Tγ Cell Deficiency in Idiopathic Thrombocytopenic Purpura (ITP) (original) (raw)
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Functional properties of lymphocytes in idiopathic thrombocytopenic purpura
Human Immunology, 2001
Idiopathic or immune thrombocytopenic purpura (ITP) is characterized by antibody-mediated destruction of platelets. The etiology is unknown. We postulated that increased autoantibody production in ITP might be attributable to either increased or prolonged expression of CD40 ligand (CD40L, CD154) in T or B lymphocytes, as has been previously observed in systemic lupus erythematosus (SLE). In addition, we hypothesized that ITP is characterized by increased levels of interleukin 4 (IL-4), a prototypic Th2 cytokine which, along with CD40 ligation, is required for B cell differentiation and production of several IgG subclasses. Cell surface CD154 expression was measured in freshly-isolated and in vitroactivated peripheral blood lymphocytes of sixteen ITP patients and eight healthy volunteers. Plasma levels of IL-4 and the prototypic Th1 cytokine interferon-gamma (IFN␥) were determined. We observed that CD154 expression in unstimulated and in vitro-activated lymphocytes did not differ between ITP patients and healthy controls. Plasma levels of the Th2 cytokine IL-4 were significantly higher in the ITP patients. These studies indicate that overexpression of CD154 in lymphocytes is unlikely to be a primary pathophysiological defect in most patients with ITP. The data support that in addition to cell membrane antigens such as CD154, soluble cytokines such as IL-4 should be considered as potential targets for therapy in this disease.
Asthma Allergy Immunology
Objective: Primary Immune Thrombocytopenia (ITP, idiopathic thrombocytopenic purpura, immune thrombocytopenic purpura) is an acquired thrombocytopenia caused by anti-platelet antibodies. The diagnosis of ITP may be challenging due to the various potential causes of thrombocytopenia, some of which are overlooked. Immunodeficiencies are also a rare cause of ITP. Although autoimmunity and, therefore, ITP are a common complication in primary immunodeficiency (PI) patients, there are not many publications in the literature that examine the frequency of PI in ITP patients and the immune abnormalities in these patients. Materials and Methods: Forty-five patients with ITP (F: 37 [78.7%], M: 10 [21.3%]) were included in the study (age 42.9 ± 15.9). Results: At least one antibody deficiency was detected in 7 patients (14.9%), and following further investigations, 2 patients (4.3%) were diagnosed with CVID, 3 (6.4%) with IgG deficiency, 1 (2.1%) with selective IgA deficiency and 1 (2.1%) with possible IgM deficiency. Immunoglobulin levels were normal while at least one abnormality was detected in 20 patients (42.6%) in peripheral lymphocyte subset analyses. The most common abnormality in this patient group was a reduced percentage of CD4+ T-cells (9 patients, 45% of patients with PLS abnormalities, 19.1% of all patients). CD3+ T-cell rates in 8 patients (17.8%), CD19+ B-cell rates in 6 (12.8%), CD3-CD16+56+ natural killer cell rates in 4 (8.5%), and CD4/CD8 cell ratios in 7 (14.9%) were reduced. In addition, the CD8+ T-cell rate in 8 patients (17%) was above the reference ranges. Conclusion: Adult patients who are diagnosed with ITP may develop a variety of immunological abnormalities in addition to hypogammaglobulinemia. Therefore, clinicians should not overlook immunological evaluation in the etiological investigation of ITP and should closely monitor patients with immunological abnormalities.
