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POEMS Syndrome


Presentation

Fatigue
  • In this case report, we present a 48-year-old male patient who presented with malaise, fatigue, diarrhea, and abdominal swelling.[ncbi.nlm.nih.gov]
  • 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 (hypothyroidism), diabetes, sexual problems, fatigue, swelling in your limbs, and problems with metabolism and other essential functions. Monoclonal plasmaproliferative disorder.[mayoclinic.org]
  • 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]
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]
  • Cutaneous manifestations are heterogeneous, with diffuse cutaneous hyperpigmentation, hemangiomas and hypertrichosis occurring more frequently.[ncbi.nlm.nih.gov]
Macula
  • OCT examination of the optic nerve head and the macula has an important additional role in the diagnosis and follow-up of POEMS syndrome and Castleman disease.[ncbi.nlm.nih.gov]
Dermatitis
  • Minqi Luo, Huimin Dong, Yuanhang Zhu, Jiangling Chen, Hongbin Zhang and Lin Li, POEMS syndrome initially presenting with acral dermatitis, International Journal of Dermatology, 55, 12, (e612-e615), (2015). Taxiarchis V. Kourelis, Francis K.[doi.org]
Intracranial Hemorrhage
  • Izumi Yamaguchi, Junichiro Satomi, Nobuaki Yamamoto, Shotaro Yoshioka, Yoshiteru Tada, Kenji Yagi, Yasuhisa Kanematsu and Shinji Nagahiro, Coexistence of Quasi-moyamoya Disease and POEMS Syndrome in a Patient with Intracranial Hemorrhage: A Case Report[doi.org]

Workup

  • 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]
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
  • 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]
Liver Biopsy
  • The liver biopsy suggested hepatoportal sclerosis, compatible with idiopathic portal hypertension (IPH). Under a band ligation program, with beta-blocker, diuretics and prophylactic anticoagulation, the patient remains stable.[ncbi.nlm.nih.gov]

Treatment

  • 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

  • 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]

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • It is clear that attention needs to be paid to prevent bacterial, fungal and viral infection after HSCT. It is also important to keep in mind that tuberculous and nontuberculous mycobacteria (NTM), in rare cases, lead to lethal complications.[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 References Rajkumar SV. POEMS syndrome. .[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]

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