Autoimmune cytopenias in chronic lymphocytic leukemia at disease presentation in the modern treatment era: is stage C always stage C?

Abstract

Anemia and thrombocytopenia at chronic lymphocytic leukemia (CLL) presentation have long been considered to be predictive of a poor prognosis, irrespective of the cause of cytopenia, yielding an advanced stage of the disease. We identified 86 patients with CLL who were diagnosed after year 2000 with Binet C disease at first presentation. Cytopenia was considered related to autoimmune conditions in 27 (31.3%; stage C “immune”) or secondary to bone marrow failure in 59 (68.6%; stage C “infiltrative”). No difference in clinical characteristics, mutational status, cytogenetics, TP53 and NOTCH1 mutations was observed between stage C “immune” and “infiltrative.” Patients with stage C “immune” had a trend toward a better overall survival than patients with stage C “infiltrative” (median 74 vs. 63 months), but the difference was not statistically significant (p = 0.30). This difference was not modified by adjustment for CLL tumor burden at presentation, and survival of stage C “immune” patients was significantly inferior compared to an unselected cohort of patients with stage A, but similar to stage B. Our findings suggest that autoimmune cytopenias at CLL diagnosis have a negative impact on patient outcome.

Keywords::

• Chronic lymphocytic leukemia
• Binet C
• Rai stage 4
• autoimmune disease
• autoimmune hemolytic anemia
• immune thrombocytopenia

Acknowledgements

This study was supported in part by grants from AViLL/AIL (Associazione Vicentina per le Leucemie, i Linfomi e il Mieloma/Associazione Italiana Leucemie) (Vicenza, Italy) and the Hematology Project Foundation (HPF, Fondazione Progetto Ematologia, Vicenza, Italy); Regione Veneto, Italy, through the “Ricerca Sanitaria Finalizzata 2006” program; and Associazione Italiana Ricerca sul Cancro (AIRC) (IG4569) to A.N., and Assistenza Studio Malati Ematologici (ASME-Milano) and Associazione Italiana Leucemie Milano to A.C.

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