patient. Because before the ASCT, her ECO had releaved the mild pulmonary hypertension. [peertechzpublications.com]

  Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with idiopathic portal hypertension (IPH). [ncbi.nlm.nih.gov]

  Other clinical features which may be present include EDEMA ; CACHEXIA ; microangiopathic glomerulopathy; pulmonary hypertension ( HYPERTENSION, PULMONARY ); cutaneous necrosis; THROMBOCYTOSIS ; and POLYCYTHEMIA. [mesh.kib.ki.se]

  The presentation as ascitic syndrome with portal hypertension is very rare, and no cases of portal hypertension associated to portal cavernomatosis, as the present case, have been reported. Conflicts of interest. [actagastro.org]

• Thrombosis

  This case evokes the need to consider the diagnosis of POEMS syndrome for young patients with symptoms of arterial thrombosis but no high risk factors of thrombosis. [ncbi.nlm.nih.gov]

  Causes of death include progressive anasarca, thrombosis, and inanition. The documents contained in this web site are presented for information purposes only. [orpha.net]

  Nevertheles, in anyone of them portal thrombosis was demonstrated, and there was described only one case of a patient with POEMS syndrome, who developed portal thrombosis. Key words. [actagastro.org]

• Acrocyanosis

  Other possible clinical manifestations include: skin changes such as hyperpigmentation, skin thickening, and excessive sweating; acrocyanosis, that is, the bluish color of the fingers and toes; Raynaud's phenomenon, a circulatory disorder characterized [gsdinternational.com]

  Report of Case A 60-year-old woman presented with a progressive sensorimotor polyneuropathy, weight loss, and acrocyanosis of the distal extremities and nose. [mayoclinicproceedings.org]

  […] adrenal, thyroid, pituitary, gonadal, parathyroid, or pancreatic) not satisfied by diabetes mellitus or hypothyroidism as these are common in the general population skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis [radiopaedia.org]

  The most common findings are hyperpigmentation, hypertrichosis, acrocyanosis, plethora and hemangiomas [ 5 ]. [karger.com]

  Extravascular volume overload (edema, pleural effusion, ascites), Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic); hypogonadism most common Skin changes (hyperpigmentation, hypertrichosis, glomeruloid angiomata, plethora, acrocyanosis [pathologyoutlines.com]

• Hyperpigmentation

  Links: polyneuropathy hepatosplenomegaly gynaecomastia secondary amenorrhoea diabetes mellitus hyperpigmentation hypertrichosis osteosclerotic myeloma [gpnotebook.co.uk]

  The most common findings are hyperpigmentation, hypertrichosis, acrocyanosis, plethora and hemangiomas [ 5 ]. [karger.com]

  JAMA Oncol. 2021 Jun 3 polyneuropathy hepatosplenomegaly gynaecomastia secondary amenorrhoea diabetes mellitus hyperpigmentation hypertrichosis osteosclerotic myeloma [gpnotebook.com]

  We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. [ncbi.nlm.nih.gov]

  Progressive skin hyperpigmentation in parallel to sensorimotor neuropathy had been developed since 20 years ago.sclerodermoid changes was evident. [authorea.com]

• Hypertrichosis

  Links: polyneuropathy hepatosplenomegaly gynaecomastia secondary amenorrhoea diabetes mellitus hyperpigmentation hypertrichosis osteosclerotic myeloma [gpnotebook.co.uk]

  JAMA Oncol. 2021 Jun 3 polyneuropathy hepatosplenomegaly gynaecomastia secondary amenorrhoea diabetes mellitus hyperpigmentation hypertrichosis osteosclerotic myeloma [gpnotebook.com]

  Increased production of VEGF is associated with the development of angiomas and hypertrichosis. Manifestation This section has been translated automatically. Mainly occurring in middle-aged patients (40-50 years). [altmeyers.org]

  Regarding the cutaneous and laboratory findings, the existence of hemangioma or hypertrichosis is indicative of POEMS syndrome. [ncbi.nlm.nih.gov]

• Flushing

  […] thyroid, pituitary, gonadal, parathyroid, or pancreatic) not satisfied by diabetes mellitus or hypothyroidism as these are common in the general population skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing [radiopaedia.org]

  The skin is commonly affected with development of hyperpigmentation, hemangiomas, hypertrichosis, leukonychia or nail clubbing, sclerodermoid changes, lipodystrophy, and flushing. The main ocular manifestation is papilledema. [orpha.net]

  Other findings included hypertrichosis, thickened skin, Raynaud's phenomenon, and clubbing; one patient had idiopathic flushing. Additional abnormalities found on physical examination are listed in Table 1. [nejm.org]

  Other skin problems may include: Localised hyperhidrosis (excessive sweating) Eruptive glomeruloid haemangiomas on the trunk and distal limbs Flushing Terry nails: white proximal and red distal nail Nail clubbing Raynaud phenomenon Facial lipotrophy [dermnetnz.org]

