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Annals of Laboratory Medicine Nov 2019POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC)...
POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.
Topics: Adult; Aged; Aged, 80 and over; Antigens, CD19; Bone Marrow; Female; Glycoproteins; Humans; Immunophenotyping; Lymphocytes; Male; Middle Aged; POEMS Syndrome; Paraproteinemias; Republic of Korea; Retrospective Studies; Young Adult
PubMed: 31240884
DOI: 10.3343/alm.2019.39.6.561 -
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 -
Clinical Neurophysiology Practice 2020We detailed the electrophysiological patterns of peripheral nerve temporal dispersion across spectrum of POEMS syndrome and Castleman disease (CD).
OBJECTIVE
We detailed the electrophysiological patterns of peripheral nerve temporal dispersion across spectrum of POEMS syndrome and Castleman disease (CD).
METHODS
Compound muscle action potentials (CMAP) duration of 3 patients with POEMS syndrome and 2 with hyaline vascular type CD without clonal plasma cell dyscrasia were retrospectively analysed.
RESULTS
Median and ulnar nerves distal CMAP duration were prolonged in all patients irrespective of plasma cell dyscrasia or M protein. All lower limbs distal CMAP responses were absent. Greatest distal CMAP duration prolongation was observed in median nerves for POEMS syndrome (17.0 ms, 158% upper limit normal) and in ulnar nerves for CD (9.8 ms, 47% upper limit normal). Distal/proximal CMAP duration ratio of <0.7 were seen in 33% of median and ulnar nerves studied among POEMS syndrome. Among nerves with ratio >0.7, all had distal CMAP duration prolongation (Range 7%-158% of upper limit normal).
CONCLUSIONS
Abnormal distal CMAP dispersion is not uncommon in POEMS syndrome and CD without clonal plasma cell dyscrasia or M protein. POEMS syndrome has greater distal CMAP duration in median and ulnar nerves, particularly in median nerve that can reach up to 150% of upper limit normal, compared to <50% in CD.
SIGNIFICANCE
Detailed electrophysiological analysis of distal CMAP duration may help in distinguishing POEMS syndrome and CD.
PubMed: 32613149
DOI: 10.1016/j.cnp.2020.05.001 -
American Journal of Hematology Feb 2022Thromboses are prevalent in POEMS syndrome, but few risk factors for POEMS-associated thrombosis have been identified. The objective of this study is to identify novel...
Thromboses are prevalent in POEMS syndrome, but few risk factors for POEMS-associated thrombosis have been identified. The objective of this study is to identify novel risk factors for POEMS-associated thrombosis. In this retrospective cohort of 230 POEMS patients, 27% developed thrombosis. Arterial events were slightly more common than venous. Stroke accounted for 26% of all thromboses and 53% of arterial events. There were differences in baseline features between the thrombosis group and the no thrombosis group, and these were driven by patients with arterial thrombosis. Risk factors for arterial thrombosis included thrombocytosis, elevated hemoglobin/hematocrit, extravascular volume overload, and splenomegaly. Hyperprolactinemia appeared to be a risk factor for venous thrombosis. The risk of thrombosis was most striking among men with elevated hemoglobin (32% vs. 5%, p < .001) and hematocrit (42% vs. 5%, p < .001) compared to men without. Most thromboses occurred prior to POEMS directed therapy, and most that occurred during therapy happened within 3 months of diagnosis. Twenty-one percent of patients with thrombosis had recurrence. In recognition of high overall rates of thrombosis in this population, all patients with POEMS syndrome should receive prophylactic antiplatelet therapy, and clinicians should consider anticoagulation in patients with risk factors for POEMS-associated thrombosis.
Topics: Adult; Female; Humans; Male; Middle Aged; POEMS Syndrome; Retrospective Studies; Risk Factors; Thrombosis
PubMed: 34822175
DOI: 10.1002/ajh.26422 -
International Journal of Clinical and... 2020Lymphadenopathy is an important characteristic of POEMS syndrome, and a Castleman disease (CD)-like pathologic change in the lymph nodes is one of the major diagnostic...
