Autoimmune cytopenia does not predict poor prognosis in chronic lymphocytic leukemia/small lymphocytic lymphoma†

Abstract

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is characterized by an acquired immune defect that can cause autoimmune complications, including anemia and thrombocytopenia. We conducted an observational study of the epidemiology, clinical presentation and significance of autoimmune complications of CLL/SLL in 132 patients from a large population (>45,000 veterans), in which at least 90% of patients with CLL/SLL have been previously identified. Over a period of 12.5 years, 12 patients (9.1%) had autoimmune complications; of these, 6 (4.5%) had autoimmune hemolytic anemia (AIHA), 5 (3.8%) had immune thrombocytopenia (ITP), and 1 (0.8%) had pure red blood cell aplasia (PRBA). All 6 cases of AIHA had a positive direct immunoglobulin test for IgG and C3d. In 6 patients, CLL/SLL was an incidental finding at the time of presentation with autoimmune cytopenia. Nine out of 10 patients responded to immunosuppressive therapy, which was complicated by serious infection in 7 cases, one of which was fatal. The major cause of mortality in patients with autoimmune complications of CLL/SLL was secondary malignancy. Survival of patients with immune cytopenia was not significantly different from CLL/SLL patients without immune cytopenia. Among patients with anemia or thrombocytopenia, mortality was significantly higher in those with bone marrow failure compared to an autoimmune etiology. We show that in a non-referred population with a high incidence of CLL/SLL, autoimmune cytopenia can occur early in the natural history of the disease. These data suggest that the Rai and Binet classifications for CLL need to be modified for patients with autoimmune cytopenia. Am.

Direct Coombs Test Positivity in B-Chronic Lymphoid Leukemia: a Marker of Advanced Clinical Disease

“…Various studies conducted in United State, Israel, Spain, England and Rome have reported a frequency of positive DAT in 4.5%, 7%, 4.3%, 7.7% and 3.7% of CLL patients respectively (Kyasa et al, 2003;Lischner et al, l988;Moreno et al, 2010;Hamblin et al, 1986;Mauro et al, 2000). However the prevalence of DAT positivity in our patients appears to be higher than these studies conducted in western world.…”

Autoimmune thrombocytopenia in non-Hodgkin's lymphomas

“…Among the hematologic autoimmune disorders, AIHA is more common than ITP while in CLL, AIHA was 3.3 times more common than ITP (8)(9)(10)(11). Both are less frequent in non-CLL-NHL and AIHA was only approximately twice as frequent as ITP (AIHA 1.57%, ITP 0.76%).…”

“…The frequency of ITP in chronic lymphocytic leukemia (CLL) and NHL has been determined in several studies . [8][9][10][11][12][13][14][15] We analyzed data of individual non-CLL NHL patients with ITP from published case reports and original series of our institute with regard to the temporal relationship between ITP and lymphoma, the frequency in NHL subtypes, laboratory data and treatment outcome.…”

Clinical implications of ZAP‐70 expressionin chronic lymphocytic leukemia

“…Moreover, progressive versus indolent forms of the disease are not identified. In addition, the mechanisms accounting for cytopenias are not taken into consideration, yet there is some indication that patients with cytopenias of immune origin may have a better outcome than those in whom the cytopenia is caused by a massive infiltration of the bone marrow by neoplastic cells (5,6). In addition, clinical stages are not good predictors of response to therapy.…”