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Technology in Cancer Research &... 2022Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare monoclonal plasma cell proliferation disorder. At present, there is no... (Meta-Analysis)
Meta-Analysis
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare monoclonal plasma cell proliferation disorder. At present, there is no unified treatment for POEMS syndrome. Here, we describe one case with POEMS syndrome with obvious neurological symptoms diagnosed in September 2020. We made a meta--analysis to assess the efficacy of treatment strategies in recent years. We retrospectively analyzed the diagnosis and treatment of this patient, and searched relevant articles in PubMed, Embase, and MEDLINE databases using MedicalSubject Headings (MeSH) (eg, POEMS, Therapy, Drug Therapy, Biological Therapy, Combined Modality Therapy, Hematopoietic Stem Cell Transplantation, Immunotherapy, Molecular Targeted Therapy, Chemoradiotherapy, and Salvage Therapy) and free words, and performed the statistical analysis. The patient's efficacy evaluation was complete response (CR) after treatment with ixazomib combined with autologous stem cell transplantation (ASCT). Overall, 20 articles consist of 6 clinical trials, 14 retrospective studies, and 936 patients were included in this meta-analysis. There was no significant difference in complete hematologic response (CR) rate between people who underwent ASCT and those who did not. However, ASCT might have a better survival rate. Ixazomib combined with ASCT therapy may be a safe and effective method for patients with POEMS syndrome.
Topics: Humans; Hematopoietic Stem Cell Transplantation; Retrospective Studies; POEMS Syndrome; Treatment Outcome; Transplantation, Autologous
PubMed: 36579839
DOI: 10.1177/15330338221123634 -
Heliyon Dec 2022POEMS syndrome is a plasma cell disease. Clinical manifestations and clinical onset are variable. In recent years, more and more cases of POEMS syndrome with...
POEMS syndrome is a plasma cell disease. Clinical manifestations and clinical onset are variable. In recent years, more and more cases of POEMS syndrome with cerebrovascular disease and ischemic stroke have been reported. However, it is rare for patients with POEMS syndrome to present with a cerebrovascular accident as the first clinical manifestation. We presented three cases of POEMS syndrome with cerebral infarction in different phases of the disease. We then searched the literature for studies involving POEMS syndrome complicated with cerebral infarction. There were 81 cases in total. In nine patients, cerebral infarctions occurred before polyneuropathy. Patients with cerebral infarction before polyneuropathy have better prognosis of POEMS than those with cerebral infarction after polyneuropathy.
PubMed: 36578403
DOI: 10.1016/j.heliyon.2022.e12201 -
Oxford Medical Case Reports Dec 2022Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) is a rare paraneoplastic syndrome, and its diagnosis is based on a series of...
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) is a rare paraneoplastic syndrome, and its diagnosis is based on a series of clinical and laboratory findings. We present the case of a 46-year-old woman who was previously diagnosed with essential thrombocythemia. The patient complained about dyspnea on exertion, nausea, burning of the lower limbs, weight loss, recurrent episodes of lower back pain and polymenorrhea. Physical examination revealed hyperpigmentation, livedo reticularis of the lower limbs, sclerodermoid changes and plectrodactyly. A computed tomography-guided bone biopsy revealed the presence of plasmacytoma, and based on a combination of clinical features such as polyneuropathy, a diagnosis of POEMS syndrome has been established. The diagnosis of POEMS syndrome demands a high index of suspicion, especially in cases of peripheral neuropathy, peripheral edema or organomegaly of unknown origin. Since the syndrome can be fatal, early diagnosis is pivotal for patients' survival and quality of life.
PubMed: 36540841
DOI: 10.1093/omcr/omac129 -
Cureus Nov 2022POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare disorder that can mimic chronic inflammatory...
POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Masquerading as Chronic Inflammatory Polyradiculoneuropathy: A Case Report.
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare disorder that can mimic chronic inflammatory demyelinating polyradiculopathy (CIDP). In this report, we present a case of a man with a new diagnosis of POEMS syndrome and a clinical picture of CIDP. He had prostate cancer (s/p prostatectomy) with known diffuse bony osteosclerotic lesions and a monoclonal gammopathy of undetermined significance (MGUS). The objective of this report is to highlight the importance of recognizing POEMS as a rare condition, differentiating it from CIDP, and initiating treatment as soon as possible. The diagnosis of POEMS can be delayed due to its extensive variety of clinical manifestations, and the extensive workup needed for the diagnosis.
PubMed: 36523729
DOI: 10.7759/cureus.31481 -
Journal of Clinical Medicine Nov 2022POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy,... (Review)
Review
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.
PubMed: 36498588
DOI: 10.3390/jcm11237011 -
Nigerian Journal of Clinical Practice Nov 2022Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare plasma cell dyscrasia disease involving multiple organs combined with... (Review)
Review
A POEMS syndrome patient with idiopathic non-cirrhotic portal hypertension received the transjugular intrahepatic portosystemic shunt: a case report and literature review.