Lymphadenopathy is an important characteristic of POEMS syndrome, and a Castleman disease (CD)-like pathologic change in the lymph nodes is one of the major diagnostic criteria. However, the characteristics of lymphadenopathy in POEMS still have not been completely elucidated. The lymph node biopsies are available only for a small proportion of patients. A simple and safe way is needed to rule CD in or out. This study aimed to analyse the features of lymphadenopathy and estimate the role of imaging methods, including computed tomography (CT) and positron emission tomography-CT (PET/CT), in the diagnosis of lymphadenopathy in patients with POEMS syndrome. We conducted a retrospective analysis of 23 patients with confirmed POEMS syndrome. All of the patients received chest and abdominal CT scan and/or superficial ultrasound examinations. Four patients underwent PET/CT examinations, and 6 patients received lymph node biopsies. Enlarged lymph nodes (short diameter ≥ 1 cm) were found in 48% (11/23) of patients, but only 1 patient had an enlarged lymph node with a diameter ≥ 2 cm. Lymph nodes with CD-like pathologic changes from 2 patients showed increased maximum standard uptake values (SUV) of F-deoxyglucose (FDG) on PET/CT, while lymph nodes with reactive pathologic changes from 2 other patients showed a normal metabolic PET/CT profile. The extent of lymph node enlargement in patients with POEMS was less than that in patients with CD . We draw the conclusion that most of the enlarged lymph nodes had diameters ≤ 2 cm, which is less than that in cases of CD and PET/CT may be helpful in determining whether enlarged lymph nodes are characterized by CD-like changes or not.
PubMed: 32055268
DOI: No ID Found -
European Journal of Ophthalmology Sep 2023Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal... (Review)
Review
Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal antibody. These disorders are associated with various systemic findings, including ophthalmological disorders. A search of PubMed, EMBASE, Scopus and Cochrane databases was performed in March 2021 to examine evidence pertaining to ocular complications in patients diagnosed with plasma cell dyscrasias. This review outlines the ocular complications associated with smoldering multiple myeloma and monoclonal gammopathy of undetermined significance, plasmacytomas, multiple myeloma, Waldenström's macroglobulinemia, systemic amyloidosis, Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes (POEMS) syndrome, and cryoglobulinemia. Although, the pathological mechanisms are not completely elucidated yet, wide-ranging ocular presentations have been identified over the years, evolving both the anterior and posterior segments of the eye. Moreover, the presenting symptoms also help in early diagnosis in asymptomatic patients. Therefore, it is imperative for the treating ophthalmologist and oncologist to maintain a high clinical suspicion for identifying the ophthalmological signs and diagnosing the underlying disease, preventing its progression through efficacious treatment strategies.
Topics: Humans; Paraproteinemias; Eye; Eye Diseases; Treatment Outcome
PubMed: 36760117
DOI: 10.1177/11206721231155974 -
Internal Medicine (Tokyo, Japan) Jul 2022Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes (POEMS) syndrome manifests as elevated levels of vascular endothelial growth factor (VEGF) and...
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes (POEMS) syndrome manifests as elevated levels of vascular endothelial growth factor (VEGF) and monoclonal gammopathy. We treated a case of POEMS syndrome showing monoclonality in both IgA-λ and IgG-κ. Serial renal biopsies before treatment and after normalization of the VEGF levels suggested that glomerular microangiopathy had developed due to VEGF, while biclonal gammopathy was not eliminated. The renal pathology, proteinuria, and renal function all clearly improved. Although severe polyneuropathy limited activities of daily living and enforced a bedridden state, the patient dramatically regained his motor function, achieving crutch walking after induction of remission. This case is highly notable due to the presence of biclonality and repeated biopsies.
