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Indian Journal of Pathology &... 2023POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical...
POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical examination, and appropriate testing can aid in establishing its diagnosis. We are presenting the case of a 75-year-old man who was diagnosed with POEMS syndrome.
Topics: Male; Humans; Aged; POEMS Syndrome; Paraproteinemias
PubMed: 37530355
DOI: 10.4103/ijpm.ijpm_857_21 -
Scientific Reports Jun 2023A higher serum vascular endothelial growth factor (VEGF) level can cause choroidal thickening in the choroid of patients with polyneuropathy, organomegaly,...
A higher serum vascular endothelial growth factor (VEGF) level can cause choroidal thickening in the choroid of patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. We aimed to determine whether fluctuations in serum VEGF levels affect choroidal vascular structures in patients with POEMS syndrome. This retrospective observational case series examined 17 left eyes of 17 patients with POEMS syndrome. Enhanced depth imaging optical coherence tomography (EDI-OCT) images were obtained, and serum VEGF levels were measured at baseline and 6 months after transplantation with dexamethasone (n = 6), thalidomide (n = 8), or lenalidomide (n = 3). EDI-OCT images were binarized using ImageJ software, and we calculated the areas of the whole choroid and the luminal and stromal areas. Subsequently, we determined whether the choroidal vascular structure had changed significantly between baseline and 6 months after treatment. Six months after treatment, serum VEGF levels and the whole choroid, luminal, and stromal areas had decreased significantly compared to the baseline values (all, P < 0.001). The mean luminal area to the whole choroidal area ratio at 6 months after treatment was 0.70 ± 0.03, which was significantly smaller than the ratio at baseline (0.72 ± 0.03; P < 0.001). Whole choroid and luminal area fluctuations were significantly positively correlated with fluctuations in serum VEGF levels (r = 0.626, P = 0.007 and r = 0.585, P = 0.014, respectively). Choroidal thickening induced by VEGF might be caused by increases in the choroidal vessel lumen area. These results may offer insights into the pathogenesis of POEMS syndrome and the role of serum VEGF in choroidal vascular structure, which may apply to other ocular diseases.
Topics: Humans; POEMS Syndrome; Vascular Endothelial Growth Factor A; Retrospective Studies; Choroid; Monoclonal Gammopathy of Undetermined Significance
PubMed: 37391497
DOI: 10.1038/s41598-023-37727-4 -
Asian Journal of Surgery Nov 2023
Topics: Humans; POEMS Syndrome; Hypesthesia; Lower Extremity
PubMed: 37308387
DOI: 10.1016/j.asjsur.2023.05.161 -
Blood Cancer Journal May 2023
Topics: Humans; Antineoplastic Combined Chemotherapy Protocols; Multiple Myeloma; POEMS Syndrome; Thalidomide
PubMed: 37253713
DOI: 10.1038/s41408-023-00859-x -
EJHaem May 2023
PubMed: 37206260
DOI: 10.1002/jha2.656 -
International Journal of Molecular... Apr 2023Castleman disease (CD) is a rare lymphoproliferative disorder that includes various clinico-pathological subtypes. According to clinical course, CD is divided into...
Castleman disease (CD) is a rare lymphoproliferative disorder that includes various clinico-pathological subtypes. According to clinical course, CD is divided into unicentric CD (UCD) and multicentric CD (MCD). MCD is further distinguished based on the etiological driver in herpes virus-8-related MCD (that can occur in the setting of HIV); in MCD associated with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes); and idiopathic MCD (iMCD). The latter can also be divided in iMCD-TAFRO (thrombocytopenia, anasarca, fever, myelofibrosis, organomegaly) and iMCD not otherwise specified. To date, CD pathogenesis is still uncertain, but CD may represent the histological and clinical result of heterogeneous pathomechanisms. Transcriptome investigations in CD lymph nodes have documented the expression and up-regulation of different cytokines; furthermore, few recent studies have shown alterations of different T-cell subsets in CD patients, suggesting a possible role of the nodal microenvironment in CD development. On this basis, our study aimed to investigate the distribution of T-cell subsets in the clinico-pathological spectrum of CD. We evaluated the CD4/CD8 ratio and the number of T-regulatory (T-reg) FOXP3+ cells in 28 CD cases. In total, 32% of cases showed a decreased CD4/CD8 ratio due to increased CD8+ T-cells, including both UCD, iMCD, and HHV8+ MCD cases. The T-reg subset analysis revealed a statistically significant ( < 0.0001) lower mean number of FOXP3+ T-reg cells in CD cases when compared with non-specific reactive lymph nodes. We did not find statistically significant differences in T-reg numbers between the different CD subtypes. These findings may suggest that alterations in T-cell subpopulations that can lead to disruption of immune system control may contribute to the numerous changes in different cellular compartments that characterize CD.
