Tools
Transfusion Selective IgA Deficiency
Introduction
Selective immunoglobulin A (IgA) deficiency is the most common immunodeficiency in individuals older than 4, after excluding other causes of hypogammaglobulinemia.[1] Patients can be asymptomatic or experience recurrent infections and autoimmune illnesses. Recurrent infections, often caused by encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae, most likely affect the sinopulmonary system, presenting as otitis media, sinusitis, and pneumonia. Long-term sequelae include end-organ damage, such as bronchiectasis. Infections of the gastrointestinal system caused by Giardia lamblia occur to a lesser extent, likely due to the increased presence of secreted IgM in the gastrointestinal tract. Additional associated conditions are asthma and allergies, celiac disease, inflammatory bowel disease, nodular lymphoid hyperplasia, systemic lupus erythematosus, Graves' disease, rheumatoid arthritis, and Myasthenia gravis.[2][3][4] False-positive pregnancy tests are possible due to heterophile antibodies.
Individuals with selective IgA deficiency may develop anti-IgA antibodies causing allergic reactions when transfused with blood products containing IgA. These reactions can range from mild symptoms such as fever, chills, hives, nausea, and flushing to severe reactions such as low blood pressure, rapid heartbeat, and life-threatening anaphylaxis.[5] Anti-IgA antibodies are most common in patients with undetectable serum IgA levels. Although studies do not conclusively link anti-IgA antibodies to these reactions, they reveal that the antibodies are significantly more prevalent in patients experiencing anaphylaxis compared to those without.[5][6] Patients with selective IgA deficiency are at a significantly higher risk of transfusion reactions compared to the general population, making careful blood product selection crucial to prevent complications.
Because most patients with selective IgA deficiency do not form anti-IgA antibodies, universal testing is unnecessary. However, clinicians should administer blood products with caution in patients with selective IgA deficiency. Experts recommend screening patients with severe selective IgA deficiency and those who have experienced a reaction to blood products in the past for anti-IgA antibodies.[7] Clinicians must implement a plan to safely administer blood products in patients who test positive for antibodies. Techniques may include sourcing blood products from IgA-deficient donors, administering washed red blood cells and platelets, administering immunoglobulin low in IgA or subcutaneously, or desensitizing the patient to blood products.
Etiology
Register For Free And Read The Full Article
Search engine and full access to all medical articles- 10 free questions in your specialty
- Free CME/CE Activities
- Free daily question in your email
- Save favorite articles to your dashboard
- Emails offering discounts
Learn more about a Subscription to StatPearls Point-of-Care
Etiology
A selective IgA deficiency-mediated anaphylactic transfusion reaction is anaphylaxis that occurs within 1 hour of transfusing plasma-containing blood products in IgA-deficient individuals with plasma anti-IgA antibodies.[8] Indirect evidence suggests that these reactions occur due to anti-IgA antibodies in IgA-deficient individuals. Experts believe that a hypersensitivity transfusion reaction is doubtful in a patient with demonstrable serum IgA levels.[9] A study using passive hemagglutination assays detected anti-IgA antibodies in 76.3% of individuals with IgA deficiency and a history of anaphylactic transfusion reactions compared to 21.7% of asymptomatic individuals with IgA deficiency.[6]
Because some individuals with IgA deficiency and anti-IgA antibodies receive blood products containing IgA without experiencing adverse effects, the role of these antibodies remains controversial.[10] Further research is necessary to clarify their full significance.[11]
Epidemiology
Selective IgA deficiency is the most common primary immunodeficiency, with a prevalence ranging from 1 in 150 to 1 in 1000 among Black, White, and Middle Eastern populations.[12] The condition occurs more frequently in males compared to females and is uncommon in patients of Asian descent.[12][13][14] Studies reveal that the actual incidence of anaphylactic reactions in patients with selective IgA deficiency is quite rare. Approximately 1 in 1200 to 1600 patients with IgA deficiency develop anti-IgA antibodies. Experts estimate that the incidence of blood transfusion reactions caused by anti-IgA antibodies is approximately 1.3 per million units of transfused blood products.[15] Data from the Centers for Disease Control reveal that anaphylaxis accounts for 9% of acute transfusion-related deaths between 2017 and 2021.[16]
Pathophysiology
The exact pathogenesis of selective IgA deficiency remains unclear and likely results from the interplay of multiple mechanisms. Please see StatPearls' companion, "Selective IgA Deficiency," for more information.
