Abstract
Autoimmune phenomena are frequently observed in patients with chronic lymphocytic leukemia (CLL) and are mainly attributable to underlying dysfunctions of the immune system. Autoimmune cytopenias (AIC) affect 4–7% of patients with CLL and mainly consist of autoimmune hemolytic anemia and immune thrombocytopenia. Although less common, non-hematological autoimmune manifestations have also been reported. Treatment of CLL associated AIC should be primarily directed against the autoimmune phenomenon, and CLL specific therapy should be reserved to refractory cases or patients with additional signs of disease progression. New targeted drugs (ibrutinib, idelalisib and venetoclax) recently entered the therapeutic armamentarium of CLL, showing excellent results in terms of efficacy and became an alternative option to standard chemo-immunotherapy for the management of CLL associated AIC. However, the possible role of these drugs in inducing or exacerbating autoimmune phenomena still needs to be elucidated. In this article, we review currently available data concerning autoimmune phenomena in patients with CLL, particularly focusing on patients treated with ibrutinib, idelalisib, or venetoclax, and we discuss the possible role of these agents in the management of AIC.
Highlights
Chronic lymphocytic leukemia (CLL) represents approximately 20% of all hematological diagnoses, with more than 20,000 new expected cases in the U.S in 2019 [1]
The variability of incidence reported among different studies reflects the heterogeneity of the analyzed cohorts, and the diagnostic challenges and the inconsistency of the criteria that were used to define Autoimmune cytopenias (AIC)
Data suggest that AIC tend to associate with an advanced stage of disease and adverse prognostic factors in CLL, but an impact on overall survival has not been definitively demonstrated
Summary
Chronic lymphocytic leukemia (CLL) represents approximately 20% of all hematological diagnoses, with more than 20,000 new expected cases in the U.S in 2019 [1]. It is well known that patients affected by CLL present a profound immune dysregulation, which determines an increased risk of secondary malignancies and opportunistic infections, and favors the occurrence of autoimmune complications, affecting the course and management of the disease [6,7,8,9,10]. Autoimmune complications have been reported in up to a quarter of CLL patients [11,12] Among these events, the most significant manifestations are autoimmune cytopenias (AIC), in which autoimmunity preferentially targets blood cells, while non-hematological autoimmunity is undoubtedly less frequent. In recent years, the availability of targeted drugs that have dramatically changed the overall prognosis of patients with CLL arguably had an impact on the management of autoimmune phenomena [5]. The aim of this article is to review available data regarding autoimmune phenomena in CLL, with a special focus on the setting of patients treated with currently approved targeted drugs and on the role of these agents in the management of autoimmune complications
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