Topics: Humans; Castleman Disease; Lymph Nodes; Antibodies, Monoclonal; T-Lymphocyte Subsets; Herpesvirus 8, Human; Forkhead Transcription Factors
PubMed: 37175521
DOI: 10.3390/ijms24097813 -
BMJ Open Diabetes Research & Care Apr 2023Our aim was to determine whether there are risk factors which increase the risk of developing dysglycemia in a child who has increased body mass index (BMI)...
INTRODUCTION
Our aim was to determine whether there are risk factors which increase the risk of developing dysglycemia in a child who has increased body mass index (BMI) (overweight/obese).
RESEARCH DESIGN AND METHODS
This was a retrospective cohort study of 715 children who had increased BMI (overweight/obese). They presented to tertiary care at KK Women's and Children's Hospital, Singapore, for metabolic risk assessment. Subjects who had more than one oral glucose tolerance test were included in order to track and analyze risk factors associated with worsening glycemic status from a previously normal glucose tolerance, impaired fasting glucose, or impaired glucose tolerance (IGT) state. Demographic characteristics, birth history, family history of metabolic syndrome, metabolic comorbidities, and interventions received were recorded. Statistical analysis was performed to determine odds ratio (OR) of worsening glycemic status progression in association with an analyzed variable, adjusted for intervention received.
RESULTS
Risk factors of developing dysglycemia can be present right from birth, as participants who were born preterm had increased odds of IGT (OR: 3.49 (1.10 to 11.03)), and a greater proportion of large-for-gestational-age (LGA)/small-for-gestational-age (SGA) babies had dysglycemia (SGA-IGT: 8.8%, SGA-diabetes mellitus (DM): 5.9%, LGA-IGT: 10.6%, LGA-DM: 11.8%) even at baseline. Being born preterm (OR: 3.49 (1.10 to 11.03)), with comorbidities of hypertension (OR: 1.61 (1.01 to 2.57)), hyperlipidemia (OR: 1.80 (1.19 to 2.72)), and fatty liver disease (OR: 2.08 (1.39 to 3.13)), was significantly associated with an increased OR of developing IGT. Risk factors for developing a worsening glycemic status, either to IGT or DM, included age >10 years (OR 4.94 (1.21 to 20.25)), BMI rise (OR 1.71 (1.17 to 2.49)), BMI increase >1.08 kg/m (OR 1.71 (1.16 to 2.51)), comorbidities of hyperlipidemia (OR 1.67 (1.12 to 2.50)), and fatty liver disease (OR 2.11 (1.43 to 3.12)).
CONCLUSIONS
A child who has increased BMI (overweight/obese) and possesses risk factors for worsening glycemic status, if intervened with routine lifestyle modification advice, may still have increased risk of developing dysglycemia and type 2 DM. Therefore, understanding their risk profile provides opportunities to have a tiered and individualized approach.
Topics: Child; Infant, Newborn; Humans; Female; Overweight; Pediatric Obesity; Retrospective Studies; Metabolic Syndrome; Weight Gain; Glucose Intolerance; Liver Diseases
PubMed: 37076191
DOI: 10.1136/bmjdrc-2022-003228 -
Cancer Reports (Hoboken, N.J.) May 2023Monoclonal gammopathies are a group of disorders associated with clonal proliferation of plasma cells that produces a monoclonal protein.
BACKGROUND
Monoclonal gammopathies are a group of disorders associated with clonal proliferation of plasma cells that produces a monoclonal protein.