Topics: Activities of Daily Living; Humans; Monoclonal Gammopathy of Undetermined Significance; POEMS Syndrome; Paraproteinemias; Vascular Endothelial Growth Factor A
PubMed: 35283379
DOI: 10.2169/internalmedicine.8714-21 -
Internal Medicine (Tokyo, Japan) May 2021TAFRO syndrome and POEMS syndrome are lymphoproliferative disorders with elevated interleukin-6 and vascular endothelial growth factor (VEGF) levels; however, their...
TAFRO syndrome and POEMS syndrome are lymphoproliferative disorders with elevated interleukin-6 and vascular endothelial growth factor (VEGF) levels; however, their underlying pathogenic mechanisms remain unclear. Similarities have been reported in the pathological findings of the lymph nodes of TAFRO syndrome, Multicentric Castleman disease (MCD), and some cases of POEMS syndrome. However, there is no consensus on the relationship among them. We encountered a case of lymphoproliferative disorder that presented with manifestations of both TAFRO syndrome and POEMS syndrome. This case may be a subtype of idiopathic MCD and will be very important for establishing the disease concept of TAFRO syndrome and POEMS syndrome.
Topics: Castleman Disease; Humans; Lymph Nodes; POEMS Syndrome; Vascular Endothelial Growth Factor A
PubMed: 33328401
DOI: 10.2169/internalmedicine.5860-20 -
Oncology (Williston Park, N.Y.) Dec 2013POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. Its main features include polyradiculoneuropathy, organomegaly,... (Review)
Review
POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. Its main features include polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Other important characteristics of POEMS include papilledema, extravascular volume overload, sclerotic bone lesions, and thrombocytosis/erythrocytosis (PEST). Vascular endothelial growth factor (VEGF) appears to play an important role in the disease and is especially useful for monitoring therapy, but it is not likely the sole factor driving the disease. The most commonly used therapies for POEMS include alkylators and steroids, high-dose chemotherapy with peripheral blood stem cell transplantation, lenalidomide, and bortezomib. The role of anti-VEGF antibodies is uncertain. In general, patients have an excellent prognosis if the diagnosis is made early and appropriate therapy is applied.
Topics: Humans; POEMS Syndrome
PubMed: 24624542
DOI: No ID Found -
The Journal of the American College of... Dec 2010Human calciphylaxis reflects a form of severe tissue compromise attributable to a unique microangiopathy that combines features of vascular thrombotic occlusion with... (Review)
Review
Human calciphylaxis reflects a form of severe tissue compromise attributable to a unique microangiopathy that combines features of vascular thrombotic occlusion with endoluminal calcification. While most frequently described in patients with renal failure, it is seen in other settings, such as multiple myeloma; polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome; cirrhosis; and rheumatoid arthritis. Although most commonly involving the skin, calciphylaxis can affect other organs including the heart and gastrointestinal tract, in which cases it falls under the appellation of systemic calciphylaxis. There are cases in which the main pathology is one of endovascular thrombosis of the vessels of the fat without discernible calcification or one manifesting a pseudoangiosarcomatous pattern, hence adding to the histomorphologic spectrum of calciphylaxis. A variety of factors contribute to this severe occlusive microangiopathy, including an underlying procoagulant state and ectopic neo-osteogenesis of the microvasculature through varied mechanisms, including increased osteopontin production by vascular smooth muscle or reduced synthesis of fetuin and GLA matrix protein, important inhibitors of ectopic neo-osteogenesis. Certain factors adversely affect outcome, including truncal and genital involvement and systemic forms of calciphylaxis. With a better understanding of its pathophysiology, more-effective therapies, such as sodium thiosulfate and biphosphanates to reduce reactive oxygen species and receptor activator of nuclear factor κβ-mediated nuclear factor κβ activity, respectively, are being developed.
PubMed: 24527153
DOI: 10.1016/j.jcws.2011.03.001