• Hirsutism

  […] syndrome A multisystem disease characterized by an acronym, POEMS, derived from Polyneuropathy–distal symmetric progressive muscle weakness, paresthesias, ↓ nerve conduction velocity; Organomegaly–hepatosplenomegaly, lymphadenopathy; Endocrinopathy–hirsutism [medical-dictionary.thefreedictionary.com]

  Patients may have the following: Hepatomegaly Hypogonadism Monoclonal gammopathy Osteoclastic bone lesions Polyneuropathy (peripheral motor and sensory manifestations) Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, [msdmanuals.com]

  […] ascities,pedal edema) endocrinopathy(adrenal,pitutary,gonadal,parathyroid)dm,hypothyroidism alone insufficient </li></ul><ul><li>Papilledema </li></ul><ul><li>Thrombocytosis or Polycythemia </li></ul><ul><li>Skin changes-hyperpigmentation,dermis thickening,hirsutism [slideshare.net]

• Skin Disease

  Where to Start DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition. [rarediseases.info.nih.gov]

  Arch Dermatol 146:615–623 Article PubMed Google Scholar Araki N et al (2016) POEMS syndrome and calciphylaxis: an unrecognized cause of abnormal small vessel calcification Rare skin diseases. [link.springer.com]

• Polyneuropathy

  POEMS is an acronym for: polyneuropathy: slow onset of mixed motor and sensory polyneuropathy peripheral nerve demyelination with some axonal degeneration organomegaly: hepatosplenomegaly endocrinopathy: gynaecomastia secondary amenorrhoea diabetes mellitus [gpnotebook.co.uk]

  Abstract Objectives: To determine the risk factors and incidence of cerebral infarction associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome. [n.neurology.org]

  The progression of the disease involves a number of neurovascular sequelae, including symmetric sensory-motor polyneuropathy resembling chronic inflammatory demyelinating polyneuropathy, cerebrovascular accidents, and papilledema associated with increased [ncbi.nlm.nih.gov]

  It is characterized by peripheral, symmetrical, ascending, sensorimotor polyneuropathy. Both demyelinating and axonal polyneuropathy are seen on electrodiagnostic studies as observed in our patient. [omcr.oxfordjournals.org]

• Papilledema

  Despite the association of POEMS with papilledema, treatment for this finding typically includes acetazolamide and therapeutic large volume lumbar punctures. [ncbi.nlm.nih.gov]

  Papilledema may present in 52% of the patients [ 5 ]. Papilledema in POEMS syndrome is not associated with raised intracranial pressure. The cause of papilledema is not known. Other common ocular symptoms are pain, diplopia and blurring of vision. [omcr.oxfordjournals.org]

  The main ocular manifestation is papilledema. Extravascular overload mainly manifests as peripheral edema, and sometimes as pleural effusion, ascites, and pericardial effusions. [orpha.net]

  Polyneuropathy (peripheral motor and sensory manifestations) Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis) Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema [msdmanuals.com]

An extensive diagnostic workup revealed POEMS syndrome. Serum concentrations of vascular endothelial growth factor (VEGF) were highly elevated, consistent with POEMS syndrome. [ncbi.nlm.nih.gov]

Some may present with known history of a specific monoclonal gammopathy, while in others, boney lesions of osteomyeloma or other evidence of a plasma cell dyscrasia are detected during workup for POEMS in patients with neuropathy. [dermatologyadvisor.com]

• Pericardial Effusion

  Findings suggestive of POEMS syndrome include pericardial effusion, left ventricular hypertrophy with moderately impaired systolic function, pulmonary hypertension, seroperitoneum, and hepatosplenomegaly. [wikidoc.org]

  The organ manifestations that significantly influence the clinical course of POEMS syndrome are defined as follows: Extravascular volume overload (pericardial effusion, ascites - 50 to 70% of patients) Organ megaly (splenomegaly, hepatomegaly in 50% of [altmeyers.org]

  Extravascular overload mainly manifests as peripheral edema, and sometimes as pleural effusion, ascites, and pericardial effusions. An increased risk of arterial and/or venous thrombosis is reported (up to 20% of patients). [orpha.net]

  Heart ultrasonic examination (serious pulmonary hypertension), abdominal ultrasonic (hepatosplenomegaly and slight ascites), chest computed tomography scan revealed (1) bilateral pulmonary emphysema, (2) slight bilateral pleural and pericardial effusion [freethesaurus.com]

  X-ray and computed tomography of the patient’s body revealed swelling of the supraclavicular lymph nodes, cardiomegaly, pleural effusion, pericardial effusion, splenomegaly and osteolytic lesions of right side acetabulum and pubica. [pulsus.com]

• Thrombocytosis

  Recognition of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease is the [ncbi.nlm.nih.gov]

