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目的提高对华氏巨球蛋白血症(WM)特殊临床表现的认识及诊治水平。方法报告2015年1月6日入院的1例以黄疸为首发表现的华氏巨球蛋白血症患者的临床资料、诊断及治疗并复习相关文献。结果华氏巨球蛋白血症是一种淋巴增殖性疾病,以骨髓淋巴浆样细胞浸润和血清单克隆免疫球蛋白M(Ig M)升高为特点,临床以黄疸为首发表现者罕见,易误诊及漏诊,给予含有利妥昔单抗的初始化学治疗方案前需监测血清Ig M水平以防出现Ig M反跳现象及相应并发症。结论对于以黄疸为首发表现的患者,在排除肝脏系统疾病后应考虑浆细胞系统疾病可能,及时安排病理活体组织检查及相应检查以明确诊断;患者在接受以利妥昔单抗为基础的治疗前需监测Ig M水平,当Ig M>50 g/L时应先采取预防性血浆置换。
Objective To improve the understanding of the special clinical manifestations of Fahrenheit macular dysuria (WM) and its diagnosis and treatment. Methods The clinical data, diagnosis and treatment of 1 case of Fahrenheit macular leukemia who presented with jaundice on January 6, 2015 were retrospectively reviewed and reviewed. Results Fahrenheit macroglobulinemia is a lymphoproliferative disorder characterized by increased myeloid lymphocyte-like cell infiltration and increased serum IgM (IgM). Clinical manifestations of jaundice are rare and misdiagnosed And misdiagnosis, serum IgM levels should be monitored before initiating chemotherapeutic regimens with rituximab to prevent IgM rebound and consequent complications. Conclusion For patients with jaundice as the first manifestation, we should consider the possibility of systemic diseases of plasma cells after excluding the diseases of the liver system, and arrange the pathological biopsy and corresponding examination in time to confirm the diagnosis. Patients undergoing rituximab-based treatment Pre-IgM levels should be monitored and prophylactic plasmapheresis should be taken when Ig M> 50 g / L.