AIMS
The main objective of this study was to describe the epidemiological and immunochemical characteristics of monoclonal gammopathies diagnosed during 19 years in a Moroccan teaching hospital.
MATERIALS AND RESULTS
This retrospective study enrolled 443 Moroccan patients with monoclonal gammopathy, patients meeting the inclusion and exclusion criteria in at the biochemistry department of Military Hospital in Rabat, the capital of Morocco, from January 2000 to August 2019. Of the 443 enrolled patients, 320 (72.23%) were men and 123 (27.77%) were women. All patients were of Caucasian origin, from 12 Moroccan regions. The patient's samples were collected and subjected to serum protein electrophoresis and serum immunofixation electrophoresis to further characterize the monoclonal protein. The mean ± SD age of the 443 participants was 62.24 ± 13.14 years. Reasons for being admitted to the hospital were as follows, bone pain (41.60%), renal failure (19.08%), alteration of the general condition (12.21%), and anemia (10.69). Plasma cell proliferative disorders in our study were as follows, multiple myeloma (MM) (45.65%), Monoclonal gammopathies of undetermined significance (MGUS) (39.05%), Waldenstrom's macroglobulinemia (5.58%), Lymphoma (2.27% + 1.2%), Chronic Lymphocytic Leukemia (2.48%), Plasma cell leukemia (1.86%), Plasmacytoma (0.62%), POEMS syndrome (0.41%), and Amyloidosis (0.84%). The most frequent isotypes in MM were the IgGκ (62) 36.5%, IgGλ (52) 30.6%, IgAκ (27) 15.9%, and the IgAλ (19) 11.2%. It is also worth noting that Free light chain MM represents 20% of all cases of MM.
CONCLUSIONS
We found that monoclonal gammopathies are age-related and affects men more than women, also the results of this study point to the delayed diagnosis of monoclonal gammopathies, since most of our patients were diagnosed at the MM stage. The most frequent isotypes were the IgGκ and IgGλ in MM and MGUS, in Waldenström macroglobulinemia were IgMκ and IgMλ and the oligoclonal profile represented only 3.70%.
Topics: Male; Humans; Female; Middle Aged; Aged; Morocco; Retrospective Studies; Paraproteinemias; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Waldenstrom Macroglobulinemia; Hospitals
PubMed: 36999311
DOI: 10.1002/cnr2.1814 -
Nagoya Journal of Medical Science Feb 2023POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystem disease characterized by plasma cell... (Review)
Review
Surgical revascularization for quasi-moyamoya disease associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome: a case report and literature review.
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystem disease characterized by plasma cell dyscrasia and overproduction of vascular endothelial growth factor, which is related to disease activity. Recent treatment strategies have improved survival of patients suffering from this disorder; however, ischemic stroke remains a poor prognostic factor. POEMS patients with ischemic stroke frequently develop cerebral large artery stenosis/occlusion, followed by progressive stroke. Post literature review, we present an ischemic stroke case of quasi-moyamoya disease linked with this syndrome that was successfully treated with surgical revascularization. A 41-year-old woman diagnosed with POEMS syndrome developed progressive ischemic stroke due to quasi-moyamoya disease, despite decreased vascular endothelial growth factor level with lenalidomide and dexamethasone treatment. She underwent superficial temporal artery to middle cerebral artery bypass with encephalo-duro-myo-synangiosis bilaterally. The postoperative course was uneventful. Two years and five months after the stroke, neuroimaging demonstrated bypass patency, neovascularization after encephalo-duro-myo-synangiosis, and no recurrence of stroke. Our case is the first to report successful surgical revascularization for a POEMS patient. Surgical revascularization may be a useful treatment option for patients with quasi-moyamoya disease associated with POEMS syndrome, especially for those who develop refractory ischemic stroke despite reduced vascular endothelial growth factor level.
Topics: Female; Humans; Adult; Moyamoya Disease; POEMS Syndrome; Vascular Endothelial Growth Factor A; Monoclonal Gammopathy of Undetermined Significance; Stroke; Ischemic Stroke
PubMed: 36923610
DOI: 10.18999/nagjms.85.1.141 -
Journal of the Peripheral Nervous... Jun 2023
PubMed: 36916657
DOI: 10.1111/jns.12542