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare plasma cell dyscrasia disease involving multiple organs combined with idiopathic non-cirrhotic portal hypertension. It has been reported only four times in the English literature. Here, we present the first case of a 62-year-old male POEMS syndrome patient with portal hypertension treated with the transjugular intrahepatic portosystemic shunt (TIPS), after he presented with a 10-day history of melena. The diagnosis of POEMS syndrome was given because the patient presented with polyneuropathy, monoclonal plasma cell proliferative disorder, sclerotic bone lesions, splenomegaly, lymphadenopathy, ascites, hypothyroidism, and hyperpigmentation. The presence of portal hypertension was confirmed by esophageal varices, congested and edematous stomach body, splenomegaly, and transudate ascites in which the serum-ascites albumin gradient of ascites fluid was over 11 g/L (a concentration considered to be associated with POEMS syndrome), as no other causes were found. The patient fasted and received conservative drug treatments on admission, but symptoms of melena soon recurred within 1 week after resuming his diet. After TIPS and venous embolization were performed, symptoms of bleeding were effectively controlled, while the patient subsequently developed hepatic encephalopathy, which ultimately led to death. The presence of gastrointestinal bleeding in POEMS syndrome with idiopathic non-cirrhotic portal hypertension indicates a poor prognosis. Given that this was the first patient to receive TIPS, and although the incidence of hepatic encephalopathy has increased, TIPS is still acceptable for refractory variceal bleeding.
Topics: Male; Humans; Middle Aged; Esophageal and Gastric Varices; Portasystemic Shunt, Transjugular Intrahepatic; Ascites; Hepatic Encephalopathy; POEMS Syndrome; Gastrointestinal Hemorrhage; Splenomegaly; Melena; Hypertension, Portal
PubMed: 36412305
DOI: 10.4103/njcp.njcp_360_22 -
Clinical Case Reports Nov 2022POEMS syndrome is a rare clonal plasma cell disease associated with multisystem involvement. We reported a case of 48-year-old woman with large volume of exudative...
POEMS syndrome is a rare clonal plasma cell disease associated with multisystem involvement. We reported a case of 48-year-old woman with large volume of exudative ascites with an increased level of λ-light chain and hepatosplenomegaly. The patient was treated with thalidomide and dexamethasone and showed a good clinical response.
PubMed: 36408092
DOI: 10.1002/ccr3.6603 -
Annals of Hematology Jan 2023
Topics: Humans; POEMS Syndrome; Multiple Myeloma; Plasma Cells; Castleman Disease; Mutation
PubMed: 36401620
DOI: 10.1007/s00277-022-05032-1 -
Journal of Postgraduate Medicine 2022Chronic diarrhea, by definition, is the passage of loose/liquid stools, with increased frequency (more than three times/day), or an output of over 200 g/day, lasting for...
Chronic diarrhea, by definition, is the passage of loose/liquid stools, with increased frequency (more than three times/day), or an output of over 200 g/day, lasting for a duration of four or more weeks. The clinical approach to identify the cause of chronic diarrhea generally depends on the local socioeconomic status. In high-income countries, systemic causes such as irritable bowel syndrome (IBS), inflammatory bowel disease, malabsorption syndromes (lactose intolerance/coeliac disease) are primarily considered. In mid- to low-income countries, infective causes like chronic bacterial, mycobacterial, fungal infections, HIV, bowel cancer are considered before systemic causes/malabsorption syndromes. Amyloidosis, more accurately, reactive amyloidosis is one of the rarer causes of chronic/persistent diarrhea. Inflammatory colitis secondary to POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) as a cause for chronic diarrhea has been reported only in a handful of cases and is often missed. We present such a case of chronic diarrhea in a middle-aged man, who was eventually diagnosed to have POEMS syndrome.
Topics: Middle Aged; Male; Humans; POEMS Syndrome; Diarrhea; Malabsorption Syndromes; Chronic Disease; Amyloidosis
PubMed: 36348608
DOI: 10.4103/jpgm.jpgm_1169_21 -
HemaSphere Nov 2022Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often... (Review)
Review
Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 12-16 months, by which time patients can be severely disabled. There are no established consensus guidelines. We provide clinicians a comprehensive blueprint for managing POEMS from diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse based on published evidence and our large single-center experience. A multidisciplinary approach is essential including expert hematologists, neurologists, histopathologists, radiologists, and neurophysiologists. The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. Response assessment is centered on clinical, neuropathy, hematological, vascular endothelial growth factor, and radiological criteria. Future clinical trials are welcomed in this setting where evidence is limited.
PubMed: 36340912
DOI: 10.1097/HS9.0000000000000796