Sadovsky R, Zazakos C
Postgrad Med. 1977 Jul;62(1):213-6. doi: 10.1080/00325481.1977.11712262.
After five months of treatment for what was believed to be tuberculosis, a 49-year-old woman had recurrence of high spiking fever, lymphadenopathy, and malaise along with an intermittent maculopapular rash. Other findings were a positive Coombs test, iron deficiency, elevated gamma globulin levels, polyclonal gammopathy, and hepatosplenomegaly. Biopsy of a cervical lymph node revealed an angioblastic pattern consistent with angioimmunoblastic lymphadenopathy. Review of biopsy material obtained six months previously showed the same changes. Antituberculosis therapy was discontinued, and a regimen of prednisone and iron replacement was begun. The patient did well initially; when symptoms returned, they were controlled by adding azathioprine to the regimen for steroid-sparing effect. However, serologic abnormalities returned and within a few months, symptoms exacerbated; despite intensive medical therapy, the patient died. Angioimmunoblastic lymphadenopathy is a recently recognized disorder with a usually progressive course. No treatment has yet been established as effective, and death usually occurs within one year after diagnosis.
一名49岁女性因疑似肺结核接受了五个月的治疗后,出现高热反复、淋巴结病、全身不适,伴有间歇性斑丘疹。其他检查结果包括库姆斯试验阳性、缺铁、γ球蛋白水平升高、多克隆丙种球蛋白病以及肝脾肿大。颈部淋巴结活检显示血管母细胞型,符合血管免疫母细胞性淋巴结病。回顾六个月前获取的活检材料,显示同样的变化。抗结核治疗停止,开始使用泼尼松和铁剂替代治疗方案。患者最初情况良好;症状复发时,通过在治疗方案中添加硫唑嘌呤以发挥激素节省效应来控制症状。然而,血清学异常再次出现,几个月内症状加重;尽管进行了强化治疗,患者仍死亡。血管免疫母细胞性淋巴结病是一种最近才被认识的疾病,通常病程呈进行性。目前尚未确定有效的治疗方法,诊断后通常在一年内死亡。