Castleman disease (CD) is a very rare disorder characterised by hyperplasia of the lymphoid tissue. The aetiology varies considerably and includes autoimmunological, infectious, autoinflammatory and paraneoplastic diseases (e.g. MGUS with POEMS syndrome). What they all have in common is usually a dysregulation/overproduction of certain cytokines and growth factors (including interleukin 6 and VEGF). The sum of these changes sometimes causes very heterogeneous symptoms and thus often makes early diagnosis difficult. The prognosis of unrecognised and untreated disease is very serious and has an average 5‑year survival rate of 55-77%. The present paper describes the case of a 79-year-old patient with refractory polyserositis who was correctly diagnosed after > 8 years.
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POEMS Syndrome.
Presse Med
January 2025
Department of Hematology and Cellular Therapy, National Reference Center "AL Amyloidosis and Other Monoclonal Immunoglobulin Deposit Diseases, University Hospital of Limoges, Limoges, France.
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, Skin changes) is a syndrome that involves a monoclonal B-cell proliferation, most often plasmacytic, and a variable number of manifestations listed or not in the acronym. These manifestations include sclerotic bone lesions, plasmacytic Castleman disease, papillary edema, peripheral edema, ascites, thrombocytosis and/or polycythemia, venous and/or arterial thrombosis, and renal, pulmonary, and cardiac impairments . Diagnosis is often delayed due to the rarity of this entity and its clinical polymorphism, which can mimic other neurological disorders.
View Article and Find Full Text PDFUnraveling Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome: Diagnostic Challenges and Therapeutic Strategies at a National Tertiary Care Center.
Cureus
December 2024
Internal Medicine, National Medical Center November 20, Mexico City, MEX.
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) syndrome is a rare paraneoplastic disorder caused by plasma cell proliferation and overproduction of cytokines, particularly vascular endothelial growth factor (VEGF). This complex syndrome affects multiple organ systems and presents with a broad range of clinical and laboratory manifestations, which can complicate both diagnosis and management. Not all components of the acronym are observed in every patient, highlighting the clinical heterogeneity of the condition.
View Article and Find Full Text PDFClinical Impact of Osteolytic Bone Lesions in POEMS Syndrome: A Single-Center Experience of 114 Patients.
Hematol Oncol
January 2025
Department of Hematology, Chiba University Hospital, Chiba, Japan.
POEMS syndrome is a multisystemic disease associated with monoclonal plasma cell disorders. Although the presence of bone lesions is included in the diagnostic criteria, their precise manifestations remain unknown. Here, we retrospectively analyzed the bone lesions in patients with POEMS syndrome and evaluated their clinical features.
View Article and Find Full Text PDFUtility of word embeddings from large language models in medical diagnosis.
J Am Med Inform Assoc
January 2025
Kennewick, WA 99338, United States.
Objective: This study evaluates the utility of word embeddings, generated by large language models (LLMs), for medical diagnosis by comparing the semantic proximity of symptoms to their eponymic disease embedding ("eponymic condition") and the mean of all symptom embeddings associated with a disease ("ensemble mean").
Materials And Methods: Symptom data for 5 diagnostically challenging pediatric diseases-CHARGE syndrome, Cowden disease, POEMS syndrome, Rheumatic fever, and Tuberous sclerosis-were collected from PubMed. Using the Ada-002 embedding model, disease names and symptoms were translated into vector representations in a high-dimensional space.
Case report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome.
Front Med (Lausanne)
December 2024
Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China.
POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy.
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