Abstract
IntroductionAdult onset Still's disease is a chronic multisystemic inflammatory disorder characterized by high spiking fever, polyarthralgia and rash. Lymphadenopathy is a prominent feature of adult onset Still's disease and is seen in about 65% of patients. Searching the medical literature using the MEDLINE database from January 1966 through November 2007 we could only find two reported cases of adult onset Still's disease that had progressed to lymphoma.Case presentationWe 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.ConclusionAlthough 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.
Highlights
Adult onset Still's disease is a chronic multisystemic inflammatory disorder characterized by high spiking fever, polyarthralgia and rash
Lymphadenopathy is a prominent feature of Adult onset Still's disease (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) 10 months after the onset of her symptoms
Summary
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]. A 32-year-old woman presented in 1991 with a 2-month history of fever reaching 39.5°C and associated with arthritis in the knees, ankles and shoulders, a nonpruritic skin rash, myalgia and weight loss Her laboratory studies, including liver function tests, were within the normal ranges except that she had an elevated erythrocyte sedimentation rate (ESR) of 110 mm/hr and lactate dehydrogenase (LDH) of 1975 IU/L (Normal Range: 200–480). A diagnosis of AOSD was made based on the Yamaguchi criteria [5] She had 2 major criteria: arthritis and fever, and 3 minor criteria: lymphadenopathy, elevated LDH and negative rheumatoid factor and antinuclear antibodies. Re-evaluation revealed CNS relapse with CSF involvement with malignant lymphoma cells, and without evidence of systemic disease She was treated with intrathecal methotrexate, intermediate dose Ara-C, high dose methotrexate and whole brain irradiation. She passed away from hepatorenal syndrome 13 years after the diagnosis of lymphoma
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