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Hematological Immune Cytopenias and Anti-Phospholipid Antibodies
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
Munther A. Khamashta, Samuel J. Machin
A small subgroup of patients with autoimmune hemolytic anemia or a positive DCT develop thrombocytopenia with raised platelet autoantibodies. This is widely known as Evans’ syndrome. In a recent study of 12 patients with Evans’ syndrome and SLE, 10 patients had evidence of aPL.27 Similarly, in 70 patients with the “primary” antiphospholipid syndrome 10% were described as having Evans’ syndrome.4 Infection preceded the development of cytopenic episodes in 50% of the SLE patient group suggesting that the infection triggered the development of aPL and the subsequent red cell and platelet immune destruction.
Hieh-Dose Immunosuppressive Chemotherapy with Autologous Stem Cell Support for Chronic Autoimmune Thrombocytopenia
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Richard D. Huhn, Patrick F. Fogarty, Ryotaro Nakamura, Cynthia E. Dunbar
The treatment schema is shown in Figure 1. Fourteen patients, aged 17 to 52 years old with disease durations of 6 months to 40 years, were evaluable for safety and therapeutic response as of November, 2001 (the study continues to enroll patients at the time of this writing). All patients had failed to obtain durable responses or had relapsed after standard treatments (corticosteroids, splenectomy and intravenous immune globulin) and had unsuccessfully undergone one or more second-line therapies. All patients had significant hemostatic complications jeopardizing life, health or daily activities. Bone marrow examinations documented normal or increased numbers of megakaryocytes; absence of morphologic evidence of marrow failure, dysplasia and neoplastic or infiltrative diseases; and absence of detectable cytogenetic abnormalities. Five patients had concurrent autoimmune hemolytic anemia (Evans syndrome).
Immunohematology
Published in Gabriel Virella, Medical Immunology, 2019
Gabriel Virella, Armand Glassman
AIHDs are the result of inherent immunological mechanisms encumbering or eliminating one or more of the elements of the hematopoietic process. Immune destruction of or reduction of functioning red blood cells results in autoimmune hemolytic anemia (AIHA). Immune impairment and reduction in the number of platelets results in immune thrombocytopenia (ITP). Autoimmune neutropenia (ANP) may involve any of the myeloproliferative or lymphoproliferative cell lines including stem cells. Multiple cell lines may be involved as in Evans Syndrome.
Approaches to patients with variants in RAG genes: from diagnosis to timely treatment
Published in Expert Review of Clinical Immunology, 2019
Adeeb A. Bulkhi, Joseph F. Dasso, Catharina Schuetz, Jolan E. Walter
In the case of AIC, first-line therapy may include corticosteroids for AIHA, high-dose IVIG and corticosteroids for ITP, and granulocyte-colony stimulating factor (G-CSF) for AN. In a large case series, AIC were dominated by AIHA, followed by ITP and AN, respectively. AIC were refractory to IVIG, steroids, and rituximab in the majority of cases (64.7%, 73.7%, and 71.4% for AIHA, ITP, and AN, respectively). The occurrence of Evans syndrome was associated with lack of response to first-line therapies [22]. Onset at older age, single-lineage involvement, and less severe cytopenia may predict a favorable response to treatment [22]. Other autoimmune disorders and single or multiorgan granulomas have been managed with high-dose IVIG and corticosteroids with limited response. Anti-TNF, e.g. infliximab, has shown a moderate effect in granulomatous disease (Table 3). Other second-line therapies such as cyclophosphamide, cyclosporine, anti-CD52 (alemtuzumab), adalimumab (TNFα inhibitor), rituximab (anti-CD20), and/or sirolimus have been tried and failed to achieve a sustained response in most patients.
Expression of BTK/p-BTK is different between CD5+ and CD5- B lymphocytes from Autoimmune Hemolytic Anemia/Evans syndromes
Published in Hematology, 2019
Ningning Duan, Manjun Zhao, Yi Wang, Yingying Qu, Hong Liu, Huaquan Wang, Limin Xing, Zonghong Shao
Autoimmune hemolytic anemia (AIHA) is an immune disease characterized by antibodies directed against autologous red blood cells (RBCs). Evans syndrome (ES) is accompanied with thrombocytopenia because of autoantibodies with platelets. Both AIHA and ES have a similar pathogenesis that features activated B lymphocytes. The survival, activation, and differentiation of B cells are regulated by the B cell antigen receptor (BCR). Bruton’s tyrosine kinase (BTK) is a member of the Tec family of kinases. BTK is a key component of the precursor-BCR signaling pathway. BTK directly promotes the production of messenger molecules, abnormally activates the BCR signaling pathway, transforms B cells into self-reactive B cells, and causes autoimmune disease [1]. The increased activity of BTK has been confirmed in systemic lupus erythematous [2] and rheumatoid arthritis [3] and has been associated with the severity of these diseases. It is conceivable that BTK plays a role in the pathogenesis of AIHA/ES.
Progress on the efficacy and potential mechanisms of rapamycin in the treatment of immune thrombocytopenia
Published in Hematology, 2022
Dan Wang, Kaniel Cassady, Zhongmin Zou, Xi Zhang, Yimei Feng
Evans Syndrome (ES) is a rare disorder that was initially described as the presence of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia with unknown etiology [33]. In one study of rapamycin in ES, five ES patients were recruited, and four had a response to rapamycin, with half of them achieving CR and the other two achieving an asymptomatic partial response [29]. Li et al. [30] recruited 12 ES patients and treated with rapamycin, and the ORR at 12 months was 91.7% with a CR rate of 33.3%.