Patients with undetectable serum IgA levels may develop anti-IgA antibodies. Upon exposure to IgA, individuals with selective IgA deficiency become sensitized and develop both IgG- and IgE-specific anti-IgA antibodies.[5] Anti-IgA-mediated anaphylaxis occurs upon subsequent exposure to IgA. IgE-mediated transfusion reactions occur immediately through a type I hypersensitivity response. In this response, the allergen triggers the secretion of IgE by plasma cells. These IgE antibodies bind to and sensitize mast cells and basophils. Subsequent exposures to the allergen cause crosslinking of preformed IgE on the sensitized mast cells and basophils. Ultimately, this causes degranulation of cells and the release of active mediators, such as histamine. Histamine causes vasodilation and smooth muscle contraction, contributing to anaphylaxis.[17]
To date, researchers have not demonstrated IgG-dependent anaphylaxis in humans. Based on animal models, proposed mechanisms of IgG-mediated anaphylactic reactions suggest that the allergen interacts with allergen-specific IgG bound to Fc-γ-RIII on macrophages and basophils. The macrophage activation results in the release of platelet-activating factor rather than histamine. ΡAF causes platelets to aggregate and release thromboxane A2 and serotonin, which cause vasoconstriction and increase vascular permeability.[18][19]
History and Physical
Patients with selective IgA deficiency can be asymptomatic or have a history of recurrent sinopulmonary infections, most commonly with the encapsulated bacteria S pneumoniae and H influenzae. Children present with recurrent otitis media, sinusitis, or pneumonia, whereas otitis media is less common in adults.[20] Anaphylactic transfusion reactions occur rapidly, typically within a few minutes of beginning the transfusion. Patients develop cutaneous, pulmonary, cardiovascular, and possibly gastrointestinal symptoms such as pruritus, urticaria, flushing, and diarrhea, quickly followed by shock, hypotension, angioedema, and respiratory distress.[8] To mitigate this risk, healthcare professionals inquire about a patient's history of previous transfusions and any prior transfusion reactions.
Patients with the following list of conditions associated with selective IgA deficiency may also be at increased risk when undergoing blood product transfusion in the presence of IgA deficiency:
- Celiac disease
- Ataxia-telangiectasia
- Juvenile rheumatoid arthritis
- Chronic hepatitis C infection
- Myotonic dystrophy
- Protein-losing enteropathy
- Graves' disease
- Type I diabetes
- Lymphoma
- Gastric adenocarcinoma
- Common variable immunodeficiency
- Allergic rhinitis
- Inflammatory bowel disease
- Patients with recurrent sinopulmonary infections
- Nodular lymphoid hyperplasia [21][22][23][24][25]
Evaluation
Patients with suspected anaphylaxis require immediate evaluation and management. Clinicians should assess vital signs, lips, tongue, oropharynx, and ability to speak for angioedema and airway patency. The presence of urticaria, flushing, and pruritus help confirm the diagnosis. Stridor, wheezing, dyspnea, increased work of breathing, retractions, persistent cough, cyanosis, poor perfusion, abdominal pain, vomiting, dysrhythmia, and hypotension are all warning signs of anaphylaxis.
Currently, there are no universal guidelines for screening for anti-IgA antibodies. The utility of testing patients with low IgA levels who have not been transfused or have not had a transfusion reaction is unclear. Some experts recommend screening for anti-IgA antibodies in patients with severe selective IgA deficiency, those with selective IgA deficiency who previously experienced a reaction to blood products, or those who have experienced two or more severe allergic transfusion reactions.[26] Healthcare professionals typically test for anti-IgA antibodies using enzyme-linked immunosorbent assay. A relatively newer, portable surface plasmon resonance sensor is gaining favor in detecting patients with selective IgA deficiency.[27] Clinicians can detect selective IgA deficiency in approximately 1 hour at the point of care, allowing for rapid identification of affected blood recipients and improved maintenance of IgA-deficient blood stores. Currently, this method may be cost-prohibitive for some sites.[27]
Treatment / Management
As previously discussed, most patients with selective IgA deficiency who receive blood transfusions do so without reactions. However, in cases where anaphylaxis occurs, prompt emergency intervention is crucial. Patients with anaphylaxis require concurrent evaluation and management. The focus is on the patient's airway, breathing, and circulation. Clinicians must stop the transfusion immediately and administer epinephrine. The dosing for epinephrine is as follows:
- Children: Administer epinephrine 1 mg/mL 0.01 mg/kg intramuscularly (IM) in the mid-outer thigh. Repeat every 5 to 15 minutes or sooner if necessary. Most children respond after a maximum of 3 injections. If poor perfusion or poor response, administer a continuous intravenous (IV) epinephrine infusion of 0.1 to 1 µg/kg/min. For children weighing more than 50 kg, the maximum dose is 0.5 mg/dose.
