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Criteria for the diagnosis of POEMS syndrome Symptom/Sign Major criteria Polyneuropathy Monoclonal plasma cell proliferative disorder
Source publication
POEMS syndrome is characterized by peripheral neuropathy (PN), a clonal plasma cell disorder (PCD), organomegaly, endocrinopathy, skin changes, edema, sclerotic bone lesions, and thrombocytosis. Based on the improved response rates observed with peripheral blood stem cell transplantation (PBSCT) in patients with other PCDs, autologous PBSCT may be...
Contexts in source publication
Context 1
... acronym POEMS refers to the polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes seen in the majority of patients, but excludes other important character- istics germane to the syndrome including the sclerotic bone lesions, Castleman disease, papilledema, peripheral edema, ascites, pleural effusions, polycythemia, thrombocytosis, fatigue, and clubbing. 1,2 Not all features are required to make the diagnosis (Table 1); at a minimum, however, a patient must have: (1) peripheral neuropathy; (2) osteosclerotic myeloma (ie, a clonal plasma cell dyscrasia and at least one sclerotic bone lesion) or Castleman disease; and (3) at least one of the other mentioned features. 1 Typically, the overriding manifestation is that of progressive symmetrical sensorimotor neuropathy; other components such as organomegaly, edema, and skin changes are overshadowed by the neuropathy. ...
Context 2
... March 1999 andDecember 2003, 16 patients with POEMS syndrome underwent PBSCT at the Mayo Clinic. Patients were classified as having POEMS syndrome if they met the criteria described in Table 1. Peripheral blood stem cells were collected using either cyclophosphamide and growth factor (n 10) or growth factor alone (n 6). ...
Similar publications
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...
POEMS syndrome is a rare paraneoplastic disorder associated with plasma cell dyscrasia. The acronym stands for coexisting polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Other relevant features of the syndrome are papilledema, sclerotic bone lesions, Castleman’s disease, thrombocytosis, weight loss and increase...
Objective. Bone lesion is crucial for diagnosing and management of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome, a rare plasma cell disorder. This study is to compare the effectiveness of X-ray skeletal survey (SS) and computed tomography (CT) for detecting bone lesions in Chinese POEMS syndrome...
POEMS syndrome is a rare paraneoplastic disorder associated with an underlying plasma cell dyscrasia presenting polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. This study reviewed the clinical characteristics of 14 POEMS patients in Zhongshan hospital. The ratio of male to female was 9 : 5, and the average age wa...
POEMS syndrome (Crow–Fukase syndrome) is a rare paraneoplastic disorder. It is characterized by peripheral neuropathy, elevated
vascular endothelial growth factors (VEGFs), monoclonal gammopathy, sclerotic bone lesions and Castleman disease. Other important
clinical features are organomegaly, edema, ascites, papilledema, endocrinopathy, skin change...
Citations
... Table 6 lists the top 10 keywords by frequency. Clusters led by "POEMS syndrome" and "endothelial growth factor" appeared most frequently, followed by "polyneuropathy" and "stem-cell transplantation", and then "multiple-myeloma" and "diagnosis" [28][29][30][31][32]. Fig. 5B shows an overlay visualization of the keywords, with earlier keywords in blue and more recent keywords in yellow. ...
Background
POEMS syndrome is a rare and complex plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, high M protein levels, and skin changes. Understanding of POEMS syndrome has advanced rapidly since the 21st century. This study aims to summarize and evaluate the research status of POEMS syndrome in the past 23 years through a bibliometric and visualization analysis, and identify research focuses and emerging hotspots for the future.
Methods
POEMS syndrome-related articles published between January 1, 2000, and March 8, 2023, were systematically retrieved from the Web of Science Core Collection. Data processing and visualization analysis were carried out using a combination of R software, HistCite, VOSviewer, and CiteSpace.
