Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas

Abstract

We analyzed 108 cases of non-CLL non-Hodgkin lymphoma (NHL) associated with autoimmune hemolytic anemia (AIHA) (+/- pure red cell aplasia (PRCA)) or Evans' syndrome. The analysis was based on cases reported in the literature, which were retrieved by means of Pubmed and Medline searches and of an original series of 121 patients with NHL as well as reference lists of papers in the field. The number of cases in various NHL subtypes was small (n = 6-25). Nevertheless, interesting and sometimes unexpected differences in sex prevalence, temporal relationship between onset of lymphoma and AIHA, stage of lymphoma, relative frequency of warm antibody-AIHA (WA-AIHA) and cold antibody (CA-AIHA), association with PRCA and response of AIHA to treatments were noted for various lymphoma entities. WA-AIHA was more frequent in B-cell lymphomas, while CA-AIHA and PRCA predominantly occurred in T-cell lymphomas. Anti-lymphoma treatment seemed to be more effective against AIHA than conventional therapy with steroids or immunoglobulin. Although generated by a literature survey, this compilation of data indicates a complex relation of lymphoma and AIHA and warrants more attention and specific studies.

Autoimmune thrombocytopenia in non-Hodgkin's lymphomas

“…This is about one third of the number of reported cases of AIHA and NHL (n= 86). 4 While cases of AIHA and ITP were reported in a similar relative frequency in MZL, HCL, myeloma and DLCL there was a large difference in follicular lymphomas, T-cell lymphomas and lymphoplasmacytic lymphoma ( Table 1). The dominant types of lymphomas in rheumatoid arthritis are DLCL and lymphoplasmacytic lymphoma, 1,53 MZL and MALT lymphoma are more common in Sjögren's syndrome and Hashimoto thyreoiditis.…”

“…1-3 Among these phenomena, hematologic autoimmune diseases are a special subgroup and include autoimmune hemolytic anemia (AIHA), 4 autoimmune thrombocytopenia (ITP), Evans's syndrome, antibodies to clotting factors, 5 lupus anticoagulants 6 and antibodies to C1 esterase inhibitor. 7 Less is known about the occurrence, prognosis and treatment of ITP in NHL.…”

Relationship between Autoimmune Diseases and Imbalances in Helper/Suppressor T-cell Populations after Rituximab-containing Chemotherapyfor Non-Hodgkin Lymphoma

“…The underlying mechanisms may vary between entities and patients. The current hypothesis for chronic lymphocytic lymphoma is that polyclonal antibodies cause AIHA (16). The pathogenesis of AIHA or all autoimmune phenomena that complicate the course of NHL remain a matter of considerable controversy.…”

“…Suppressor T cells are important regulators of immune responses to self antigens and defective suppressor T-cell function may allow the emergence of autoreactive lymphocytes with consequent autoantibody production. Immune dysregulation may also be caused by damage to thymic function as a result of irradiation and chemotherapy [16,17]. It has been shown in experimental models that various organ-specific autoimmune diseases may develop by depleting T-cell subsets including CD4 and CD8 cells [18].…”

Splenic marginal zone lymphoma with Evans’ syndrome, autoimmunity, and peripheral gamma/delta T cells

“…Autoimmune phenomena like Evans' syndrome, lupus anticoagulants, autoimmune thrombocytopenia, and autoimmune hemolytic anemias are frequently associated with lymphomas [4,5]. Recently, systemic lupus erythematosus (SLE) was associated with an increased risk of diffuse large B cell and marginal zone lymphomas [3].…”