- Adults: Administer epinephrine 1 mg/mL 0.3 to 0.5 mg IM in the mid-outer thigh. Repeat as necessary every 5 to 15 minutes or sooner if necessary. As with children, most adults respond after a maximum of 3 injections. Failure to respond warrants IV epinephrine 0.1 µg/kg/min titrated based on cardiac rate and function, blood pressure, and oxygen levels.
Immediate intubation is necessary if stridor or respiratory distress is present.[28] All healthcare team members should prepare for quick intubation in cases of any airway involvement. If intubation is not necessary and the patient is able to do so, clinicians should place the patient in a recumbent position with elevated feet and administer 15 L high-flow oxygen, using a non-rebreather mask to provide 70% to 100% oxygen. Poor perfusion requires IV fluids. Infants and children should receive 20 mL/kg of rapid normal saline IV boluses, repeated as necessary. Adults can receive 1 to 2 L. Clinicians may switch to Lactated Ringer solution if large volumes of fluid are necessary to prevent a hyperchloremic metabolic acidosis. Short-acting ß-agonists such as albuterol help alleviate bronchospasm resistant to epinephrine. The adult dose is 2.5 to 5 mg in 3 mL using a nebulizer or 2 to 3 puffs using a metered dose inhaler repeated as necessary. Children should receive 2.5 mg using a nebulizer. Additional vasopressors, such as vasopressin, may be necessary if patients do not respond to IV fluids and epinephrine.
Antihistamines such as diphenhydramine and cetirizine help alleviate pruritus and urticaria. However, they do not treat the symptoms of anaphylaxis, such as hypotension, shock, or airway obstruction. Although antihistamine administration can relieve symptoms, it should only be given after epinephrine administration. Studies show no benefit of glucocorticoids in treating anaphylaxis.[29] However, some experts suggest that glucocorticoids may be beneficial for patients with severe symptoms requiring hospitalization or those with a history of asthma and persistent bronchospasm after other anaphylaxis symptoms resolve. The recommended dose is methylprednisolone 1 to 2 mg/kg/d for 1 to 2 days.(A1)
Anaphylaxis has a biphasic reaction in approximately 5% of patients.[30] Therefore, clinicians must determine the appropriate length of time to monitor patients who experience anaphylaxis. In general, patients who experience severe anaphylaxis who do not readily respond to epinephrine or require more than one dose of epinephrine warrant hospital admission for observation. A minimum of 4 hours of observation appears safe for some patients with uncomplicated anaphylaxis.[31] All patients should leave with an epinephrine auto-injector or a prescription for 2 injectors and instructions to pick them up at the pharmacy immediately. Patients should also receive personalized written education regarding the symptoms of anaphylaxis and what to do if they experience symptoms.
