We experienced a 47-year-old Japanese female with polyneuropathy, edema, hypertrichosis, hyperpigmentation, and white nail, which were diagnostic as having Crow-Fukase syndrome. Laboratory and radiological evaluation showed neither plasma cell dyscrasia nor monoclonal gammopathy. Increased factor VIII activity and thrombocytosis, which suggested thrombotic tendency, were observed at the exacerbation of clinical symptoms. In her third exacerbation, she presented marked cyanosis in her right foot, and angiography confirmed narrowing of arteries at the ankle. Increased serum interleukin-6 was also observed, and the production of interleukin-6 by endothelial cells of cutaneous angioma was shown. Possible role of interleukin-6 in Crow-Fukase syndrome was discussed.
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POEMS Syndrome.
Presse Med
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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.
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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.
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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
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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").
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Case report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome.
Front Med (Lausanne)
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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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