Hematologic disorders in systemic lupus erythematosus

Abstract

A total of 194 cases of patients suffering from systemic lupus erythematosus were reviewed. The hematologic disorders that appeared in the classification criteria were: autoimmune hemolytic anemia (Coombs-positive) in 14 patients (7%), leukopenia in 38 patients (19%), lymphopenia in 28 (14%) and thrombocytopenia in 10 patients (5%). Three patients had the combination of Coombs-positive hemolytic anemia with thrombocytopenia (Fisher-Evans syndrome). Leukopenia was combined with other disorders in 21 cases: leukopenia and lymphopenia in 15 cases; 2 cases of neutropenia, lymphopenia and thrombocytopenia; one case of leukopenia with thrombocytopenia; 3 cases of autoimmune hemolytic anemia with leukopenia. There was no combination of hemolytic anemia with lymphopenia. A patient with Evans-Fisher syndrome also had cryoglobulinemia. In 8 cases of hemolytic anemia the lupus anticoagulant was positive, and the serology was reactive (false positive) in 4 cases; with a combination of autoimmune hemolytic anemia, positive lupus anticoagulant and false-positive serology in some occasions. The statistics include 25 deaths in the group with hematologic disorders, of which 19 patients underwent necropsy and 6 did not. The findings showed 13 cases with significant infections, 9 with vasculitis and 7 patients with necropsy had antiphospholipid syndrome. One patient who died with a diagnosis of pulmonary embolism and myocardial infarction did not undergo the lupus anticoagulant test. Two living patients have antiphospholipid syndrome.