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Diseases (Basel, Switzerland) Nov 2023POEMS syndrome-characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes-is an uncommon and complex paraneoplastic disorder encompassing...
Unraveling the Neurological Complexity of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome: A Report of a Challenging Case of a Young Woman and Cutting-Edge Advancements in the Field.
POEMS syndrome-characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes-is an uncommon and complex paraneoplastic disorder encompassing a diverse array of symptoms. Here we report the challenging case of a 34-year-old female who sought medical attention at the emergency department due to distal lower limb weakness. She was breastfeeding her first child at that time. Her condition rapidly deteriorated, making it difficult for her to perform simple tasks independently. Initially, she struggled with activities like jumping or climbing stairs. Eventually, her ability to walk was also compromised. These symptoms underscored the swift evolution of her polyneuropathy. Nerve conduction studies and electromyography confirmed a diagnosis of mixed demyelinating and axonal polyneuropathy. Subsequent investigations, including bone marrow biopsy and immunochemistry testing, revealed a plasma cell disorder characterized by lambda monoclonal gammopathy, along with elevated levels of vascular endothelial growth factor (VEGF > 8000 pg/mL). This pivotal finding led to the diagnosis of POEMS syndrome, prompting the initiation of antineoplastic therapy (daratumumab-lenalidomide-dexamethasone) to manage this condition. An autologous cell transplantation was planned. The rarity of POEMS syndrome and its diverse clinical manifestations often lead to an incorrect or delayed diagnosis. Our case underscores the importance of considering this syndrome in patients presenting with acute or subacute polyneuropathy, even if the patients are young. In conclusion, this case elucidates the diagnostic complexities of POEMS syndrome, emphasizing the integral role of comprehensive multidisciplinary evaluations and the potential influence of increased VEGF as a diagnostic key element and possible therapeutic target.
PubMed: 37987277
DOI: 10.3390/diseases11040167 -
The American Journal of Dermatopathology Dec 2023
Topics: Humans; POEMS Syndrome; Plasmacytoma; Bone and Bones
PubMed: 37982467
DOI: 10.1097/DAD.0000000000002561 -
Transplantation and Cellular Therapy Feb 2024POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare form of plasma cell dyscrasia often treated with...
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare form of plasma cell dyscrasia often treated with high-dose chemotherapy and autologous stem cell transplantation (ASCT). ASCT has resulted in satisfactory and sustained therapeutic outcomes. However, a substantial number of patients eventually experience disease progression, requiring second-line treatment. Therefore, it would be of further benefit to identify patients who will acquire the best long-term survival after ASCT. The aim of this study was to fully reveal the outcomes of patients undergoing ASCT in a large series with long-term follow-up. Long-term outcomes of 239 patients with newly diagnosed POEMS syndrome undergoing ASCT at a single center were evaluated retrospectively. Rates of hematologic complete response (CR) and vascular endothelial growth factor (VEGF) complete response (CR) were 57.3% and 68.6%, respectively, with 90.5% of patients achieving an overall clinical response. At a median follow-up of 94 months, the 5-year overall survival (OS) rate was 92.8%, and the 5-year time to next-line treatment (TTNT) rate was 72.2% (median TTNT, 96 months). Patients achieving CR (5-year TTNT rate, 82.5% versus 60.7%; P < .0001) or CR (5-year TTNT rate 83.7% versus 54.2%; P < .0001) had better survival outcomes compared to non-CR group patients. Dual hematologic and VEGF complete responses carry further benefit for survival (median TTNT, 129 months versus 68 months; P < .0001). Seven cases of second primary malignancy were recorded, all of which were solid tumors. Front-line ASCT resulted in excellent long-term survival in patients with POEMS syndrome, with the best survival observed in those achieving dual hematologic and VEGF CRs.
Topics: Humans; Hematopoietic Stem Cell Transplantation; POEMS Syndrome; Vascular Endothelial Growth Factor A; Retrospective Studies; Treatment Outcome; Transplantation, Autologous
PubMed: 37931801
DOI: 10.1016/j.jtct.2023.11.001 -
Journal of Cutaneous Pathology Feb 2024Papillary hemangioma (PH) is a recently described vascular tumor with a predilection for the skin of the head and neck. Histopathologically, it is characterized by a...
Papillary hemangioma (PH) is a recently described vascular tumor with a predilection for the skin of the head and neck. Histopathologically, it is characterized by a bland endothelial proliferation arranged in a papillary configuration, bearing resemblance to glomeruloid hemangioma seen in the context of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes syndrome. The largest cutaneous PH reported to date measured 1.5 cm in greatest dimension. Here, we report a case of PH with an alarming size of 10 cm. We present this case to add to the limited literature on this rare tumor, highlight the histopathologic differences between PH and its mimics, and emphasize the variable nature of PH tumor size.
Topics: Humans; POEMS Syndrome; Hemangioma; Skin Neoplasms; Skin; Vascular Neoplasms
PubMed: 37916657
DOI: 10.1111/cup.14554 -
Heliyon Oct 2023POEMS syndrome is a rare and complex plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, high M protein levels, and skin changes....
BACKGROUND
POEMS syndrome is a rare and complex plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, high M protein levels, and skin changes. Understanding of POEMS syndrome has advanced rapidly since the 21st century. This study aims to summarize and evaluate the research status of POEMS syndrome in the past 23 years through a bibliometric and visualization analysis, and identify research focuses and emerging hotspots for the future.
