Autoimmune cytopenias in chronic lymphocytic leukemia

Abstract

Chronic lymphocytic leukemia (CLL) is frequently complicated by secondary autoimmune cytopenias (AIC) represented by autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), pure red cell aplasia, and autoimmune granulocytopenia. The distinction of immune cytopenias from cytopenias due to bone marrow infiltration, usually associated with a worse outcome and often requiring a different treatment, is mandatory. AIHA and ITP are more frequently found in patients with unfavorable biological risk factors for CLL. AIC secondary to CLL respond less favorably to standard treatments than their primary forms, and treating the underlying CLL with chemotherapy or monoclonal antibodies may ultimately be necessary. Am. J. Hematol. 89:1055–1062, 2014. © 2014 Wiley Periodicals, Inc.

Bendamustine and rituximab combination in the management of chronic lymphocytic leukemia‐associated autoimmune hemolytic anemia: A multicentric retrospective study of the French CLL intergroup (GCFLLC/MW and GOELAMS)

“…Very few data on CLL-associated AIHA are available, especially in relapsed AIHA [12]. We report herein the results of the combination of BR in a rather large series of 26 patients presenting with AIHA associated to CLL.…”

Treatment of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma Presenting Simultaneously with Acquired Hemophilia and Warm Autoimmune Hemolytic Anemia

“…Treatment of WAHA and CLL has been found to be successful with rituximab, cyclophosphamide, and dexamethasone, resulting in a response rate of 89.5% in a small cohort of 26 patients (8). When also considering AHA, the combination of cyclophosphamide, steroids, and rituximab achieved a quick and durable response.…”

“…Th e diagnosis of WAHA is confirmed when other forms of anemia have been excluded and there are laboratory signs of hemolysis. Hemolysis in CLL develops secondary to IgG antibodies produced by nonmalignant B-cell clones directed against red blood cell antigens, leading to clearance by the spleen and extravascular hemolysis (8). Th e fi rst-line treatment is steroids, 1 mg/kg/day, but this results in long-term remission in <15% to 20% of patients (9).…”

The role of rituximab in adults with warm antibody autoimmune hemolytic anemia

“…28 Rituximab in some special presentations of wAIHA Chronic lymphocytic leukemia-associated AIHA Autoimmune cytopenias are well-known complications of chronic lymphocytic leukemia (CLL), AIHA being the most common with an estimated incidence of up to 10%. 29 CLL patients with AIHA seem to have a better prognosis compared with those with advanced stage (Binet C) showing anemia due to bone marrow infiltration, but have a worse survival compared with early stage (Binet A) patients without anemia. 30,31 For patients diagnosed with CLL-associated wAIHA, firstline therapy consists of steroids, similar to primary wAIHA.…”