골수형성이상증후군에 동반된 자가면역용혈빈혈

Abstract

Autoimmune paraneoplastic syndromes are encountered in patients with myelodysplastic syndromes. A review of case reports suggested 10% of myelodysplastic syndrome patients may experience various autoimmune syndromes, associated with immunological-mediated hematologic abnormalities, such as Coombs' negative hemolytic anemia. In patients with myelodysplastic syndrome, Coombs' negative hemolytic anemia may be underdiagnosed due to its association with reticulocytopenia. Therefore, the findings from a peripheral blood smear and measurement of haptoglobin level are important. Autoimmune manifestations respond to immunosuppressive agents, including steroids. Herein, a case with Coombs' negative hemolytic anemia, diagnosed with myelodysplastic syndrome from a bone marrow biopsy, is reported, with a review of the previously reported literature.