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A clinical case of a 55-year-old man with syndrome of primary Sjögren, seronegative, normoglobulinemic and normocomplementary with 7 years of evolution, which developed acute lymphoblastic leukemia, having suffered recurrent lympho-adenopathy and paresthetic meralgia as previous clinical signs, is presented. Clinical, serological and immunologic characteristics of this syndrome are reviewed, highlighting its rare appearance among men. While appearance of malignant lymphoproliferation disorders is well known, evolution to acute or chronic myeloid leukemia is very rare and so far, an acute lymphoblastic leukemia as the one presented in this study has never been described.
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