Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Primary Immunodeficiency and Thrombocytopenia Publisher Pubmed



Mohtashami M1, 2 ; Razavi A2, 3, 4 ; Abolhassani H2, 5 ; Aghamohammadi A2 ; Yazdani R2
Authors
Show Affiliations
Authors Affiliations
  1. 1. Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran
  2. 2. Research Center for Immunodeficiencies, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Department of Animal Biology, Faculty of Biology Sciences, University of Kharazmi, Tehran, Iran
  4. 4. Cancer Immunology Project (CIP), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  5. 5. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden

Source: International Reviews of Immunology Published:2022


Abstract

Primary immunodeficiency (PID) or Inborn errors of immunity (IEI) refers to a heterogeneous group of disorders characterized by immune system impairment. Although patients with IEI manifest highly variable symptoms, the most common clinical manifestations are recurrent infections, autoimmunity and malignancies. Some patients present hematological abnormality including thrombocytopenia due to different pathogenic mechanisms. This review focuses on primary and secondary thrombocytopenia as a complication, which can occur in IEI. Based on the International Union of Immunological Societies phenotypic classification for IEI, the several innate and adaptive immunodeficiency disorders can lead to thrombocytopenia. This review, for the first time, describes manifestation, mechanism and therapeutic modalities for thrombocytopenia in different classes of IEI. © 2021 Taylor & Francis Group, LLC.
Related Docs
View other Related Docs
1. Inborn Errors of Immunity and Cancers, Cancer Immunology: A Translational Medicine Context# Second Edition (2020)
2. Primary Immunodeficiencies and Cancers, Cancer Immunology: A Translational Medicine Context (2015)
3. Common Presentations and Diagnostic Approaches, Stiehm's Immune Deficiencies: Inborn Errors of Immunity (2020)
4. Congenital Neutropenia and Primary Immunodeficiency Diseases, Critical Reviews in Oncology/Hematology (2019)
5. Pulmonary Manifestations of Predominantly Antibody Deficiencies, Pulmonary Manifestations of Primary Immunodeficiency Diseases (2018)
6. Clinical, Immunological, and Genetic Features in Patients With Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (Ipex) and Ipex-Like Syndrome, Journal of Allergy and Clinical Immunology: In Practice (2020)
7. Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis, Journal of Clinical Immunology (2025)
8. Management of Inborn Errors of Immunity in the Genomic Era, Turkish Archives of Pediatrics (2022)
Experts (# of related papers)
View all Related Experts
Nima Rezaei (52)
Alireza Shafiei Esfidvajani (5)
Other Related Docs
9. B- and T-Cell Subset Abnormalities in Monogenic Common Variable Immunodeficiency, Frontiers in Immunology (2022)
10. Role of Apoptosis in Common Variable Immunodeficiency and Selective Immunoglobulin a Deficiency, Molecular Immunology (2016)
11. Toward the Stratification and Personalization of Common Variable Immunodeficiency Treatment, Expert Opinion on Orphan Drugs (2016)
12. Application of Flow Cytometry in Predominantly Antibody Deficiencies, Endocrine# Metabolic and Immune Disorders - Drug Targets (2021)
13. The Cytoskeletal Regulator Hem1 Governs B Cell Development and Prevents Autoimmunity, Science Immunology (2020)
14. Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management, Journal of Investigational Allergology and Clinical Immunology (2020)
15. Primary Immunodeficiency Diseases in Iran: Past, Present and Future, Archives of Iranian Medicine (2021)
16. Known and Potential Molecules Associated With Altered B Cell Development Leading to Predominantly Antibody Deficiencies, Pediatric Allergy and Immunology (2021)
17. Cellular and Molecular Mechanisms of Immune Dysregulation and Autoimmunity, Cellular Immunology (2016)
18. Autoinflammatory Disorders, Translational Autoimmunity: Autoimmune Diseases in Different Organs (2022)
19. Evaluation of Specific Antibody Responses in Patients With Selective Iga Deficiency and Ataxia Telangiectasia, Endocrine# Metabolic and Immune Disorders - Drug Targets (2022)