Results
Since entering the 21st century, 3677 authors from 1125 institutions in 68 countries/regions have published 830 original and review articles on POEMS syndrome in 408 journals so far, among which the USA, Japan, and China published the most articles, and Mayo Clinic, Udice French Research Universities, and Peking Union Medical College listed the top three most prolific institutions. However, collaborative research across countries and groups in the study of POEMS syndrome remain significantly limited. Angela Dispenzieri ranked first in POEMS syndrome research from every aspect of authors, producing the most papers and contributing the most-cited article, followed by Satoshi Kuwabara and Sonoko Misawa. Internal medicine was the most productive journal on POEMS syndrome. "endothelial growth factor" was the keyword with the highest occurrence except for "POEMS syndrome", and "bevacizumab", "lenalidomide", "dexamethasone", and "management" were recognized as emerging topics.
Conclusion
This study utilized bibliometric and visualization analysis to systematically summarize the research of POEMS syndrome in the first two decades of the 21st century, offering a data-based and objective perspective on the field of POEMS syndrome and guiding researchers in the identification of novel research directions.
... With ASCT, case series suggest 100% of patients achieve at least some neurologic improvement, 18,60,99,152,158,161,[163][164][165][166][167][168][169][170][171][172][173][174][175][176][177][178] 180 The complete hematologic response rate was 46%, the neurologic response rate was 95%, and the VEGF response rate was 83%. After a median follow-up of 34 months, the estimated 3-year OS and PFS were 90% and 75%, respectively. ...
Disease Overview
POEMS syndrome is a life‐threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, 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.
Diagnosis
The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.
Risk Stratification
Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate.
Risk‐Adapted Therapy
For those patients with a dominant plasmacytoma, first‐line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low‐dose conventional therapy or as high‐dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
... The multisystem involvement of POEMS and its clinical polymorphism (the constellation of non-specific signs and symptoms) affecting different species usually delay diagnosis, with a median of 13-18 months [5,8] between onset of symptoms and diagnosis of POEMS [3]. ...
Introduction: POEMS syndrome consisting of polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) and skin changes (S), is a rare paraneoplastic syndrome resulting from an underlying plasma cell disorder. The multi-organ involvement associated with this syndrome extends beyond those listed in the acronym, such as extravascular volume overload (pleural effusion, ascites, oedema), sclerotic bone lesions, papilledema and thrombocytosis, and not all of the features listed in the acronym are required for diagnosis. Observation: we report a rare case of POEMS syndrome in an 83-year-old man with bilateral pleural effusions, initially attributed to pleural tuberculosis given the endemic context. Conclusion: the pleural involvement in patients with POEMS in our endemic tuberculosis context makes diagnosis difficult, delaying treatment. However, a good history and physical examination followed by appropriate investigations can differentiate this syndrome from other diseases.
... Induction therapy may reduce the incidence of complication [53]. Normalization of serum VEGF level at ASCT may prolong OS and PFS [54][55][56][57][58][59][60][61][62][63][64][65][66][67]. ...
POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.
... This effect implied the presence of a soluble factor that is responsible for the endothelial disruption, with possible candidates being VEGF and angiopoietin-2 (Ang-2), whose levels are elevated during disease flares [65,67]. These factors contribute to endothelial permeability in states such as sepsis [68][69][70]; at the same time, their levels are increased in diabetes and obesity and contribute to complications such as nephropathy and retinopathy, where endothelial hyperpermeability and degradation of the endothelial glycocalyx is a key factor [71,72]. Although an association between metabolic diseases and ISCLS has not been recognized, it is possible that the chronic endothelial dysfunction in these states could be a favorable background in a hyperinflammatory state like COVID-19. ...
