A 66-year-old woman presented with anemia in January 2006. She was admitted to our Department in February, after laboratory data showed pancytopenia and bone marrow biopsy reticulin fibrosis. The results of the diagnostic work-up, which included the anti-SS-A antibody, anti-SS-B antibody positivity and salivary gland scintigraphy, Schirmer test and Rose Bengal test, supported the classification criteria of Sjögren syndrome. Due to secondary myelofibrosis accompanied by Sjögren syndrome, she was started on prednisolone (PSL) and recovered completely from the anemia and thrombocytopenia. After the PSL was tapered, a recent follow-up indicated that the peripheral blood had normalized with the PSL therapy. As a causal disease of autoimmune myelofibrosis in collagen disease, systemic lupus erythematosus occurs frequently. This patient is considered to be a rare case in whom secondary myelofibrosis was accompanied by Sjögren syndrome.
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