Geoepidemiology and autoimmune manifestations of lymphoproliferative disorders

The pathogenesis of lymphoproliferative disorders represents an underlying dysfunction in lymphocyte development and homeostasis. This typically manifests with the accumulation of lymphocytes in the bone marrow, peripheral blood or lymph nodes and spleen. It has been hypothesized that the mechanism for elimination of dysfunctional and autoreactive lymphocytes is defective and contributes to the pathogenesis of these disorders. Based on this hypothesis, it would not be surprising to find an increased incidence of autoimmune manifestations that are associated with these disorders. Autoimmune paraneoplastic syndromes (PNS) are often the result of a dysfunctional immune response, which is related to an underlying malignant process. While all the PNS that are associated with lymphoproliferative disorders do not have an autoimmune basis, many do. Here we review the literature of PNS associated with lymphoproliferative disorders. A concise overview of each syndrome is provided with a focus on clinical manifestations, diagnostics, pathophysiology, and treatment. While the treatment is typically directed at managing the underlying lymphoproliferative process, and can be assumed to be case throughout this review, there are exceptions that are described within the context of each disorder.