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Discover Oncology Mar 2024Low serum cholesterol levels are associated with increased tumor morbidity and mortality. However, the relationship between serum lipid profile and POEMS syndrome...
Low serum cholesterol levels are associated with increased tumor morbidity and mortality. However, the relationship between serum lipid profile and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is still unclear. The aim of our study was to clarify the importance of the serum lipid profile in predicting the severity and prognosis of patients with POEMS syndrome. Forty-three patients with newly diagnosed POEMS syndrome admitted to the Department of Hematology of Jiangsu Provincial People's Hospital between August 2013 and February 2023 were selected. They had explicit serum lipid profiles. There were 27 males and 16 females with a median age of 54 years (range, 28-77 years). Survival curves were plotted using the Kaplan-Meier method, and comparisons between the two groups were performed using the log-rank test. The Cox proportional-hazards model examined risk factors associated with the prognosis of POEMS syndrome. Receiver-operator characteristic (ROC) curves assessed the predictive accuracy. 23 (53.5%) patients had low total cholesterol (TC) levels. Low levels of TC were concerned with unfavorable progression-free survival (PFS) (p = 0.007) and overall survival (OS) (p = 0.004), and at the same time, the low circulating TC concentration was an independent risk factor for PFS (p = 0.020) and OS (p = 0.011). Low TC values could improve the risk stratification, especially in high-risk patients. In conclusion, low serum TC levels may predict inferior prognosis in patients with POEMS syndrome; in future clinical application, low TC may be a reliable indicator of prognosis.
PubMed: 38436809
DOI: 10.1007/s12672-024-00912-6 -
Annals of Hematology Apr 2024
Topics: Humans; Extracellular Traps; POEMS Syndrome; Vasculitis, Leukocytoclastic, Cutaneous; Neutrophils
PubMed: 38436672
DOI: 10.1007/s00277-024-05651-w -
Rare : Open Research in Rare Diseases 2024POEMS Syndrome is a constellation of findings including Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Calciphylaxis, a...
POEMS Syndrome is a constellation of findings including Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Calciphylaxis, a microangiopathy involving vascular calcification and thrombotic occlusions, occurs rarely in POEMS. We present a case of prominent calciphylaxis that antedated the diagnosis of POEMS. The patient presented with extensive ecchymoses progressing to necrotic lesions in the setting of acute renal injury. Previously, she had chronic slowly progressive polyneuropathy, splenomegaly, hypothyroidism, amenorrhea, and ascites. Calciphylaxis was diagnosed on skin biopsy, and POEMS was diagnosed based upon clinical findings plus a bone marrow biopsy showing 15% lambda chain restricted plasma cells. Treatment for the calciphylaxis was supportive with fluids, tissue debridement, wound vacuum devices and antibiotics for secondary infection. Myeloma was treated with bortezomib and steroids. All aspects of the patient's manifestations improved. We conclude that calciphylaxis can be a prominent feature of POEMS and can appear prior to recognition of the full-blown syndrome.
PubMed: 38435320
DOI: 10.1016/j.rare.2024.100019 -
Medicine Mar 2024This article presents the case of a patient with recurrent chronic diarrhea and cachexia who was misdiagnosed, followed by a literature review to summarize the reasons... (Review)
Review
RATIONALE
This article presents the case of a patient with recurrent chronic diarrhea and cachexia who was misdiagnosed, followed by a literature review to summarize the reasons for misdiagnosis of POEMS syndrome and the treatment strategies.
PATIENT CONCERNS
The diagnosis and treatment of this patient suggest that with the improvement of M-protein detection levels, the diagnosis of patients with low M-protein levels, such as those with POEMS syndrome, has been greatly aided.
DIAGNOSES
POEMS syndrome requires polyneuropathy and monoclonal plasma cell proliferation as mandatory diagnostic criteria. Therefore, patients presenting with polyneuropathy should routinely undergo M-protein testing and consider the possibility of POEMS syndrome.
INTERVENTIONS
The patient, in this case, was treated primarily with relatively conservative immunomodulatory agents.
OUTCOMES
During follow-up after treatment, the patient's diarrhea and malnutrition showed significant improvement.
LESSONS SUBSECTIONS
POEMS syndrome has low clinical specificity and a high rate of misdiagnosis. However, once a definitive diagnosis is made, the treatment outcome is favorable.
Topics: Humans; POEMS Syndrome; Treatment Outcome; Diagnostic Errors; Diarrhea
PubMed: 38428848
DOI: 10.1097/MD.0000000000037200 -
Indian Journal of Nuclear Medicine :... 2023Polyneuropathy, organomegaly, endocrinopathy, M protein elevation, and skin changes (POEMS) syndrome is one of the rare paraneoplastic disorders of the multiorgan...