Capillary leak syndrome is an under-diagnosed condition leading to serious hypoalbuminemia with diffuse edema, pulmonary edema, severe hypotension, and possibly death. Sepsis leading to hemophagocytic lymphohistiocytosis (HLH) is a major risk factor; however, capillary hyper-permeability is the core underlying pathophysiological mechanism. Endothelial dysfunction plays a major role in cardiometabolic disease through insulin resistance, lipotoxicity, and, eventually, oxidative stress and chronic inflammation. We review the literature concerning the aforementioned mechanisms as well-established risk factors for adverse COVID-19 outcomes. We especially focus on data regarding the underlying endothelial effects of SARS-CoV-2 infection, including direct damage and increased vascular leakage through a hyper-inflammatory cascade and diminished nitric oxide bioavailability. Interestingly, an increased incidence of hypoalbuminemia has been observed in patients with severe COVID-19, especially those with underlying cardiometabolic disease. Importantly, low albumin levels present a strong, positive association with poor disease outcomes. Therefore, in this review article, we highlight the important role of cardiovascular risk factors on endothelium integrity and the possible link of endothelial damage in the hypoalbuminemia-associated adverse prognosis of COVID-19 patients.
... The mechanism of the male penile erection, as well as cross-section, is shown in Figure 5A,B, where the aorta is directly connected to the penal artery. A significant blood input is essential for successful sexual performance [101,102]. As previously stated, normal penile erection is a neurovascular event that causes sexual stimulation and the release of NO hormones from endothelial cells [103,104]. ...
Purpose:
The role of erectile dysfunction (ED) has recently shown an association with the risk of stroke and coronary heart disease (CHD) via the atherosclerotic pathway. Cardiovascular disease (CVD)/stroke risk has been widely understood with the help of carotid artery disease (CTAD), a surrogate biomarker for CHD. The proposed study emphasizes artificial intelligence-based frameworks such as machine learning (ML) and deep learning (DL) that can accurately predict the severity of CVD/stroke risk using carotid wall arterial imaging in ED patients.
Methods:
Using the PRISMA model, 231 of the best studies were selected. The proposed study mainly consists of two components: (i) the pathophysiology of ED and its link with coronary artery disease (COAD) and CHD in the ED framework and (ii) the ultrasonic-image morphological changes in the carotid arterial walls by quantifying the wall parameters and the characterization of the wall tissue by adapting the ML/DL-based methods, both for the prediction of the severity of CVD risk. The proposed study analyzes the hypothesis that ML/DL can lead to an accurate and early diagnosis of the CVD/stroke risk in ED patients. Our finding suggests that the routine ED patient practice can be amended for ML/DL-based CVD/stroke risk assessment using carotid wall arterial imaging leading to fast, reliable, and accurate CVD/stroke risk stratification.
Summary:
We conclude that ML and DL methods are very powerful tools for the characterization of CVD/stroke in patients with varying ED conditions. We anticipate a rapid growth of these tools for early and better CVD/stroke risk management in ED patients.
... For fit patients, high-dose chemotherapy and/or autologous stem cell transplantation (ASCT) appear to be the best strategies [3][4][5][6]. ...
Autologous stem cell transplantation (ASCT) is a good option in fit young patients affected by POEMS syndrome. Few data are published on how to treat these patients in case of relapse. In this paper we described our two patients who underwent a second ASCT. From our experience, we can confirm that a second transplant is a real treatment option with good results and limited side effects. It provided excellent response and long-term disease-free survival in POEMS patients who relapsed after a first transplant, including late relapses.
... After autologous peripheral blood stem cell transplantation (aPBSCT) in nine patients, a significant relationship was only observed between VEGF level decreases and response of hypertrichosis (P = 0.007). Some clinical studies (11,12) and cases (9,(13)(14)(15) reported skin responses with overall improvement after systemic therapies but did not assess possible correlations of skin responses with VEGF levels. Therefore, we still know little about skin responses after systemic treatments of POEMS syndrome. ...
