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Clinical Lymphoma, Myeloma & Leukemia Jan 2022POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal plasma protein [M protein], and skin changes) is a rare paraneoplastic disorder associated...
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal plasma protein [M protein], and skin changes) is a rare paraneoplastic disorder associated with underlying plasma cell neoplasia. Although limited-stage disease can be treated with radiotherapy, treatment for the more advanced disease remains unclear. The most commonly used therapies for POEMS syndrome include alkylators and steroids, high-dose chemotherapy with autologos stem cell transplantation, lenalidomide, and bortezomib. In general, patients tend to have excellent prognosis if the diagnosis is made early and appropriate therapy is used. Here we present a systematic review of the efficacy and safety of treatment regimens used to treat POEMS syndrome in the adult population. Combinations of immunomodulatory agents with corticosteroids were most frequently utilized regimens with durable hematological and neurological responses. Combinations of proteasome inhibitors and alkylating agents with corticosteroids, although less frequently utilized, appear to have reasonable safety and efficacy profiles.
Topics: Female; Humans; Male; Middle Aged; POEMS Syndrome
PubMed: 34507924
DOI: 10.1016/j.clml.2021.07.033 -
Eye (London, England) Jul 2022Paraneoplastic syndromes affecting the visual system are a group of conditions that arise in the systemic malignancy framework. In this review, we have provided a... (Review)
Review
Paraneoplastic syndromes affecting the visual system are a group of conditions that arise in the systemic malignancy framework. In this review, we have provided a detailed and comprehensive overview of the published literature on the various ophthalmic paraneoplastic manifestations. A systematic review of many databases has been performed to identify ample literature on the paraneoplastic syndromes related to ophthalmology. We have discussed here the clinical features, pathogenesis, and treatment strategies of various ophthalmic paraneoplastic syndromes. It can be challenging to distinguish these disorders from their non-paraneoplastic counterparts and to determine the appropriate systemic assessment for the tumour responsible, to have a proper approach towards the management of the syndrome. METHOD: We searched PubMed, Science Direct and Journal of Ophthalmology for studies published in English between 1995 and April 2020, incorporating the general search term "paraneoplastic ocular syndrome" with connecting terms relevant to subheadings-e.g. Key search terms were cancer-associated retinopathy, (CAR), melanoma-associated retinopathy, (MAR), paraneoplastic retinopathy, autoimmune retinopathy, autoimmune-related retinopathy, and optic neuropathy, (ARRON), acute zonal occult outer retinopathy, (AZOOR), paraneoplastic vitelliform maculopathy, paraneoplastic vitelliform retinopathy, bilateral diffuse uveal melanocytic proliferation, (BDUMP), paraneoplastic optic neuropathy, (PON), polyneuropathy, organomegaly, endocrinopathy, monoclona gammopathy, and skin changes syndrome (POEMS) and various other terms. References from identified studies have been reviewed and included if deemed appropriate, valid, and scientifically important. If referenced in a selected English paper, we contemplated papers in other languages too. We preferentially selected papers that have been published in the last 10 years, but we have included relevant older references.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Optic Nerve Diseases; Paraneoplastic Syndromes, Ocular; Retinal Diseases; Retinal Neoplasms
PubMed: 34345027
DOI: 10.1038/s41433-021-01676-x -
Muscle & Nerve Jun 2016We conducted a systematic literature review on psychological and behavioral comorbidities in patients with inflammatory neuropathies. In Guillain-Barré syndrome (GBS),... (Review)
Review
We conducted a systematic literature review on psychological and behavioral comorbidities in patients with inflammatory neuropathies. In Guillain-Barré syndrome (GBS), psychotic symptoms are reported during early stages in 30% of patients. Typical associations include mechanical ventilation, autonomic dysfunction, inability to communicate, and severe weakness. Anxiety and depression are frequent comorbidities. Anxiety may increase post-hospital admissions and be a predictor of mechanical ventilation. Posttraumatic stress disorder may affect up to 20% of ventilated patients. Sleep disturbances are common in early-stage GBS, affecting up to 50% of patients. In chronic inflammatory demyelinating polyradiculoneuropathy, memory and quality of sleep may be impaired. An independent link between depression and pretreatment upper limb disability and ascites was reported in POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin) syndrome, with an association with early death. Hematological treatment of POEMS appears effective on depression. Published literature on psychological/behavioral manifestations in inflammatory neuropathies remains scarce, and further research is needed. Muscle Nerve 54: 1-8, 2016.
