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Autoimmunity and Novel Therapies in Immune-Mediated Thrombocytopenia

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Autoimmunity and Novel Therapies in Immune-Mediated Thrombocytopenia Autoimmunity and Novel Therapies in Immune-Mediated Thrombocytopenia Dana Yehudai,a Elias Toubi,b Yehuda Shoenfeld,c and Zahava Vadaszb Immune-mediated thrombocytopenic purpura (ITP) is recognized as a cell-specific autoimmune disorder, yet, multifactorial in origin. The development of thrombocytopenia is well proven to be mediated by both humoral (anti-platelet antibodies) and cellular (T-cell) mediated mechanisms. In some cases other autoantibodies are also induced, eg, antinuclear antibody (ANA), anti-dsDNA, and anti-cardiolipin, in addition to anti-platelet antibodies. The persistance of these autoantibodies during the course of ITP could herald future development of another autoimmune disease, eg, systemic lupus erythematosus (SLE) or anti-phospholipid syndrome (APS). Due to the better understanding of the pathophysiology of ITP, new novel therapies were introduced aiming to achieve long-lasting remissions. In this review we will focus on the autoimmune nature of the disease and on some of the mechanisms of action of these new therapies. Semin Hematol 50:S100–S108. C 2013 Published by Elsevier Inc. Open access under CC BY-NC-ND license. mmune thrombocytopenic purpura (ITP) is con- they require various therapeutic interventions, sidered a classical autoimmune disorder charac- including splenectomy and other novel therapies terized as the occurrence of thrombocytopenia such as rituximab and syk inhibitors.5 Though many I 3 (platelet count o 100 Â 10 /mL. Platelet destruc- reports have pointed to the notion that ITP is tion by the spleen and morphologic abnormalities predominantly a disorder of young women, some of megakaryocytes followed by their altered matura- have shown a progressive increase in incidence with tion are suggested to be the main pathogenic age. However, the demographics of ITP probably abnormalities in ITP. Later, ITP was well defined to depend on regional differences due to different be the result of a plasma factor proven as specific predisposing infections, and the early diagnosis or anti-platelet antibodies and finally defining ITP as an the better treatments of B-cell neoplasms.6 autoimmune disorder.1–3 Recent literature accepts When ITP is isolated and no evidence for systemic the classification of three phases of ITP: (1) the new- diseases is proven, primary ITP is then defined; onset phase that occurs within 3 months from primary ITP represents 80% of all cases. However, diagnosis; (2) the persistent phase, lasting between secondary ITP is defined when various well-proven 3 and 12 months from diagnosis; and (3) the chronic infectious or autoimmune diseases are responsible phase, defined as lasting more than 12 months.4 for the development of thrombocytopenia. These Patients who are diagnosed with ITP who repeti- include viral infections such as hepatitis C virus tively bleed considered to have ‘‘severe’’ ITP and (HCV), cytomegalovirus (CMV), and Helicobacter pylori, and also cases of ITP that were recognized 7,8 aDepartment of Internal Medicine A, Bnai-Zion Medical Center. The as a result of vaccination. Many viral particles such Rappaport Faculty of Medicine, Technion-Haifa, Israel. as HCV were described to polyclonally activate B b Division of Allergy and Clinical Immunology, Bnai-Zion Medical cells by binding CD81 (part of the B cell co-receptor) Center, The Rappaport Faculty of Medicine, Technion-Haifa, Israel. cZabludowicz Center for Autoimmune Diseases, Sheba Medical or other receptors in the case of CMV, predisposing Center, Sakler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, to the expansion of autoreactive B cells, the produc- Israel. tion of anti-viral antibodies that cross-react with Publication of this article was supported by the International Coop- membrane platelet antigens. In some studies the erative ITP Study Group (ICIS). Conflicts of interest: none. presence of multiple autoantibodies was docu- Address correspondence to Yehuda Shoenfeld, MD, FRCP, Zabludowicz mented and suggested to be the result of epitope Center for Autoimmune Diseases, Sheba Medical Center, Tel- spreading.9,10 A wide spectrum of autoimmune dis- Aviv University, Tel-Aviv, Israel. E-mail: [email protected] 0037-1963 eases such as systemic lupus erythematosus (SLE), & 2013 Published by Elsevier Inc. Open access under CC BY-NC-ND license. rheumatoid arthritis (RA), antiphospholipid syn- http://dx.doi.org/10.1053/j.seminhematol.2013.03.015 drome (APS), and immune deficiencies like common S100 Seminars in Hematology, Vol 50, No 1, Suppl 1, January 2013, pp S100–S108 Autoimmunity and novel therapies S101 variable immune deficiency (CVID) are among the and B cells. The T-cell response was inhibited by list of diseases reported to be responsible for secon- anti-FcgRI antibody. Thus, splenic macrophages that dary ITP. Chronic lymphocytic leukemia (CLL) and take up opsonized platelets via FcgRI are major APCs various lymphomas are additional diseases in the list for cryptic glycoprotein peptides, and are central