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During long term follow up (median 10 years) of 412 patients with primary Sjogren's syndrome (pSS) 46; (11,2%) women developed non-Hodgkin's lymphoma (NHL). Median pSS duration before development of NHL was 17 years. 43 (93,3%) patients had B-cell and 3 (6,7%) - T-cell NHL. All types of NHL were present except precursor cell lymphomas. Diffuse large B-cell lymphoma (LBCL) (39%) and MALT lymphomas (21%) prevailed. Nodal NHL with extranodal involvement (52,2%) were the most frequent. Nodal (21,8%) and extranodal (26%) lymphomas were less frequent. The most frequent target organs in lymphoproliferative disease (LPD) were lymph nodes (74%), salivary glands (45,5%), lungs (26%), bone marrow (19,5%), liver (17,5%), spleen (13%), lachrymal glands (6,5%). Waldeyer's throat ring (4%), oral cavity mucous membrane, ovary and brain (2%) were involved rarely. Immunoglobulin-secreting variant of lymphoma was revealed in 53,7% of cases. LPD developed predominantly in patients with systemic features and late stage of pSS. Significant increase of parotis, mixed monoclonal cryoglobulinemia, generalized lymphadenopathy, presence of more than 5 focuses of lymphoid infiltration in small salivary glands biopsies and thrombocytopenia were predictors of NHL development in pSS (p

We describe a rare case of myeloid/NK cell precursor acute leukemia (MNKL) in a 38-year-old woman. Upon diagnostic examination, a tumor invasion of bone marrow, lymphatic nodes, liver and spleen was found. The proportion of blasts in peripheral blood reached 54%. The blasts were polymorphic, with round or irregularly shaped nuclei. The tumor cells were negative for myeloperoxidase, non-specific esterase, and lipid staining. The blasts not only expressed CD13+ and CD33+ panmyeloid antigens, but also carried the markers of NK-cell differentiation (CD16+, CD56+, Perforin+). The leukemoid infiltration of liver, acute hepatitis and development of liver cell failure were the distinctive features of the described case. The issues pertaining to differential diagnostics and classification of MNKL are also discussed.