Topics: Databases, Bibliographic; Guillain-Barre Syndrome; Humans; Mood Disorders; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Psychotic Disorders; Sleep Wake Disorders
PubMed: 26999767
DOI: 10.1002/mus.25112 -
Leukemia & Lymphoma Sep 2014The purpose of this pooled analysis was to synthesize all available data so as to evaluate the efficacy and safety of lenalidomide in patients with POEMS syndrome.... (Meta-Analysis)
Meta-Analysis Review
The purpose of this pooled analysis was to synthesize all available data so as to evaluate the efficacy and safety of lenalidomide in patients with POEMS syndrome. Eligible articles were identified by a search in MEDLINE and ClinicalTrials.gov databases using a predefined combination. Eligible cases of patients treated in our department were additionally included. Overall, 51 patients were included. The median age of patients was 54.5 years (range: 32-79 years). Lenalidomide was given as first- or second-line treatment in 28.6% and 47.6% of patients, respectively. Hematological responses included complete response in 18.6%, very good partial response in 39.5% and partial response in 37.2% of cases. Vascular endothelial growth factor (VEGF) reduction was reported in all cases. Neuropathy improved in 92.0% of cases and stabilized in 8%. The progression-free survival (PFS) estimate at 12 months was 93.9%. Lenalidomide can represent a safe and effective option for the treatment of patients with POEMS.
Topics: Adult; Aged; Angiogenesis Inhibitors; Humans; Immunologic Factors; Lenalidomide; Middle Aged; POEMS Syndrome; Thalidomide; Treatment Outcome
PubMed: 24295131
DOI: 10.3109/10428194.2013.869329 -
The Cochrane Database of Systematic... Jun 2012POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with monoclonal... (Review)
Review
BACKGROUND
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with monoclonal plasma cell proliferative disorder and multiorgan involvement. The pathogenesis of POEMS syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably secreted by plasmacytomas, is likely to be responsible for most of the characteristic symptoms. POEMS syndrome is a potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy, massive pleural effusion or ascites, or thromboembolic events. There is a need for efficacious therapy to improve prognosis. This is the first update of a review first published in 2008.
OBJECTIVES
To assess the effects of treatment for POEMS syndrome.
SEARCH METHODS
We searched the Cochrane Neuromuscular Disease Group Specialized Register (23 February 2012), CENTRAL (2012, Issue 2), MEDLINE (January 1966 to February 2012), EMBASE (January 1980 to February 2012) and CINAHL Plus (January 1937 to February 2012) for all papers on POEMS syndrome
SELECTION CRITERIA
We sought all randomized and quasi-randomized controlled trials, and non-randomized controlled studies. Since we discovered no such clinical trials, we assessed and summarized all retrospective case series including five or more patients in the 'Discussion' section.
DATA COLLECTION AND ANALYSIS
Two review authors independently reviewed and extracted details of all potentially relevant trials with any treatment for POEMS syndrome. We then collated and summarized information on the outcome.
MAIN RESULTS
We found no randomized or non-randomized prospective controlled trials of treatment for POEMS syndrome. We summarized the results of retrospective case series containing five or more patients in the 'Discussion' section.
AUTHORS' CONCLUSIONS
There are no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome on which to base practice.
Topics: Adrenal Cortex Hormones; Hematopoietic Stem Cell Transplantation; Humans; Melphalan; POEMS Syndrome; Retrospective Studies; Thalidomide
PubMed: 22696361
DOI: 10.1002/14651858.CD006828.pub3 -
The Cochrane Database of Systematic... Oct 2008POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan... (Review)
Review
BACKGROUND
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement and monoclonal plasma cell-proliferative disorder. The pathogenesis of POEMS syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably secreted by plasmacytomas, is likely to be responsible for most of the characteristic symptoms. POEMS syndrome is a potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy, massive pleural effusion or ascites, or thromboembolic events. There is a need for efficacious therapy to improve prognosis.
OBJECTIVES
To provide the best available evidence from randomised controlled trials on treatment for POEMS syndrome.
