Seropositive rheumatoid arthritis with dermatomyositis sine myositis, angioimmunoblastic lymphadenopathy with dysproteinemia-type T cell lymphoma, and B cell lymphoma of the oropharynx.

Abstract

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder that often progresses to high grade T cell lymphoma. We describe a 63-year-old woman with longstanding seropositive rheumatoid arthritis who developed fever, cutaneous findings of dermatomyositis, a diffuse pruritic maculopapular rash, enlarged lymph nodes, polyclonal elevated serum gammaglobulins, and an IgG lambda paraprotein. Lymph node biopsies yielded tissue with characteristic changes of AILD and T cell lymphoma. Interleukin 6 (IL-6) was present during the early, active phase of disease, and circulating IL-6 and IL-2 were detected one month before tumor recurrence. Two years after AILD and T cell lymphoma were diagnosed, she developed a B cell lymphoma that involved the oropharynx.