to of all the above, all of which do not exceed 20%.11–13 the maintenance of anti-platelet autoantibody pro- duction in ITP patients.17 In another study, it was reported that macrophages activated by C-reactive THE DIVERSITY OF IMMUNE-MEDIATED protein (CRP) transfer suppression of ITP. Suppres- ABERRATIONS IN ITP sion of ITP by CRP-treated splenocytes required Early studies showed already that when isolated FcgRI on the donor cell and FcgRIIb in the recipient platelets from ITP patients were incubated with mice. These findings suggest that CRP generates autologous lymphocytes, they induced their trans- suppressive macrophages through FcgRI, which formation and resulted in increased interleukin (IL)-2 then act through an FcgRIIb-dependent pathway in productions. The responsive cells were proven to be the recipient to decrease platelet clearance.18 CD4þ T cells and were shown to react specifically against modified glycoprotein (GP)IIb-IIIa on acti- EFFECTOR VERSUS T-REGULATORY CELLS vated platelets. In addition to CD4þ T-cell activation, it was also noticed that the stimulation of ITP B cells IN ITP resulted in the in vitro production of anti-platelet The immune response to glycoproteins in ITP is antibodies. However, the same stimulation of normal usually modulated by CD4þ effector cells and CD8þ B cells did not induce such autoantibodies. Of cytotoxic cells. In thymus, T cells lose either the interest is that the depletion of CD19þ B cells was CD4 or CD8 antigens and are released as either followed by a complete disappearance of these CD4þ effector or CD8þ cytotoxic T cells (CTLs). antibodies. Using antigen-specific assays such as Some self-reactive T cells survive thymic depletion, immunoprecipitation, immunoblot, and antigen cap- and persist in peripheral blood as autoreactive and ture techniques, these autoantibodies were shown mediate autoimmunity. Normally, autoreactive T to be highly specific, and to recognize antigens that cells are controlled by many peripheral self- are derived from either a single or multiple glyco- tolerance mechanisms such as altered co- proteins. The most identified autoantibodies in ITP stimulatory molecules and or failure of T-regulatory are those against GPIIb-IIIa and/or GPIb-IX, but there cell (Treg) function. When self-tolerance fails, CD4þ have also been reports of autoantibodies against T-effector cells react with specific antigens pre- GPIa-IIa and GPIV.14,15 When assessed positively, sented by MHCII molecules in association with a these autoantibodies are considered a diagnostic proper expression of co-stimulatory molecules. hallmark of ITP with a sensitivity of 49%–66% and When T cells of ITP patients were stimulated by a specificity of 78%–93%; however, negative results platelet antigens, one could notice increased pro- do not rule out this diagnosis.16 The most likely duction of both Th1 and Th2 cytokines. Th1 cells explanation for negative assays is that other mecha- produce IL-2, interferons, and tumor necrosis factor nisms are involved in the pathophysiology of ITP (TNF), whereas Th2 cells produced IL-4, IL-13, and that do not involve anti-platelet antibodies, such as IL-10, which are considered important in inhibiting T-cell–dependent platelet destruction or inhibition cell-mediated immune responses. The role of Th17 of platelet production. (known to be associated with many immune- The persistent destruction of platelets by macro- mediated diseases) in the pathogenesis of chronic phages induces the continuous processing and pre- ITP was assessed. Higher levels of IL-17A and Th17- sentation of the above membrane glycoproteins by related cytokines, and an increased percentage of IL- antigen-presenting cells (APCs), which is a crucial 17A producing CD4þ and neutrophils, were step for the generation of pathogenic anti-platelet observed in ITP patients.19–21 In addition, T- antibodies. Aiming to better understand this process, effector cells are increasingly recruited into bone GPIIb/GPIIIa-reactive T-cell lines generated from ITP marrow and other involved organs in ITP. To patients were cultured with autologous freshly iso- determine the reason for this, expression of chemo- lated splenic macrophages, B cells, or dendritic cells kine receptors such as CX3CRI and CXCR4 was (DCs). Macrophages induced the proliferation of analyzed in the bone marrow of ITP patients. Here, GPIIb/IIIa-reactive T-cell lines without an exogenous T-cell surface expression of these chemokine recep- antigen, but B cells and DCs required glycoprotein tors was increased in patients compared with con- peptides to stimulate the T cells. Cultured macro- trols. Furthermore, the number of CD3þ T cells in phages that captured opsonized platelets promoted bone marrow, but not in blood, along with increased anti-GPIIb/GPIIIa antibody production in mixed cul- Fas expression was also found, emphasizing the tures of autologous GPIIb/GPIIIa-reactive T-cell lines importance of cellular immunity in ITP22 (Figure 1). S102 D. Yehudai et al. in comparison to healthy individuals. The number of Tregs was significantly lower in ITP patients in the severe phase, and in patients positive for anti-GPIIb/ IIIa antibody.
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