Angioimmunoblastic lymphadenopathy: common symptoms, uncommon diagnosis

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.