The Eurasian Journal of Medicine, 2010
Objective: Immune thrombocytopenic purpura (ITP) is also known as idiopathic thrombocytopenic purpura. Increased platelet destruction and insuffi cient platelet production are both responsible for its etiopathogenesis. ITP can be diagnosed after excluding other possible causes of thrombocytopenia. Materials and Methods: One hundred forty-three cases of chronic ITP that were monitored in a hematology clinic were retrospectively evaluated. All cases received fi rst line treatment of 1 mg/kg/day prednisolone. Corticosteroid nonresponsive (CN) cases and corticosteroid-dependent (CD) cases underwent splenectomies. Results: The rate of CN/CD cases was found to be 53% (n=76). Sixtysix percent of these cases (n=50) underwent splenectomies. The ratio of non-responsive cases to relapse cases after splenectomy (SN/SR) was 30% (n=15). The total number of cases was 41, including those without splenectomy (n=26) and with SY/SR (n=15). Helicobacter pylori (Hp) eradication, immunosuppressive agents and danazol treatments were administered to patients (n=10, n=14 and n=4, respectively). Currently, 13 patients are being monitored without treatment. Fifteen patients who were non-responsive to Hp eradication treatment, immunosuppressive treatment or danazol treatment are still being monitored without any treatment. Conclusion: Optimal treatment is not available for splenectomy-resistant cases of ITP. The response rates for Hp eradication treatment, immunosuppressive treatments and anabolic agents are low. Therefore, larger studies with more patients are required using new agents, such as thrombopoietin (TPO) receptor agonists and anti-CD20 monoclonal antibodies.
Clinical and Applied Thrombosis/Hemostasis, 2010
We investigated the prevalence of antinuclear antibody (ANA), thyroid antimicrosomal (AMA) and antithyroglobulin (ATA), antigliadin (AGA) immunoglobulin G (IgG)-A, anti-endomisium (EMA) IgG-A, and tissue transglutaminase (tTG) IgG-A in 87 patients with chronic idiopathic thrombocytopenic purpura (cITP) and in 95 healthy controls. Antinuclear antibody positivity was found in 13 of 87 patients and 3 of 95 controls ( P = .007). Antithyroglobulin positivity was found in 27 of patients and in 7 of the controls (P < .001). AMA positivity was found in 20 of patients and 8 of the controls (P = 0.008). Antigliadin IgG was positive in 17 patients and 1 controls (P < .001) whereas Antigliadin IgA was positive in 9 of patients and in 1 of the controls (P = .007). Anti-endomisium (IgG and IgA were not different between both groups. Tissue transglutaminase IgG was detected in 7 of patients and in 1 of the controls (P = .029). Tissue transglutaminase IgA was detected in 5 of patients and in ...
Immune Thrombocytopenic Purpura in Review
2021
Background: Immune thrombocytopenic purpura (ITP) characterized by high risk of bleeding, this bleeding is due to 2 main factors the first is the damage of the platelets which is mediated by antibodies and also disordered platelet synthesis, all the previous characteristics identify Immune thrombocytopenic purpura (ITP) as autoimmune disease. Aim: In this review, we will look into the prevalence, pathophysiology, diagnosis and management of immune thrombocytopenic purpura. Conclusion: ITP is a serious disease that cause sever bleeding that can be life threatening in some cases, the causes of this disease are idiopathic mostly but it is classifies as autoimmune disease, the diagnosis of ITP is mainly by excluding other causes that can give the same symptoms. Treatment is classified into three steps if one fails, we move to the next one starting from corticosteroids, then splenectomy and finally the new group of medications whose mechanism, and data are not sufficient so more studies ...
