Non-Hodgkin's lymphoma in a woman with adult-onset Still's disease: a case report

We describe a woman who was diagnosed with adult onset Still's disease and developed lymphoma 10 months after the onset of her symptoms. She initially presented with fever and arthritis of the knees, ankles and shoulders, along with a nonpruritic skin rash, myalgia and weight loss. On physical examination she was found to have several enlarged anterior cervical lymph nodes and left posterior auricular lymph nodes all of which were non-tender, immobile and rubbery. Excisional biopsy of the cervical lymph nodes was negative for malignancy. Bone marrow biopsy was also negative for malignancy. She was treated with prednisone. She remained in good health until she presented 10 months later with low back pain, dyspnea and weight loss. Work up revealed malignant lymphoma. She was treated with chemotherapy and was doing well until she presented with abdominal pain. Work up revealed a cirrhotic liver and ascites. She then passed away from hepatorenal syndrome 13 years after the diagnosis of lymphoma. To our knowledge, this is the third reported case of such an occurrence.Although the association between adult onset Still's disease and lymphoma has been rarely reported, careful screening for this malignancy in patients suspected to have adult onset Still's disease is warranted.Adult onset Still's disease (AOSD) is a chronic multisystemic inflammatory disorder of unknown origin characterized by a high spiking fever, polyarthralgia, a salmon pink evanescent rash, and hepatosplenomegaly [1]. Notable laboratory features of the disease are increased serum levels of C-reactive protein (CRP), leukocytosis, liver dysfunction, negative results for both rheumatoid factor and antinuclear antibodies, and an increased incidence of hyperferritinemia [2,3]. Lymphadenopathy is a prominent feature of AOSD seen in about 65% of patients [4] and necessitating in most instances a biopsy to rule out lymphoma.We describe a woman who was diagnosed with AOSD and developed non-hodgkin's lymphoma (NHL)