20. Specific Immune Response and Cytokine Production in Cd70 Deficiency, Frontiers in Pediatrics (2021)
21. Immunodeficiencies, Clinical Immunology (2022)
22. Autoimmune Diseases, Clinical Immunology (2022)
23. The Heterogeneous Pathogenesis of Selective Immunoglobulin a Deficiency, International Archives of Allergy and Immunology (2019)
24. Monogenic Polyautoimmunity in Primary Immunodeficiency Diseases, Autoimmunity Reviews (2018)
25. Primary Immunodeficiencies Associated With Ebv-Induced Lymphoproliferative Disorders, Critical Reviews in Oncology/Hematology (2016)
26. Clinical, Immunological, Molecular and Therapeutic Findings in Monogenic Immune Dysregulation Diseases: Middle East and North Africa Registry, Clinical Immunology (2022)
27. Autoimmune Disorders Associated With Common Variable Immunodeficiency: Prediction, Diagnosis, and Treatment, Expert Review of Clinical Immunology (2022)
28. The Role of Toll-Like Receptors in B-Cell Development and Immunopathogenesis of Common Variable Immunodeficiency, Expert Review of Clinical Immunology (2016)
29. Agammaglobulinemia: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management, Endocrine# Metabolic and Immune Disorders - Drug Targets (2020)
30. Expanding the Clinical and Immunological Phenotypes and Natural History of Malt1 Deficiency, Journal of Clinical Immunology (2022)
31. Autoimmunity in Primary T-Cell Immunodeficiencies, Expert Review of Clinical Immunology (2016)
32. Autoimmunity in Primary Antibody Deficiencies, International Archives of Allergy and Immunology (2017)
33. Evaluation of Known Defective Signaling-Associated Molecules in Patients Who Primarily Diagnosed As Common Variable Immunodeficiency, International Reviews of Immunology (2016)
34. Identifying Novel Mutations in Iranian Patients With Lps-Responsive Beige-Like Anchor Protein (Lrba) Deficiency, Immunological Investigations (2021)
35. Autoimmunity in Patients With Selective Iga Deficiency, Journal of Investigational Allergology and Clinical Immunology (2015)
36. Clinical, Immunological, and Genetic Features in Patients With Nfkb1 and Nfkb2 Mutations: A Systematic Review, Journal of Clinical Immunology (2024)
37. Efficacy and Safety of Syk and Btk Inhibitors in Immune Thrombocytopenia: A Comprehensive Review of Emerging Evidence, Mediators of Inflammation (2025)
38. Revealing Disease Subtypes and Heterogeneity in Common Variable Immunodeficiency Through Transcriptomic Analysis, Scientific Reports (2024)
39. Germline Biallelic Mutation Affecting the Transcription Factor Helios Causes Pleiotropic Defects of Immunity, Science Immunology (2021)
40. Dengue Virus Infection: How Platelet-Leukocyte Crosstalk Shapes Thrombotic Events and Inflammation, Molecular Biology Reports (2025)
41. Primary Immunodeficiency Associated With Hypopigmentation: A Differential Diagnosis Approach, Allergologia et Immunopathologia (2021)
42. The Middle East and North Africa Diagnosis and Management Guidelines for Inborn Errors of Immunity, Journal of Allergy and Clinical Immunology: In Practice (2023)
43. Adverse Reactions in a Large Cohort of Patients With Inborn Errors of Immunity Receiving Intravenous Immunoglobulin, Clinical Immunology (2021)
44. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders, Journal of Investigational Allergology and Clinical Immunology (2017)
45. Atypical Ataxia Presentation in Variant Ataxia Telangiectasia: Iranian Case-Series and Review of the Literature, Frontiers in Immunology (2022)
46. Investigating the Variation of Trec/Krec in Combined Immunodeficiencies, Iranian Journal of Allergy# Asthma and Immunology (2021)
47. The Hyper Igm Syndromes: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis and Management, Clinical Immunology (2019)
48. The First Iranian Cohort of Pediatric Patients With Activated Phosphoinositide 3-Kinase-Δ (Pi3kδ) Syndrome (Apds), Immunological Investigations (2022)
49. Diagnostic Approach to the Patients With Suspected Primary Immunodeficiency, Endocrine# Metabolic and Immune Disorders - Drug Targets (2020)
50. Griscelli Syndrome Type 2: A Rare Case With Apparently Normal Skin and Hair Pigmentation, Acta Medica Iranica (2020)