…, 2009
Immune thrombocytopaenic purpura (ITP) is a clinical syndrome in which a decreased number of circulating platelets (thrombocytopaenia) manifests as a bleeding tendency, easy bruising (purpura), or extravasation of blood from capillaries into skin and mucous membranes (petechiae). Present study was done to observe the clinico-haematological features of ITP in Adults and to analyse the association of autoimmune disorders with ITP in Pakistani patients. It was a cross-sectional descriptive study conducted at Shaikh Zayed Hospital, Lahore, from 1st January 2006 to 30 th June 2007. The study included 44 adult patients of both genders diagnosed as having ITP according to WHO guidelines. Bone marrow biopsy was carried out in all patients and other causes of thrombocytopaenia were carefully excluded. Antinuclear antibodies, rheumatoid factor, HBs Ag, anti HCV, HIV were also done. The data was analysed by SPSS version 10. Results showed peak incidence in third decade with female to male ratio of 3.1:. Bleeding and bruising were common symptoms of ITP. Seven (15.9%) of 44 patients had serological evidence of systemic autoimmune disorders, i.e., SLE or RA. Platelet count was significantly lower in SLE patients than in entire cohort. It was concluded that adult ITP is predominantly seen in young females, presents with bleeding from more than 2 sites and may be associated with autoimmune disorders at the time of diagnosis.
Clinical & Experimental Immunology, 2014
Immune thrombocytopenic purpura (ITP) is acquired autoimmune disease in children characterized by the breakdown of immune tolerance. This work is designed to explore the contribution of different lymphocyte subsets in acute and chronic ITP children. Imbalance in the T helper type 1 (Th1)/Th2 cytokine secretion profile was investigated. The frequency of T (CD3 + , CD4 + , CD8 +) and B (CD19 +) lymphocytes, natural killer (NK) (CD16 + 56 +) and regulatory T (Treg) [CD4 + CD25 +high forkhead box protein 3 (FoxP3) + ] cells was investigated by flow cytometry in 35 ITP children (15 acute and 20 chronic) and 10 healthy controls. Plasma levels of Th1 cytokines [interferon (IFN-γ) and tumour necrosis factor (TNF-α)] and Th2 [interleukin (IL)-4, IL-6 and IL-10)] cytokines were measured using enzyme-linked immunosorbent assay (ELISA). The percentage of Treg (P < 0•001) and natural killer (NK) (P < 0•001) cells were significantly decreased in ITP patients compared to healthy controls. A negative correlation was reported between the percentage of Treg cells and development of acute (r = −0•737; P < 0•01) and chronic (r = −0•515; P < 0•01) disease. All evaluated cytokines (IFN-γ, TNF-α, IL-4, IL-6 and IL-10) were elevated significantly in ITP patients (P < 0•001, P < 0•05, P < 0•05, P < 0•05 and P < 0•001, respectively) compared to controls. In conclusion, our data shed some light on the fundamental role of immune cells and their related cytokines in ITP patients. The loss of tolerance in ITP may contribute to the dysfunction of Tregs. Understanding the role of T cell subsets will permit a better control of autoimmunity through manipulation of their cytokine network.
Annals of Hematology, 1996
In chronic idiopathic thrombocytopenic purpura (ITP) platelet destruction is caused by antibodies directed against plate][et membrane glycoproteins (GP), and the predominant autoantigens are known to be GPIb/IX and GPIIb/IIIa. In a recent study we reported that these antibodies frequently had a restricted light chain phenotype, thereby supporting a clonal origin. Similar findings and the presence of clonal B-cell populations in immune thrombocytopenias have been reported by others. In the present study we further explored the hypothesis of clonal B-cell expansions in chronic ITP. Twenty patients with chronic ITP were investigated. Antibodies were detected with an ELISA (MAIPA) specific for GPIb/IX and GPIIb/IIIa; circulating clonal B lymphocytes were assessed by flow-cytometric (FACS) clonal-excess analysis and by analyzing Ig-gene rearrangements (CDR3) with the PCR technique. Nine patients displayed a GP-specific antibody restricted to either kappa or lambda phenotype. However, FACS analysis and Ig-gene rearrangement studies did not disclose any circulating clonal B-cell population. Considering the sensitivity of the FACS analysis and Ig-gene rearrangement for detection of clonal B-cell populations, the hypothesis of clonally derived autoantibodies in ITP is still valid. Most probably, the clonal B-cell expansion responsible for the production of autoantibodies in ITP, if present, is below the detection limit for the techniques employed.