Hematologic complications of primary immune deficiencies

Blood Reviews
C Cunningham-Rundles

Abstract

Primary immune deficiencies have an estimated overall incidence of 1 in 10,000 individuals. These disorders are diverse, depending on the specific immune functions involved, and lead to chronic or recurrent infections, inflammatory conditions, and a variety of autoimmune diseases. The most common autoimmune disorder is immune thrombocytopenic purpura (ITP), followed by autoimmune hemolytic anemia (AHA). While cytopenias are common in all the congenital immune diseases, they are particularly common in the antibody defects, common variable immunodeficiency and selective immunoglobulin A deficiency. In common variable immunodeficiency, ITP occurred in 7.6% of the patients and AHA in 4.8%. Treatment options include corticosteroids, intravenous immunoglobulin (i.v.Ig), anti-D, and splenectomy. Although the association between cytopenias and congenital immune deficiency is unclear, defects in T-cell regulation, cytokine defects, abnormal apoptosis, and abnormal production of immunoglobulins with autoimmune features are potential mechanisms.

References

Jan 1, 1992·International Archives of Allergy and Immunology·R S Liblau, J F Bach
Apr 8, 1998·Seminars in Immunology·G J Silverman
Oct 28, 1998·Proceedings of the National Academy of Sciences of the United States of America·Y LevyC A Reynaud
Jul 22, 1999·Clinical Immunology : the Official Journal of the Clinical Immunology Society·C Cunningham-Rundles, C Bodian
May 3, 2000·Clinical and Experimental Immunology·L HammarströmD Webster

❮ Previous
Next ❯

Citations

Nov 19, 2013·Journal of Clinical Immunology·Beatriz Tavares Costa-CarvalhoAntonio Condino-Neto
Jul 3, 2009·International Archives of Allergy and Immunology·Sean DeaneM Eric Gershwin
Sep 27, 2013·PloS One·Joon ParkCharlotte Cunningham-Rundles
Jul 26, 2013·American Journal of Rhinology & Allergy·Jonathan S Tam, John M Routes
Jan 14, 2011·Haematologica·Nathalie AladjidiUNKNOWN Centre de Référence National des Cytopénies Auto-immunes de l'Enfant (CEREVANCE)
Oct 9, 2009·International Reviews of Immunology·Nima RezaeiChristoph Klein
Jun 5, 2013·Expert Review of Clinical Immunology·Hassan AbolhassaniAsghar Aghamohammadi
Dec 12, 2012·Autoimmunity·Marilyn Diaz
Jul 3, 2013·Joint, Bone, Spine : Revue Du Rhumatisme·Camila Cristina BoninJozélio Freire de Carvalho
Nov 14, 2015·The Journal of Allergy and Clinical Immunology. in Practice·Francisco A BonillaKlaus Warnatz
Nov 3, 2010·Joint, Bone, Spine : Revue Du Rhumatisme·Maxime SamsonPaul Ornetti
Feb 23, 2010·Autoimmunity Reviews·Joseph C Turbyville, V Koneti Rao
Feb 2, 2010·Hematology/oncology Clinics of North America·Victor Blanchette, Paula Bolton-Maggs
Nov 21, 2015·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Emil Kofod-OlsenTrine H Mogensen
Sep 1, 2009·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Omür Ardeniz, Charlotte Cunningham-Rundles
Mar 31, 2009·Seminars in Hematology·James B Bussel
Aug 12, 2008·Lancet·Miguel A ParkRoshini S Abraham
Apr 3, 2008·Pediatric Clinics of North America·Victor Blanchette, Paula Bolton-Maggs
May 13, 2008·Immunobiology·Asghar AghamohammadiSaeed Reza Ghaffari
Jul 29, 2006·Pediatric Clinics of North America·James W Verbsky, William J Grossman
Jun 14, 2005·The British Journal of Dermatology·N OkiyamaK Nishioka
Oct 12, 2015·Immunology and Allergy Clinics of North America·Jordan K Abbott, Erwin W Gelfand
Apr 7, 2009·British Journal of Haematology·Helen Chapel, Charlotte Cunningham-Rundles
Jan 6, 2004·European Journal of Haematology·Véronique Le GuernLuc Mouthon
Feb 14, 2004·Immunological Reviews·Jeffrey C Rathmell
May 27, 2010·Scandinavian Journal of Immunology·G J ArasonB R Ludviksson
Oct 7, 2006·Seminars in Immunology·Ulrich Salzer, Bodo Grimbacher
Aug 22, 2006·Autoimmunity Reviews·Daniel Brandt, M Eric Gershwin
Jan 25, 2006·Autoimmunity Reviews·Adina Kay Knight, Charlotte Cunningham-Rundles
Jul 5, 2005·Journal of Autoimmunity·Julie Wang, Charlotte Cunningham-Rundles
Jun 1, 2005·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Jimmy KoCharlotte Cunningham-Rundles
Dec 4, 2009·Pediatric Hematology and Oncology·Mehdi Mohammad G AdeliLarry Williams
Mar 25, 2010·Blood·Charlotte Cunningham-Rundles
Jan 31, 2015·Chinese Medical Journal·Lian-Jun LinXin-Min Liu
Apr 12, 2016·Expert Review of Clinical Immunology·Gholamreza AziziAsghar Aghamohammadi
Jun 9, 2007·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Adina K KnightCharlotte Cunningham-Rundles

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Apoptosis

Apoptosis is a specific process that leads to programmed cell death through the activation of an evolutionary conserved intracellular pathway leading to pathognomic cellular changes distinct from cellular necrosis

Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia (AIHA) occurs when antibodies directed against the person's own red blood cells (RBCs) cause them to burst (lyse), leading to an insufficient number of oxygen-carrying red blood cells in the circulation. Discover the latest research on AIHA here.

Related Papers

Autoimmunity Reviews
Daniel Brandt, M Eric Gershwin
Scandinavian Journal of Infectious Diseases
Christopher M BurtonMartin Iversen
Current Allergy and Asthma Reports
Yoshikazu Morimoto, John M Routes
Pediatric Hematology and Oncology
Rahul NaithaniV P Choudhry
© 2022 Meta ULC. All rights reserved