Polyneuropathy, organomegaly, endocrinopathy, M protein elevation, and skin changes (POEMS) syndrome is one of the rare paraneoplastic disorders of the multiorgan association whose initialism describes the principal clinical features as polyneuropathy, organomegaly, endocrinopathy, M-protein elevation, and skin changes caused by an underlying plasma cell disorder, one of the major diagnostic criteria for which is the presence of an osseous lesion. We present a case of POEMS syndrome with nearly all signs and symptoms on clinical and hematological examinations, which was further investigated with MRI of the whole spine and 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan, as the latter is a very useful imaging modality for evaluation of any paraneoplastic syndromes, including myeloproliferative disorders such as plasmacytoma or multiple myeloma, and lymphoproliferative or other malignancies.
PubMed: 38390530
DOI: 10.4103/ijnm.ijnm_44_23 -
Frontiers in Neurology 2023We present the case of a 54-year-old woman with reasonable blood sugar control who presented with a diabetic foot combined with severe peripheral neuropathy and vascular...
We present the case of a 54-year-old woman with reasonable blood sugar control who presented with a diabetic foot combined with severe peripheral neuropathy and vascular disease. Lower limb muscle weakness, muscle atrophy, skin pigmentation, and emaciation were also observed. Although her muscle strength improved after glucocorticoid treatment, it remained challenging to account for the other symptoms in this particular patient with chronic inflammatory demyelinating polyneuropathy. Plump liver and spleen, hidden bone lesions combined with seemingly unexplained cerebral infarction, and serous effusion led us to suspect polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. The abnormal proliferation of monoclonal plasma cells and a significant increase in vascular endothelial growth factor (VEGF) levels confirmed the diagnosis of POEMS syndrome. After 1 month of treatment with lenalidomide and dexamethasone, the diabetic foot ulcers healed, and the symptoms of myasthenia and fatigue improved. Diabetic feet may represent only the tip of the iceberg of an underlying POEMS syndrome. Our report aimed to increase awareness of this rare yet significant situation, advocating for the prompt identification and treatment of POEMS syndrome.
PubMed: 38344111
DOI: 10.3389/fneur.2023.1336382 -
World Journal of Clinical Cases Jan 2024Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that encompass multiple systems. The most common...
BACKGROUND
Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that encompass multiple systems. The most common clinical symptoms of POEMS syndrome are progressive sensorimotor polyneuropathy, organ enlargement, endocrine disorders, darkening skin, a monoclonal plasma cell proliferative disorder, and lymph node hyperplasia. The organomegaly consists of hepatosplenomegaly and/or lymphadenopathy; cases of cardiomyopathy are rare. Diagnoses are often delayed because of the atypical nature of the syndrome, exposing patients to possibly severe disability. Therefore, identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.
CASE SUMMARY
Herein, we report the case of a 59-year-old woman with POEMS syndrome that involved dilated cardiomyopathy. The patient presented to the hospital with complaints of shortness of breath and discomfort in the chest. The patient reported previous experiences of limb numbness. During hospitalization, the brain natriuretic peptide levels were 3504.0 pg/mL. Color doppler echocardiography showed an enlarged left side of the heart, along with ventricular wall hypokinesis and compromised functioning of the same side of the heart. Abdominal color ultrasonography revealed that the patient's spleen was enlarged. Observations from cardiac magnetic resonance imaging showed that the left side of the heart was enlarged. Slight myocardical fibrosis was also observed. Electromyography was described as a symmetric sensorimotor demyelinating polyneuropathy. Further immunoelectrophoresis of the serum showed the presence of a monoclonal IGA λ M protein. The vascular endothelial growth factor levels were 622.56 pg/mL. Flow cytometric and immunohistochemical staining of the bone marrow detected no monoclonal plasma cells. Finally, the patient was diagnosed with POEMS syndrome associated with dilated cardiomyopathy. The chest-related discomfort and the shortness of breath resolved after the administration of lenalidomide and dexamethasone.
CONCLUSION
When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin, the POEMS syndrome is the most possible diagnosis.
PubMed: 38322456
DOI: 10.12998/wjcc.v12.i3.601 -
Therapeutic Advances in Neurological... 2024Due to the rarity of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, the best first-line treatment has not been...
BACKGROUND
Due to the rarity of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, the best first-line treatment has not been established, although there are several options in guidelines. The preferred treatments vary according to the preference of the physician and anecdote.
OBJECTIVES
First, to analyze the efficacy of a new treatment mode in POEMS syndrome that uses the four-cycle treatment as the induction regimen, followed by sequential transplantation as the consolidation regimen for transplantation-eligible patients, or received another two-cycle treatment for transplantation-ineligible patients. Second, to compare the efficacy and safety of regimens with a proteasome inhibitor (bortezomib-cyclophosphamide-dexamethasone, BCD) or without a proteasome inhibitor (cyclophosphamide-dexamethasone ± thalidomide, CD ± T).