Patients with severe selective IgA deficiency should wear medical alert identification that notifies healthcare professionals of their condition, the potential risk of anaphylactic reaction to plasma-containing blood products, and the need for anti-IgA testing before receiving transfusions.[32] All facilities administering blood products must actively recognize and promptly treat any reactions to transfused blood products. Methods to prevent anaphylactic reactions in patients with anti-IgA antibodies include exclusively using products from IgA-deficient donors and transfusing washed platelets and red blood cells.[33] Because clinicians are unable to wash plasma, clinicians must use plasma from an IgA-deficient donor. Fresh frozen plasma (FFP) has a shelf-life of 12 months, and blood banks may be more likely to have FFP from IgA-deficient donors. However, if FFP from a donor who is not IgA-deficient is unavailable, clinicians may elect to administer FFP slowly under close monitoring as the likely benefit of FFP outweighs the risk of anaphylaxis. Recombinant activated coagulation factor VII (rFVIIa) is also a possible alternative. There are some case reports where rFVIIa is used as an alternative treatment for systemic bleeding disorders instead of FFP or platelets.[8]
For patients with anti-IgA antibodies who require immunoglobulin replacement, subcutaneous immunoglobulin is a viable alternative. In a retrospective study of patients with adverse effects to blood products or immunoglobulins, approximately 25% of the patients experienced reactions due to anti-IgA antibodies. When these patients received subcutaneous immunoglobulin, they experienced no adverse effects. Some patients showed undetectable levels of anti-IgA antibodies, allowing them to tolerate the administration of intravenous immunoglobulin. Experts believe that the effect is due to the formation of antibody-IgA complexes, quickly removed from the circulation.[34] If subcutaneous immunoglobulin is not an acceptable option, IV immunoglobulin from an IgA-deficient donor is the most suitable choice.[35] Desensitization is an additional management option. Clinicians expose affected patients to a continuous infusion of gradually increasing concentrations of IgA-containing immunoglobulin preparations.[36] (B2)
Differential Diagnosis
The presence of a severe transfusion reaction should prompt the clinician to consider other potential causes of the patient's symptoms, such as:
- Anaphylactic transfusion reaction due to other allergens
- Acute hemolytic transfusion reactions
- Transfusion-related acute lung injury
- Circulatory overload
- Sepsis
- Angioedema
- Hemolytic anemia
- Cold agglutinin disease
- Disseminated intravascular coagulation
- Coincident exposure to allergens
- Urticarial transfusion reaction
- Febrile nonhemolytic transfusion reaction
- Hypotensive primary response [8][37]
Prognosis
The risk of an anaphylactic reaction to plasma-containing blood products is rare but potentially life-threatening. Data from the Centers for Disease Control and Prevention reveals two definite and two probable fatalities due to transfusion-related anaphylaxis in 2021. Although the case fatality rate for anaphylaxis is relatively low, healthcare professionals must be able to evaluate and treat patients promptly and effectively when they suspect an IgA-mediated anaphylactic transfusion reaction.
Complications
As discussed earlier, the most severe complication of transfusion-selective IgA deficiency is anaphylaxis and death.
Deterrence and Patient Education
Most patients with selective IgA deficiency remain asymptomatic, but many seek medical attention due to recurrent sinopulmonary infections, Giardia lamblia infections, autoimmune disorders, or anaphylactic transfusion reactions. Although rare, a significant concern in selective IgA deficiency is the potential development of anti-IgA antibodies in individuals with undetectable IgA levels. These antibodies can trigger anaphylactic reactions on exposure to IgA-containing blood products, including whole blood, red blood cells, platelets, fresh frozen plasma, cryoprecipitate, granulocytes, or IV immunoglobulins. Anaphylaxis can occur within seconds to minutes of transfusion initiation and may present with hypotension, shock, angioedema, respiratory distress, wheezing, pruritus, urticaria, flushing, abdominal pain, or diarrhea.
Patient education is paramount in preventing anti-IgA-mediated anaphylaxis. Clinicians should counsel individuals with selective IgA deficiency and their caregivers about transfusion risks and necessary precautions. Patients should:
- Inform all healthcare providers of their IgA deficiency before any transfusion.
- Wear a medical alert bracelet or necklace to ensure rapid identification in emergencies.
- Ensure their medical records explicitly document their IgA deficiency.
- If a transfusion is required, use IgA-deficient blood products, washed red blood cells, or platelets to minimize IgA exposure.
- Recognize early signs of an adverse reaction, such as difficulty breathing, hypotension, or hives, and seek immediate medical attention.
By fostering awareness and encouraging proactive management, clinicians can help prevent life-threatening transfusion reactions and empower patients to take an active role in their health care.
Enhancing Healthcare Team Outcomes
Selective IgA deficiency is the most common primary immunodeficiency, characterized by an isolated deficiency of serum IgA with normal IgG and IgM levels in individuals older than 4, after excluding other causes of hypogammaglobulinemia. Although many affected individuals remain asymptomatic, some experience recurrent sinopulmonary or gastrointestinal infections, autoimmune disorders, and allergic diseases. A critical concern in selective IgA deficiency is the potential for anaphylactic transfusion reactions due to anti-IgA antibodies, particularly in patients with undetectable IgA levels. Although not all individuals with selective IgA deficiency develop these antibodies, those who do are at higher risk of severe immune responses when exposed to IgA-containing blood products. Symptoms of transfusion reactions range from mild fever, chills, and hives to life-threatening anaphylaxis with hypotension, respiratory distress, and shock. To mitigate risks, clinicians should educate affected patients on transfusion precautions, including wearing medical alert identification, documenting IgA deficiency in medical records, and using IgA-deficient or washed blood products when necessary. Screening for anti-IgA antibodies is recommended in patients with severe selective IgA deficiency or a history of transfusion reactions. By implementing careful transfusion practices and patient education, clinicians can minimize the risk of life-threatening reactions in individuals with selective IgA deficiency.