  / polycythemia Laboratory Monoclonal light chain (especially lambda) VEGF levels are high Thrombocytosis or erythrocytosis, absence of cytopenias Radiology description Bone lesions Signs of volume overload (e.g. pleural effusion, ascites) Prognostic [pathologyoutlines.com]

  Treatment of thrombocytosis should be considered in patients without a contraindication. Treatment of the syndrome may be the best approach to decreasing risk of cerebral infarction in these patients. [n.neurology.org]

• Human Herpesvirus 8

  Castleman`s Disease and POEMS Syndrome: case reporte and review of literature Abstract Castleman’s disease is a lymphoproliferative disorder related with human herpesvirus 8 and HIV, presenting in two different ways. [seer.ufrgs.br]

  About 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8). [msdmanuals.com]

  Because POEMS syndrome is associated with Castleman disease and angioma formation, a role for human herpesvirus 8 (HHV-8) has been postulated; however, early studies have not demonstrated an association. [emedicine.com]

• Pleural Effusion

  Her chest X-ray and computed tomographic imaging showed bilateral pleural effusion, and her chest drainage revealed chylothorax. Detailed examination failed to reveal the definitive cause of the chylothorax. [ncbi.nlm.nih.gov]

  Bone pain Demonstration of clonal plasma cells Neuropathy Pleural effusion Renal failure Board review answer #1 C and D. Neuropathy and pleural effusion; volume overload and neuropathy are characteristic features of POEMS syndrome. [pathologyoutlines.com]

  effusion, papilledema, and fever. [msdmanuals.com]

Treatment of thrombocytosis should be considered in patients without a contraindication. Treatment of the syndrome may be the best approach to decreasing risk of cerebral infarction in these patients. [n.neurology.org]

Forty-one treatment-naive patients were enrolled from April 2014 to November 2014. [ncbi.nlm.nih.gov]

Abstract POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a multisystem disorder with a good long-term prognosis. [ncbi.nlm.nih.gov]

Prognosis The prognosis is generally good if diagnosis is early and appropriate treatment is provided. Causes of death include progressive anasarca, thrombosis, and inanition. [orpha.net]

New agents and new application of stem-cell transplantation have dramatically improved outcomes for these previously uniformly poor prognosis disorders. [mayoclinic.pure.elsevier.com]

Common stroke etiologies comprised suspected vascular structural abnormalities leading to vessel dissection and stenosis, in addition to embolism from a proximal source. [n.neurology.org]

Although the etiology of ONJ is not known, it is likely that several factors are at play, including endothelial cell damage, decreased angiogenesis, and microvascular compromise. [ncbi.nlm.nih.gov]

Summary Epidemiology Exact prevalence and incidence rates are not known. The only estimates are from Japan. In this country, prevalence has been reported to be about 1/330,000. Male predilection has been reported (2.5:1). [orpha.net]

Jump to navigation Jump to search POEMS syndrome Microchapters Home Patient Information Overview Historical Perspective Pathophysiology Causes Differentiating POEMS syndrome from other Diseases Epidemiology and Demographics Risk Factors Screening Natural [wikidoc.org]

[…] hyperplasia, 11 - 30% of cases, most commonly multicentric Castleman disease) Terminology POEMS acronym coined in 1980 by Bardwick et al. ( Medicine (Baltimore) 1980;59:311 ) Other names: osteosclerotic myeloma, Takatsuki syndrome, Crow-Fukase syndrome Epidemiology [pathologyoutlines.com]

Epidemiology[3] POEMS syndrome is probably rare (of the order of hundreds of cases are reported) but may be under-diagnosed and the incidence is unknown. Peak incidence is age 40-60. Aetiology and pathogenesis[1, 4] The cause is unknown. [patient.info]

Vascular endothelial growth factor (VEGF) plays an essential role in the pathophysiology of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome. [ncbi.nlm.nih.gov]

Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms, signs and laboratory findings. Patients must have both mandatory criteria and ≥ 1 major and ≥ 1 minor criteria. [msdmanuals.com]

Pathophysiology The pathophysiology of POEMS syndrome is not well understood. In all patients, a plasma cell disorder underlies the development of the syndrome; however, the mechanism by which this occurs is unknown. [emedicine.com]

In this work we studied the clinicopathologic characteristics of the POEMS syndrome to improve early diagnosis to prevent irreversible damage. [ncbi.nlm.nih.gov]

Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 POEMS syndrome echocardiography and ultrasound On the Web Most recent articles Most cited articles Review articles CME Programs Powerpoint slides [wikidoc.org]

Prevention What are the risk factors for POEMS syndrome? There are no known risk factors for POEMS syndrome. Can POEMS syndrome be prevented? POEMS isn’t preventable. [my.clevelandclinic.org]

First, symptomatic treatments are always critical and may prevent life-threatening complications. Second, in most cases, control of the underlying B-cell lymphoproliferation may improve symptoms of the disease. [cancernetwork.com]