A case of adult-onset Still's disease complicated by non-Hodgkin's lymphoma

Abstract

Left cervical lymphadenopathy developed in a 50-year-old male who had a history of adult-onset Still's disease for the preceding 18 months. Still's disease is characterized by rash, fever, and leukocytosis. Lymphadenopathy has been reported in about 60% of the patients, and most histopathologic studies have shown non-specific reactive hyperplasia. However, in this case, an open biopsy of the cervical node revealed a histology of diffuse large B-cell lymphoma. The B-cell malignant lymphoma that developed may have resulted from a sequential progression of a previous stage of benign lymphoproliferative lesion. Our case suggests that the pathophysiology of adultonset Still's disease involves the stimulation of lymphoid systems to the point of progression towards lymphoma. Malignant lymphoma should be added to the list of life-threatening complications which, although rare, are associated with this disease.

Keywords

adult onset Still's disease lymphadenopathy malignant lymphoma

Get full access to this article

View all access options for this article.

References

1 Bujak J , Aptekar RG , Decker JL , Wolff SM . Juvenile rheumatoid arthritis presenting in the adult as fever of unknown origin . Medicine 1973; 52: 431-444 .

2 Yamaguchi M , Ohta A , Tsunematsu T et al. Preliminary criteria for classification of adult still's disease . J Rheumatol 1992; 19: 424-430 .

3 Trotta F , Dovigo L , Scapoli G et al. Immunoblastic malignant lymphoma in adult onset Still's disease . J Rheumatol 1993; 20: 1788-1792 .

4 Schlesinger BE , Forsyth CC , White RHR et al. Observations on the clinical course and treatment of 100 cases of Still's disease . Arch Dis Child 1961; 36: 65-76 .

5 Dorfman RF , Warnke R . Lymphadenopathy simulating malignant lymphomas . Hum Pathol 1974; 5: 519-550 .

6 Valente RM , Banks PM , Conn DL . Characterization of lymph node histology in adult onset Still's disease . J Rheumatol 1989; 16: 349-354 .

7 Ohta A , Matsumoto Y , Ohta T , Kaneoka H , Yamaguchi M . Still's disease associated with necrotizing lymphadenitis (Kikuchi's disease): report of 3 cases . J Rheumatol 1988; 15: 981-983 .

8 Quaini F , Manganelli P , Pileri S et al. Immunohistological characterization of lymph nodes in two cases of adult onset Still's disease . J Rheumatol 1991; 18: 1418-1423 .

9 Zulman J , Jaffe R , Talal N . Evidence that the malignant lymphoma of Sj6gren's syndrome is a monoclonal B-cell neoplasm . N Engl J Med 1978; 299: 1215-1220 .