Autoimmune Cytopenias in Chronic Lymphocytic Leukemia

Abstract

The clinical course of chronic lymphocytic leukemia (CLL) may be complicated at any time by autoimmune phenomena.The most common ones are hematologic disorders, such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Pure red cell aplasia (PRCA) and autoimmune agranulocytosis (AG) are, indeed, more rarely seen. However, they are probably underestimated due to the possible misleading presence of cytopenias secondary to leukemic bone marrow involvement or to chemotherapy cytotoxicity. The source of autoantibodies is still uncertain, despite the most convincing data are in favor of the involvement of resting normal B-cells. In general, excluding the specific treatment of underlying CLL, the managementof these complications is not different from that of idiopathic autoimmune cytopenias or of those associated to other causes. Among different therapeutic approaches, monoclonal antibody rituximab, given alone or in combination, has shown to be very effective.

Immune Cytopenias in Patients With Chronic Lymphocytic Leukemia (Peculiarities, Prognostic Markers)

“…Older age, male gender correlated with the development of ITP [14]. It was reported that IC is more common in patients with unmutated IGHV status [23,24]. It is considered that a high level of β2-microglobulin [24,25], increased CD 38 [22,25], high ZAP70 [24,26,27,28] may be antecedents of IC.…”

Chronic Lymphocytic Leukemia Presenting as a Subcortical Watershed Infarct

“…Cytokines production from CLL cells can result in inhibition of normal erythropoiesis or megakaryopoiesis, leading to development of AIHA or ITP [14]. CLL cells can rarely act as effector cells, secreting a pathological monoclonal antibody [15]. CLL cells may also be stimulated through their polyreactive B-cell receptor (BCR) that recognizes autoantigens [15].…”

“…CLL cells can rarely act as effector cells, secreting a pathological monoclonal antibody [15]. CLL cells may also be stimulated through their polyreactive B-cell receptor (BCR) that recognizes autoantigens [15].…”

Specific Associations Between Clinical Signs, Immune Cells, Disease Genetic Background and Burden in a Group of Patients with B-Cell Chronic Lymphocytic Leukemia

“…The expansion of these B-cells is associated with severe defects of T-cell responses and homeostasis. Accordingly, we and others have documented proofs of a systemic immune dysregulation in CLL patients, including increased circulating T cell numbers with disease stage, imbalanced Th1/Th2 profiles, increased circulating numbers of regulatory phenotypes, and reduced B-cell capacity for antigen presentation, among others, which may lead to immunodeficiency or autoimmune manifestations related to the disease [1,2]. Additionally, hepato-and splenomegaly and the occurrence of enlarged lymph nodes (LN) with various localizations are considered the most frequent clinical manifestations in B-CLL patients [3].…”