Ab stra ct Sjö gren syndrome (S S) is a chronic autoimmune dis order character ize d by lympho c y tic infiltration of exo cr ine g lands. Its c or relation w ith B-c ell-typ e lymphoma is justly do cumente d. We intro duc e a patient w ith S S c omplicate d w ith hair y-c ell leukemia (HCL). A 51-year-old female patient, who was diag no s e d w ith pr imar y S S sinc e 12 years ag o, was admitte d to the ho spital b e caus e of intractable vomiting and ab dominal dis c omfor t due to hug e splenome galy. The blo o d c ount show e d anemia, thromb op enia, and s evere leuko c y tosis. B one mar row biop sy and flow-c y tometr ic analysis was diag no stic for HCL . She under w ent chemotherapy and splene ctomy b e caus e of symptomatic splenom-egaly. Prev ious studies and available literature de clare a clo s e link b etw e en B-c ell ne oplasia and autoimmune dis eas es. Als o, the ev idenc es lead to the supp o sition that S S and other autoimmune dis orders may for m par t of a sp e ctr um of lymphoproliferative dis eas es which share c ommon or ig ins w ith clonal ne oplastic B-c ell proliferation.
Keyword: Sjö gr en syndrome, Hair y-c ell leukemia, Non Ho dgkin lymphoma, Chronic lympho cyctic leukemia