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

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

  Abnormal hormone levels that can result in underactive thyroid (hypothyroidism), diabetes, sexual problems, fatigue, swelling in your limbs, and problems with metabolism and other essential functions. Monoclonal plasmaproliferative disorder. [mayoclinic.org]

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

  Abnormal hormone levels that can result in underactive thyroid gland, diabetes, sexual problems, fatigue, swelling in your limbs and problems with metabolism and other essential functions. Monoclonal plasma-proliferative disorder. [humanitas.net]

• Soft Tissue Mass

  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, [ncbi.nlm.nih.gov]

• Acrocyanosis

  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]

  0004054 Sclerosis of skull base Dense bone of skull base 0002694 Sclerotic vertebral endplates 0004576 Thickened skin Thick skin 0001072 Thrombocytosis Increased number of platelets in blood 0001894 Weight loss 0001824 5%-29% of people have these symptoms Acrocyanosis [rarediseases.info.nih.gov]

  These changes most commonly are hyperpigmentation and/or hypertrichosis (abnormal amount of hair growth over the body) but less commonly include glomeruloid hemangioma, signs or symptoms of Hypervolemia (e.g. edema and ascites ), acrocyanosis (blue discoloration [en.wikipedia.org]

• Enlarged Blind Spot

  blind spots, or progressive constriction of the visual field. 8 22 38 119 Sclerotic bone lesions occur in most patients. [bloodjournal.hematologylibrary.org]

• Retinal Hemorrhage

  Retinal manifestations included retinal hemorrhage, subretinal fluid, macular edema, and cotton wool spot. The difference in mean serum VEGF concentrations between patients with and without ODE was significant (P = 0.017). [ncbi.nlm.nih.gov]

• Cotton Wool Spots

  Retinal manifestations included retinal hemorrhage, subretinal fluid, macular edema, and cotton wool spot. The difference in mean serum VEGF concentrations between patients with and without ODE was significant (P = 0.017). [ncbi.nlm.nih.gov]

• Hypertrichosis

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

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

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]

• Multiple Lytic Bone Lesions

  The prognosis is best for patients with a single lytic lesion. The prognosis is worst for patients with a plasma cell disorder involving the bone marrow. [13] Patients with multiple lytic bone lesions have an intermediate prognosis. [emedicine.com]

• Right Pleural Effusion

  CT showed a massive right pleural effusion and a sclerotic lesion in the right ninth rib. Histopathological examination of the rib revealed IgA lambda-type plasmacytoma. Serum vascular endothelial growth factor was extremely high at 5530 pg/mL. [ncbi.nlm.nih.gov]

• Thrombocytosis

  In addition to peripheral neuropathy, the presence of hepatosplenomegaly, skin changes, polycythaemia and thrombocytosis prompted for further investigations. [ncbi.nlm.nih.gov]

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

Some patients progress after radiation therapy and require systemic treatment. Alkylators are the main treatment, along with lenalidomide and to a lesser extent thalidomide and bortezomib. [orpha.net]

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]

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]

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

Plasma Cell Leukemia: Causes, Symptoms, Treatment, Prognosis, Risk Factors [epainassist.com]

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]

Etiology not established, likely secondary to imbalance of inflammatory cytokines. [ajnr.org]

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]

[…] 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]

The epidemiologic and clinical characteristics of the patients are summarized in Table 1. [jamanetwork.com]

Review article: portal vein obstruction–epidemiology, pathogenesis, natural history, prognosis and treatment. Aliment Pharmacol Ther 2015; 41: 276-292. Ganti AK, Pipinos I, Culcea E, Armitage JO, Tarantolo S. [actagastro.org]

Epidemiology Frequency POEMS syndrome is rare. Several hundred cases have been described in the literature; however, the incidence may be underreported because the syndrome may go unrecognized. [emedicine.com]

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]

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]

Clinical trials Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease. POEMS syndrome care at Mayo Clinic June 23, 2017 Rajkumar SV. [mayoclinic.org]

Paba-Prada explained that the best way to prevent a relapse is a stem cell transplant using my own cells,” Anyanwu relates. [blog.dana-farber.org]

Michael Tomasson, Mahmoud Ali, Vanessa De Oliveira, Qian Xiao, Yogesh Jethava, Fenghuang Zhan, Adam Fitzsimmons and Melissa Bates, Prevention Is the Best Treatment: The Case for Understanding the Transition from Monoclonal Gammopathy of Undetermined Significance [doi.org]