Autoimmunity and Immune-Dysregulation in Hematological Diseases

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Immunology and Immunotherapy".

Deadline for manuscript submissions: closed (15 August 2023) | Viewed by 3203

Special Issue Editors


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Guest Editor
Department of Pathology, School of Medicine, Case Western Reserve University, Cleveland, OH, USA
Interests: cryptococcus neoformans; transgenic mice; platelet transfusion; apheresis; genetics; platelets; antibodies; cells; blood; hematopoiesis
Special Issues, Collections and Topics in MDPI journals

E-Mail Website
Guest Editor
Department of Pathology, School of Medicine, Case Western Reserve University, Cleveland, OH, USA
Interests: hematology; hemostasis and coagulation; apheresis and stem cell therapy

Special Issue Information

Dear Colleagues,

Immunological diseases require prompt recognition and testing using diagnostic algorithms that minimize the possibility of long-term sequelae and/or complications. These autoimmune diseases can be experienced due to the generation of cell-mediated or antibody-mediated responses to self-antigens in most tissue systems. Indeed, the formation of autoantibodies mediating pathological presentations in a myriad of diseases requiring initiation and, in many cases, maintenance of chronic immunosuppression exemplifies some of the challenges facing clinicians across disciplines. Hematological diseases caused by formation of such autoantibodies can present with anemia, thrombocytopenia, and hypercoagulability among other features that, depending on the targeted antigen, require specific therapeutics to be initiated. Furthermore, autoimmune diseases have been associated with an elevated risk of developing hematological malignancies, particularly lymphoproliferative tumors.

Thus, the goal of this Special Issue is to examine autoimmunity in hematological disease processes mediated by autoantibody formation and/or immune cell dysfunction. Original submissions describing new developments in the pathophysiology, diagnosis, and therapeutics of hematological diseases with autoimmune components are encouraged. Submissions describing current insights regarding underlying pathophysiological mechanisms, new biologicals or new ways of using existing agents, and reviews are also welcome. Case reports will be considered if the description involves significant novel findings to merit publication or if they include a thorough review of the covered topic.

Dr. Robert Maitta
Dr. Xiangrong He
Guest Editors

Manuscript Submission Information

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Keywords

  • autoimmunity
  • hematological
  • autoantibody
  • T cell
  • immune dysregulation

Published Papers (2 papers)

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Research

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12 pages, 960 KiB  
Article
The Effects of Biologics on Hematologic Malignancy Development in Patients with Ankylosing Spondylitis, Psoriasis, or Psoriatic Arthritis: A National Cohort Study
by Chia-Jung Tsai, Yu-Chih Lin, Chung-Yu Chen, Chih-Hsing Hung and Yi-Ching Lin
Biomedicines 2023, 11(9), 2510; https://doi.org/10.3390/biomedicines11092510 - 11 Sep 2023
Cited by 1 | Viewed by 1472
Abstract
Biologics are used for ankylosing spondylitis (AS), psoriasis, and psoriatic arthritis (PsA) treatment. The association between biologics and the development of hematologic malignancies is controversial, and data on patients with AS, psoriasis, and PsA are scarce. This retrospective cohort study used data from [...] Read more.
Biologics are used for ankylosing spondylitis (AS), psoriasis, and psoriatic arthritis (PsA) treatment. The association between biologics and the development of hematologic malignancies is controversial, and data on patients with AS, psoriasis, and PsA are scarce. This retrospective cohort study used data from 2010 to 2020 from Taiwan’s National Health Insurance Research Database (NHIRD). Patients with AS, psoriasis, and PsA were divided into a biologics and non biologics group after 1:10 propensity score matching. The hematologic malignancy incidences and the time-/dose-dependent effects on biologics were analyzed by Poisson regression to evaluate the incidence rate ratio (IRR). Of the 4157 biologics users and 38,399 non biologics users included in the study, 10 and 72 persons developed hematologic malignancies, respectively. Biologics only significantly increased the risk of hematologic malignancies in non-Hodgkin’s lymphoma (IRR: 2.48, 95% confidence interval (CI): 1.28–4.80). Different treatment patterns, types of biologics prescribed, cumulative defined daily doses, comorbidities, and comedications did not significantly affect hematologic malignancy development. A significantly increased risk was observed when biologics had been prescribed for 1–2 years (IRR: 2.95, 95% CI: 1.14–7.67). Clinical professionals should be aware of a patients’ risk of hematologic malignancies during the second year of biologic treatment. Full article
(This article belongs to the Special Issue Autoimmunity and Immune-Dysregulation in Hematological Diseases)
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11 pages, 743 KiB  
Review
Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura
by Hallie H. Dolin and Robert W. Maitta
Biomedicines 2024, 12(3), 621; https://doi.org/10.3390/biomedicines12030621 - 11 Mar 2024
Cited by 1 | Viewed by 1214
Abstract
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, but potentially disabling or even deadly, thrombotic microangiopathy with a well-studied mechanism of ADAMTS13 deficiency or dysfunction. While established treatments are largely effective, the standard ADAMTS13 testing required to definitively diagnose TTP may cause delays in [...] Read more.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, but potentially disabling or even deadly, thrombotic microangiopathy with a well-studied mechanism of ADAMTS13 deficiency or dysfunction. While established treatments are largely effective, the standard ADAMTS13 testing required to definitively diagnose TTP may cause delays in diagnosis and treatment, highlighting the need for rapid and effective diagnostic methods. Additionally, the heterogeneous presentation and varied inciting events of TTP suggest more variation in its mechanism than previously thought, implying three potential pathways rather than the accepted two. The recent discovery of ADAMTS13 conformation as a potential contributor to TTP in addition to the proposal of using the absolute immature platelet count (A-IPC) as a biomarker, present novel areas for monitoring and treatment. A-IPC in particular may serve as a more rapid and accurate diagnostic test to distinguish TTP from non-TTP TMAs and to monitor treatment response and relapse. These considerations highlight the need to further study TTP in order to improve best practices and patient care. Full article
(This article belongs to the Special Issue Autoimmunity and Immune-Dysregulation in Hematological Diseases)
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