METHODS
POEMS syndrome-related articles published between January 1, 2000, and March 8, 2023, were systematically retrieved from the Web of Science Core Collection. Data processing and visualization analysis were carried out using a combination of R software, HistCite, VOSviewer, and CiteSpace.
RESULTS
Since entering the 21st century, 3677 authors from 1125 institutions in 68 countries/regions have published 830 original and review articles on POEMS syndrome in 408 journals so far, among which the USA, Japan, and China published the most articles, and Mayo Clinic, Udice French Research Universities, and Peking Union Medical College listed the top three most prolific institutions. However, collaborative research across countries and groups in the study of POEMS syndrome remain significantly limited. Angela Dispenzieri ranked first in POEMS syndrome research from every aspect of authors, producing the most papers and contributing the most-cited article, followed by Satoshi Kuwabara and Sonoko Misawa. was the most productive journal on POEMS syndrome. "endothelial growth factor" was the keyword with the highest occurrence except for "POEMS syndrome", and "bevacizumab", "lenalidomide", "dexamethasone", and "management" were recognized as emerging topics.
CONCLUSION
This study utilized bibliometric and visualization analysis to systematically summarize the research of POEMS syndrome in the first two decades of the 21st century, offering a data-based and objective perspective on the field of POEMS syndrome and guiding researchers in the identification of novel research directions.
PubMed: 37842561
DOI: 10.1016/j.heliyon.2023.e20612 -
Muscle & Nerve Nov 2023Vascular thrombosis is prevalent among patients with polyneuropathy, organomegaly, endocrinopathy M-protein, and skin changes (POEMS) syndrome. The endothelial cells in...
INTRODUCTION/AIMS
Vascular thrombosis is prevalent among patients with polyneuropathy, organomegaly, endocrinopathy M-protein, and skin changes (POEMS) syndrome. The endothelial cells in the endoneurium are often hypertrophied and the lumen is frequently occluded. Consequent local hypoxia may increase vascular endothelial growth factor (VEGF), which induces hypercoagulation and vascular permeability.
METHODS
This study presents two patients in the fifth decade of life, who had rare nerve biopsy findings of vascular occlusion mainly by platelets. Before the cases presented here, we encountered nine confirmed POEMS patients whose nerve biopsies did not show similar findings.
RESULTS
A small artery and a vein were occluded, but no atherosclerotic changes were observed. The endothelial cells that adhered to the packed platelets lost their junctions.
DISCUSSION
Platelet aggregation, degranulation, and ischemia may cause a loose endothelial barrier and leak proinflammatory cytokines, such as interleukin-12. This may increase production of VEGF and may cause nerve demyelination. Small vessel platelet thrombosis may contribute to the pathogenesis of this disorder.
Topics: Humans; Vascular Endothelial Growth Factor A; POEMS Syndrome; Endothelial Cells; Peripheral Nerves; Thrombosis
PubMed: 37837287
DOI: 10.1002/mus.27986 -
International Journal of Surgery Case... Oct 2023Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare multisystemic disease that is named for its constellation...
INTRODUCTION
Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare multisystemic disease that is named for its constellation which can easily be mistaken for other disorders.
CASE PRESENTATION
In present study we evaluated a 39-year-old man with hypercalcemia and parathyroid hormone. He underwent two failed surgeries for parathyroid adenoma but due to the presence of other features of the syndrome, a diagnosis of POEMS syndrome was made. His symptoms were multiple peripheral neuropathies, monoclonal plasma cell proliferative disorder, multiple bony lesions alongside endocrinopathy, lymphadenopathy, extravascular volume overload, mildly hypocellular marrow with polymorphic population, trilineage hematopoiesis, and progressive maturation less than 5 % plasma cell in bone marrow biopsy and aspiration. Patient was treated with the administration of Pomalidomide and autologous stem cell transplantation after which his symptoms improved and laboratory test results normalized.
DISCUSSION
POEMS syndrome is a rare disorder that is challenging to diagnose due to its variable clinical presentation and similarity to other disorders. However, specific criteria can help distinguish it from other syndromes. In this case, we witness the comorbidity of Ganglioneuroma. Also, hyperglycemia with hypercalcemia and high PTH in this patient are not common endocrine disorders in POEMS syndrome which there are some possible explanations for these symptoms' presentation.
CONCLUSION
Although endocrine disorders are an important part of POEMs syndrome, some characteristic of this disease is unknown, and it becomes more difficult to diagnose in early stages, that in these cases, thorough physical examinations and laboratory results may help with the early diagnosis.
PubMed: 37793230
DOI: 10.1016/j.ijscr.2023.108877 -
Indian Journal of Hematology & Blood... Oct 2023
PubMed: 37786834
DOI: 10.1007/s12288-023-01637-2 -
Frontiers in Pharmacology 2023Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is a multisystem disorder that has limited treatment...
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is a multisystem disorder that has limited treatment options. Here, we described a case of a 55-year-old female subject who was treated for multiple drugs, but the skin symptoms continued to progress; the patient responded well to baricitinib. This suggests that JAK/STAT signaling pathways play an essential role in the pathological process of POEMS syndrome.
PubMed: 37767406
DOI: 10.3389/fphar.2023.1191158 -
Annals of Hematology Jan 2024
Review
Topics: Humans; Plasmacytoma; Lymphadenopathy; POEMS Syndrome
PubMed: 37743381
DOI: 10.1007/s00277-023-05465-2