Chronic myelomonocytic leukemia and primary Sjögren's syndrome

Although associations of Sjögren's syndrome (SS) with malignant affections are well known, an association with chronic myelomonocytic leukemia (CMML) is infrequent. Our report concerns an 80-year-old woman with chronic polyarthrosis, hospitalized for an anemic syndrome, who also complained of buccal dryness. Clinical examination showed simply parotid swelling and discrete splenomegaly. The diagnosis of SS appeared to be primary. The hemogram, myelogram and bone biopsy indicated CMML. During SS, the possibility of occurrence of a lymphoproliferative syndrome is well documented, and other malignant affections are much less likely to be found in the absence of immunosuppressive treatment. The particular case of SS and associated CMML detected at the same time suggests either the favorable role of monocytic proliferation on immunity or a stem clonal anomaly affecting monocytes and B lymphocytes.