SEARCH STRATEGY
We searched the Cochrane Neuromuscular Disease Group Trials Register (March 2008), MEDLINE (from January 1966 to March 2008), EMBASE (from January 1980 to March 2008) and CINAHL (from January 1982 to March 2008) for randomized controlled trials, quasi-randomized trials, historically controlled studies, and trials with concurrent controls. We adapted this strategy to search MEDLINE from 1966 and EMBASE from 1980 for comparative cohort studies, case-control studies and trials, and case series.
SELECTION CRITERIA
All randomized and quasi-randomized controlled trials, and non-randomized controlled studies were sought. Since we discovered no such clinical trials , we assessed and summarized all retrospective case series including five or more patients in the 'Discussion' section.
DATA COLLECTION AND ANALYSIS
Two authors independently reviewed and extracted details of all potentially relevant trials with any treatment for POEMS syndrome. We then collated and summarized information on the outcome.
MAIN RESULTS
We found no randomized or non-randomized prospective controlled trials of treatment for POEMS syndrome. We summarized the results of retrospective case series containing five or more patients in the Discussion section.
AUTHORS' CONCLUSIONS
There are no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome on which to base practice.
Topics: Humans; POEMS Syndrome
PubMed: 18843731
DOI: 10.1002/14651858.CD006828.pub2 -
AIDS Reviews 2008The objective of this study is to systematically review the epidemiology and the clinical and virologic aspects of multicentric Castleman's disease in HIV-positive... (Review)
Review
The objective of this study is to systematically review the epidemiology and the clinical and virologic aspects of multicentric Castleman's disease in HIV-positive patients and to evaluate treatment strategies and outcome, especially in relation to HAART administration. The authors have conducted a systematic review of the English literature for all cases of newly diagnosed multicentric Castleman's disease in HIV-positive patients. The 25 studies which met the selection criteria included 84 HIV-positive patients with multicentric Castleman's disease (20 pre-HAART and 64 post-HAART era). Of them, the majority (90%) were men with 33 months median time from detection of HIV-positivity to multicentric Castleman's disease diagnosis in the HAART era. Fever and lymphadenopathy were the most common presenting symptoms and cytopenias, hypoalbuminemia, polyclonal hypergammaglobulinemia and raised C-reactive protein the most frequently revealed laboratory findings. Kaposi's sarcoma was present in 72% of the patients and respiratory system involvement in 34%. Although the majority of cases reported were positive for human herpesvirus-8, none of the reviewed patients was found to suffer from polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Of the 48 patients on HAART, 64% were already on HAART at multicentric Castleman's disease diagnosis, having a better immunologic profile and a lower incidence of Kaposi's sarcoma than the 35% of patients who initiated HAART after multicentric Castleman's disease diagnosis. Nevertheless, the two groups did not have significantly different mortality rates (30 vs. 38%). At multicentric Castleman's disease diagnosis, a wide range of CD4 counts was recorded, suggesting that disease presentation could occur at any CD4 count. With regard to treatment, the study confirmed the high rates of response with rituximab (anti-CD20 monoclonal). Monochemotherapy seems to give short-lived responses, which require maintenance to be sustained. Polychemotherapy with CHOP has given long-term remission in a subset of patients. Other regimens used in the treatment of HIV-related multicentric Castleman's disease were antiviral agents, immunomodulatory agents, and thalidomide. The fatality rate among HIV-related multicentric Castleman's disease cases reviewed was 44%, significantly lower than that of HIV-negative individuals (65%), while median survival of the latter was 29 months longer than that of HIV-infected individuals. The fatality rate among pre-HAART patients was 75 vs. 29% among HAART patients. Infection, multiorgan failure, Kaposi's sarcoma, non-Hodgkin lymphoma and progressive multicentric Castleman's disease were the most often reported causes of death. In conclusion, multicentric Castleman's disease is a lymphoproliferative disorder with an increasing prevalence in HIV-infected individuals. Even though life expectancy in multicentric Castleman's disease seems to have significantly improved in the HAART era, it remains a disease with a poor prognosis and an increased incidence of non-Hodgkin lymphoma in the HIV-context.
Topics: Antineoplastic Agents; Antiretroviral Therapy, Highly Active; Castleman Disease; HIV Infections; Humans
PubMed: 18385778
DOI: No ID Found