Background
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare paraneoplastic disease with a high prevalence of skin symptoms. Few studies have focused on skin responses to systemic treatment of this disease.Objective
To evaluate skin responses after treatment with low-dose lenalidomide plus dexamethasone and determine their relationship with vascular endothelial growth factor (VEGF) and hematological responses.MethodsA total of 41 consecutive Chinese patients who were at least 18 years of age with newly diagnosed POEMS syndrome were enrolled. 36 of them completed 12 cycles of treatment. Skin, serum VEGF, hematological and neurological responses were documented at 1, 2, 3, 6, 9, and 12 months during treatment.ResultsSkin manifestations were not associated with serum VEGF levels at baseline. Of the patients with hyperpigmentation, hemangiomas, hypertrichosis, white nails, or acrocyanosis separately, 26/34 (76.5%), 11/30 (36.7%), 14/15 (93.3%), 21/21 (100%), and 4/4 (100%) achieved complete responses at 12 months. Reduction in hyperpigmentation after 12 months reflected a good VEGF response (P = 0.017).Conclusion
Low-dose lenalidomide plus dexamethasone therapy is effective in reversing skin changes in patients with POEMS syndrome.Clinical Trial RegistrationClinicaltrials.gov, identifier NCT01816620
... 9 Neurological symptoms and general medical condition both underwent remarkable improvement in association with normalized VEGF levels observed after stem cell transplantation. [9][10][11] Then, quite a few new drugs have been developed to treat myeloma. Given the negative aspects of transplantation procedures as noted above and rapid progress with respect to the development of new treatments for myeloma, some of these new agents, among them several immunomodulatory drugs and proteasome inhibitors, have been evaluated for their safety and efficacy in patients diagnosed with POEMS syndrome. ...
POEMS syndrome and Guillain‐Barré syndrome (GBS) are rare and potentially intractable peripheral neuropathies. This review considers the development of new therapeutic interventions for these disorders using drug repositioning strategies. The prognosis for patients diagnosed with POEMS syndrome was previously quite poor; however, therapeutic interventions based on those used successfully for the treatment of myeloma have significantly improved the prognosis of this disease. While many new drugs have been developed to treat myeloma, thalidomide is the only drug with a safety and efficacy profile that has been documented by the results of a randomized controlled trial. While POEMS syndrome is an extraordinarily rare disease, prospective controlled trials should be performed to establish evidence‐based therapeutic strategies. Similarly, GBS is an acute, immune‐mediated polyneuropathy. While many patients achieve full recovery, others go on to experience long‐lasting neurological sequelae; this latter group includes patients who were treated appropriately with immunoglobulins or plasmapheresis. Complement activation has been associated with severe nerve injury in patients diagnosed with GBS; indeed, inhibition of complement activation prevented disease‐associated paralysis and nerve injury in a relevant murine model. Eculizumab, an antibody that targets complement protein C5, is a potent inhibitor of complement activation that has been approved for the treatment of complement‐mediated disorders. A recent phase 2 clinical trial of eculizumab for GBS performed in Japan revealed that eculizumab may be effective for the treatment of patients with this disease. Further confirmation of this finding will be a major step forward with respect to the management of GBS.
... Outstanding efficacy of HDT with ASCT in POEMS syndrome has been reported from a number of case series. [40][41][42][43][44][45][46][47] The most remarkable effect of transplantation is rapid recovery for patients with severe neuropathy, even for patients who required a wheel chair. 43 46 The necessity of maintenance therapy after transplantation to prevent disease relapse should be investigated in the future. ...
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare cause of demyelinating polyneuropathy associated with monoclonal proliferation of plasma cells. Because the disorder shows diverse symptoms, its diagnosis is often difficult. Previously reported diagnostic criteria had many items and their accuracy was not confirmed. Recently new diagnostic criteria are proposed, which consist of only seven items and have better diagnostic accuracy. Since around 2000, treatments for myeloma, such as high‐dose chemotherapy with autologous stem cell transplantation, immunomodulatory drugs, and proteasome inhibitors, have been applied to POEMS syndrome. A recent national survey conducted in Japan has revealed that the prognosis has been prominently improved by these plasma cell‐targeting therapeutic interventions: 10‐year overall survival was 93%. Conversely, 24% of the patients were still treated with corticosteroid alone as an initial treatment, suggesting that recognition of the disorder is not sufficient. Accurate diagnosis and proper treatment in the early phase of the disease are essential to further improve prognosis of POEMS syndrome. The present review focuses on diagnosis, treatment, and the current status of the disease in Japan.
