PDF(Pubmed)
Abstract：
UNASSIGNED: We report an observation of a young patient presenting with severe type 1 cryoglobulinemic vasculitis revealing a monoclonal gammopathy of clinical significant. Treatment with Melphalan-Thalidomide Prednisone improved the symptoms. Early diagnosis would prevent serious tissue damage.
UNASSIGNED: Monoclonal gammopathy encompass diverse clinical forms. Only the cancerous form, multiple myeloma (MM), is treated based on specific diagnostic criteria. A new clinical entity, monoclonal gammopathy of clinical significance (MGCS), warrants special attention due to its need for specific treatment. It involves patients with signs of potentially severe organ involvement that do not meet MM criteria. We present the case of a 34-year-old Malagasy woman with severe type I cryoglobulinemic vasculitis associated with noncancerous monoclonal gammopathy, showing a favorable outcome after treatment with Thalidomide. Symptoms included toe necrosis, a severe ulcer on the left calf evolving for 3 months, and stocking-like dysesthesias. Investigations revealed monoclonal gammopathy at 30.1 g/L, proteinuria at 1 g/24 h, medullary plasma cell at 6%, and circulating cryoglobulin of Ig kappa type. CRAB criteria (anemia, hypercalcemia, renal insufficiency, and osteolysis) were absent. Treatment with Thalidomide, combined with corticosteroids and local care for 4 months, resulted in ulcer healing, disappearance of dysesthesias, and persistent normalization of gammaglobulin. Our case underscores the importance of specific treatment for MGCS.
UNASSIGNED: Monoclonal gammopathy encompass diverse clinical forms. Only the cancerous form, multiple myeloma (MM), is treated based on specific diagnostic criteria. A new clinical entity, monoclonal gammopathy of clinical significance (MGCS), warrants special attention due to its need for specific treatment. It involves patients with signs of potentially severe organ involvement that do not meet MM criteria. We present the case of a 34-year-old Malagasy woman with severe type I cryoglobulinemic vasculitis associated with noncancerous monoclonal gammopathy, showing a favorable outcome after treatment with Thalidomide. Symptoms included toe necrosis, a severe ulcer on the left calf evolving for 3 months, and stocking-like dysesthesias. Investigations revealed monoclonal gammopathy at 30.1 g/L, proteinuria at 1 g/24 h, medullary plasma cell at 6%, and circulating cryoglobulin of Ig kappa type. CRAB criteria (anemia, hypercalcemia, renal insufficiency, and osteolysis) were absent. Treatment with Thalidomide, combined with corticosteroids and local care for 4 months, resulted in ulcer healing, disappearance of dysesthesias, and persistent normalization of gammaglobulin. Our case underscores the importance of specific treatment for MGCS.
摘要：
■我们报告了一名年轻患者的观察结果，该患者表现为严重的1型冷球蛋白血症性血管炎，显示出具有临床意义的单克隆丙种球蛋白病。美法仑-沙利度胺泼尼松治疗可改善症状。早期诊断可以防止严重的组织损伤。
■单克隆丙种球蛋白病包括多种临床形式。只有癌变的形式，多发性骨髓瘤（MM），根据特定的诊断标准进行治疗。一个新的临床实体，临床意义的单克隆丙种球蛋白病（MGCS），由于需要特殊治疗，因此值得特别注意。它涉及具有不符合MM标准的潜在严重器官受累迹象的患者。我们介绍了一名34岁的马达加斯加妇女，患有与非癌性单克隆丙种球蛋白病相关的严重I型冷球蛋白血症性血管炎，沙利度胺治疗后显示良好的结果。症状包括脚趾坏死，左小腿严重溃疡持续3个月,和长袜般的美感。调查显示单克隆丙种球蛋白病为30.1g/L，蛋白尿在1克/24小时，6％的髓质浆细胞，和循环的Igκ型冷球蛋白。CRAB标准（贫血，高钙血症,肾功能不全,和骨质溶解）不存在。用沙利度胺治疗，结合皮质类固醇和局部护理4个月，导致溃疡愈合，审美障碍的消失，丙种球蛋白持续正常化。我们的案例强调了MGCS特定治疗的重要性。
■单克隆丙种球蛋白病包括多种临床形式。只有癌变的形式，多发性骨髓瘤（MM），根据特定的诊断标准进行治疗。一个新的临床实体，临床意义的单克隆丙种球蛋白病（MGCS），由于需要特殊治疗，因此值得特别注意。它涉及具有不符合MM标准的潜在严重器官受累迹象的患者。我们介绍了一名34岁的马达加斯加妇女，患有与非癌性单克隆丙种球蛋白病相关的严重I型冷球蛋白血症性血管炎，沙利度胺治疗后显示良好的结果。症状包括脚趾坏死，左小腿严重溃疡持续3个月,和长袜般的美感。调查显示单克隆丙种球蛋白病为30.1g/L，蛋白尿在1克/24小时，6％的髓质浆细胞，和循环的Igκ型冷球蛋白。CRAB标准（贫血，高钙血症,肾功能不全,和骨质溶解）不存在。用沙利度胺治疗，结合皮质类固醇和局部护理4个月，导致溃疡愈合，审美障碍的消失，丙种球蛋白持续正常化。我们的案例强调了MGCS特定治疗的重要性。