DESIGN
We conducted a retrospective study using real-world data from Capital Medical University, Xuanwu Hospital.
METHODS
A total of 34 newly diagnosed POEMS syndrome patients met Dispenzieri's diagnostic criteria, and those who completed at least four cycles of treatment from July 2013 to March 2021 were included.
RESULTS
The overall vascular endothelial growth factor (VEGF) response rate of this new treatment mode was 100%. The cumulative VEGF complete remission (CR) rate was 67.9%, and the cumulative complete hematological response (CR) rate was 55.6%. During the median 49-month follow-up, the 5-year-overall survival (OS) rate was 90.7%, the 3-year-progression-free survival (PFS) rate was 78.4%, and the 5-year-PFS rate was 73.8%. The BCD regimen achieved a 75% CR rate (median time from diagnosis to CR = 130 days) and 66.7% CR rate (median time from diagnosis to CR = 218 days). In addition, the VEGF response was less than the partial remission (PR) after four-cycle induction treatment, which, together with a decrease on the Overall Neurological Limitation Scale of less than three points 1 year after consolidation treatment, was an independent poor prognostic factor.
CONCLUSION
Bortezomib was well-tolerated by patients with POEMS syndrome. Compared with CD ± T regimen, BCD as the induction regimen achieved better VEGF response and earlier hematological remission. Autologous stem cell transplantation used as consolidation therapy further improved the neurological and hematological remission rates, resulting in better OS and PFS.
PubMed: 38288324
DOI: 10.1177/17562864231219151 -
Hepatology Forum 2024We present a case of POEMS syndrome from Turkiye, a rare, multisystemic condition resulting from plasma cell dyscrasia. POEMS is an acronym representing its cardinal...
We present a case of POEMS syndrome from Turkiye, a rare, multisystemic condition resulting from plasma cell dyscrasia. POEMS is an acronym representing its cardinal features: Peripheral neuropathy; Organomegaly; Endocrinopathy; Monoclonal plasma-cell proliferative disorder; and Skin changes. The syndrome has an estimated prevalence of 0.3 per 100,000 individuals and typically manifests in the fifth or sixth decade of life. Progressive peripheral neuropathy is the syndrome's most prominent symptom. To ensure an accurate diagnosis, a thorough medical history, physical examination, and comprehensive diagnostic evaluations are essential. These evaluations should include serum immunoelectrophoresis, serum cytokines, and growth factors, a skeletal survey, and a bone marrow biopsy. Early recognition and treatment of POEMS syndrome are crucial to prevent debilitating progression and to optimize clinical outcomes.
PubMed: 38283273
DOI: 10.14744/hf.2022.2022.0035 -
Journal of Korean Medical Science Jan 2024POEMS syndrome is a rare form of plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. Owing to its...
BACKGROUND
POEMS syndrome is a rare form of plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. Owing to its low incidence, there are few reports regarding this syndrome. This multicenter study included 84 patients diagnosed with POEMS syndrome in South Korea.
METHODS
We retrospectively evaluated 84 patients diagnosed with POEMS syndrome at 8 hospitals in South Korea between January 2000 and October 2022. The clinical characteristics and treatment outcomes were analyzed.
RESULTS
The median patient age was 53 years (range, 26-77 years), and 63.1% of the patients were male. All patients had peripheral neuropathy, and 81 (96.4%) had monoclonal plasma cell proliferation. Plasma vascular endothelial growth factor levels were available for 32 patients with a median of 821 pg/mL (range, 26-12,900 pg/mL). Other common features included skin changes (54.2%), volume overload (71.4%), and organomegaly (72.6%). Of the 84 patients, 75 received initial treatment (local radiotherapy, 6 [8.0%]; chemotherapy, 17 [22.7%]; both chemotherapy and local radiotherapy, 9 [12.0%]), upfront autologous stem cell transplantation (ASCT), 43 (57.3%; with induction chemotherapy, n = 12, 16.0%; without induction chemotherapy, n = 31, 41.3%). The median follow-up duration was 40.7 months. The 5-year overall survival (OS) was 78%, and the 5-year progression-free survival (PFS) was 55%. Patients who underwent upfront ASCT and were diagnosed after 2014 had a longer OS and PFS.
CONCLUSION
The demographics of Korean patients with POEMS syndrome were similar to those reported previously. Because of the introduction of new treatment agents and the reduced rate of transplant-related mortality related to ASCT, the treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.
Topics: Humans; Male; Adult; Middle Aged; Aged; Female; POEMS Syndrome; Hematopoietic Stem Cell Transplantation; Vascular Endothelial Growth Factor A; Retrospective Studies; Transplantation, Autologous; Republic of Korea
PubMed: 38225781
DOI: 10.3346/jkms.2024.39.e5