Autoimmune cytopenia in chronic lymphocytic leukemia/small lymphocytic lymphoma: changes in clinical presentation and prognosis

• Published in: Leukemia & lymphoma 2009-01, Vol.50 (8), p.1261-1268
• Main Authors: Zent, Clive S., Ding, Wei, Reinalda, Megan S., Schwager, Susan M., Hoyer, James D., Bowen, Deborah A., Jelinek, Diane F., Tschumper, Renee C., Call, Timothy G., Shanafelt, Tait D., Kay, Neil E., Slager, Susan L.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Anemia, Hemolytic, Autoimmune - diagnosis; Anemia, Hemolytic, Autoimmune - epidemiology; Anemia, Hemolytic, Autoimmune - etiology; Anemia, Hemolytic, Autoimmune - therapy; autoimmune hemolytic anemia; Blood Component Transfusion; Chronic lymphocytic leukemia; Cohort Studies; Combined Modality Therapy; cytopenia; Female; Follow-Up Studies; Humans; immune thrombocytopenia; Immunosuppressive Agents - therapeutic use; Leukemia, Lymphocytic, Chronic, B-Cell - complications; Leukemia, Lymphocytic, Chronic, B-Cell - diagnosis; Male; Middle Aged; Neutropenia - epidemiology; Neutropenia - etiology; Neutropenia - immunology; pure red blood cell aplasia; Purpura, Thrombocytopenic, Idiopathic - diagnosis; Purpura, Thrombocytopenic, Idiopathic - epidemiology; Purpura, Thrombocytopenic, Idiopathic - etiology; Purpura, Thrombocytopenic, Idiopathic - therapy; Red-Cell Aplasia, Pure - diagnosis; Red-Cell Aplasia, Pure - epidemiology; Red-Cell Aplasia, Pure - etiology; Red-Cell Aplasia, Pure - therapy; Retrospective Studies; Sensitivity and Specificity; Splenectomy; Survival Analysis; Treatment Outcome
• ISSN: 1042-8194; 1029-2403
• DOI: 10.1080/10428190903026492

Improved medical care could have altered the clinical presentation and survival of patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) complicated by autoimmune disease cytopenia (AID cytopenia). We reviewed the clinical characteristics, treatment, and outcome of AID cytopenia that was diagnosed in 75 (4.3%) of 1750 patients with CLL seen at a single institution over 10 years. When compared with the historical reported data, our study shows a lower rate of autoimmune hemolytic anemia (2.3%), and similar rates of immune thrombocytopenia (2.0%), and pure red blood cell aplasia (0.5%). AID cytopenia occurred at all stages of CLL, responded well to treatment, did not alter overall survival, and contributed to death in only 6 (12%) patients. We propose that more sensitive and accurate diagnostic methods for CLL have decreased the perceived prevalence of AID cytopenia and that improvements in management could have increased the survival of these patients.