以血管外容量负荷增大为首诊表现的POEMS综合征临床特征:单中心回顾性研究
Clinical characteristics of POEMS syndrome initially diagnosed as increased extravascular volume load: a single-center retrospective study
POEMS综合征是一种罕见的浆细胞恶液质,临床表现包括多发性神经病、单克隆免疫球蛋白病、血管外容量负荷增大、内分泌病、脏器肿大和皮肤改变等。由于患者多系统受累,就诊时复杂不典型的症状使得早期诊断面临挑战。该病首发症状以周围神经病变——肢体麻木最常见,而关于血管外容量负荷增大的病例报道少见。研究收集并分析两组不同首发症状(血管外容量负荷增大、肢体麻木)患者的临床资料,总结其临床特点和治疗反应。
更多POEMS syndrome is a rare plasma cell dysplasia. Its clinical manifestations include polyneuropathy, monoclonal protein, increased extravascular volume load, endocrinopathy, organomegaly and skin changes. The complex and atypical symptoms at presentation make early diagnosis challenging due to multiple system involvement. Peripheral neuropathy, limb numbness, is the most common initial symptom of this disease. However, case reports of increased extravascular volume load are rare. This article collected and analyzed the clinical data of two groups of patients with different initial symptoms (increased extravascular volume load and limb numbness). The clinical characteristics and treatment responses were summarized.
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