Effective patient-centered care, particularly in the context of transfusion and selective IgA deficiency, depends on strong collaboration and communication among healthcare professionals, including clinicians, advanced practitioners, pharmacists, and other healthcare providers. Each professional brings unique expertise to the team to optimize patient outcomes and safety. Clinicians and advanced practitioners play key roles in diagnosing and managing selective IgA deficiency, considering its potential impact on transfusion reactions. Nurses are critical in monitoring the patient during transfusion procedures, ensuring that any adverse reactions are quickly identified and addressed. Pharmacists review medications that may interact with transfusion protocols and ensure that appropriate blood products are selected based on the patient's condition. Communication is essential across these roles to ensure that everyone involved in the patient's care is informed about the risks and needs associated with IgA deficiency. Coordinating care requires a shared understanding of the patient's medical history, allergies, and transfusion requirements to minimize risks, such as anaphylactic reactions or immune responses. This multidisciplinary approach prioritizes patient safety and proactive management of potential complications, ensuring patients receive comprehensive care that aligns with their individual needs and preferences. Ultimately, a collaborative, well-coordinated team fosters a supportive environment where each healthcare professional enhances the patient's well-being and improves clinical outcomes.
References
Yel L. Selective IgA deficiency. Journal of clinical immunology. 2010 Jan:30(1):10-6. doi: 10.1007/s10875-009-9357-x. Epub 2010 Jan 26 [PubMed PMID: 20101521]
Level 3 (low-level) evidenceCinicola BL, Pulvirenti F, Capponi M, Bonetti M, Brindisi G, Gori A, De Castro G, Anania C, Duse M, Zicari AM. Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story. Medicina (Kaunas, Lithuania). 2022 Jan 15:58(1):. doi: 10.3390/medicina58010129. Epub 2022 Jan 15 [PubMed PMID: 35056437]
Hua L, Guo D, Liu X, Jiang J, Wang Q, Wang Y, Liu T, Li F. Selective IgA Deficiency with Multiple Autoimmune Comorbidities: A Case Report and Literature Review. Iranian journal of immunology : IJI. 2023 May 31:20(2):232-239. doi: 10.22034/iji.2023.97452.2513. Epub 2023 May 9 [PubMed PMID: 37158141]
Level 3 (low-level) evidenceAmiel A, Van Gucht T, Bolliet M, Aussenac-Belle L, David P, Plastaras L, Martinot M. Selective IgA Deficiency and Aseptic Liver Abscess as Initial Indicators of Crohn's Disease in a Young Woman: A Case Study. The American journal of case reports. 2024 Dec 24:25():e944829. doi: 10.12659/AJCR.944829. Epub 2024 Dec 24 [PubMed PMID: 39718979]
Level 3 (low-level) evidenceRachid R, Bonilla FA. The role of anti-IgA antibodies in causing adverse reactions to gamma globulin infusion in immunodeficient patients: a comprehensive review of the literature. The Journal of allergy and clinical immunology. 2012 Mar:129(3):628-34. doi: 10.1016/j.jaci.2011.06.047. Epub 2011 Aug 11 [PubMed PMID: 21835445]
Level 3 (low-level) evidenceSandler SG, Eckrich R, Malamut D, Mallory D. Hemagglutination assays for the diagnosis and prevention of IgA anaphylactic transfusion reactions. Blood. 1994 Sep 15:84(6):2031-5 [PubMed PMID: 8081004]
Brown R, Nelson M, Aklilu E, Kabani K, Yang S, Blayney B, Popp H. An evaluation of the DiaMed assays for immunoglobulin A antibodies (anti-IgA) and IgA deficiency. Transfusion. 2008 Oct:48(10):2057-9. doi: 10.1111/j.1537-2995.2008.01830.x. Epub 2008 Jul 24 [PubMed PMID: 18657081]
Sandler SG, Zantek ND. Review: IgA anaphylactic transfusion reactions. Part II. Clinical diagnosis and bedside management. Immunohematology. 2004:20(4):234-8 [PubMed PMID: 15679455]
Tacquard C, Boudjedir K, Carlier M, Muller JY, Gomis P, Mertes PM. Hypersensitivity transfusion reactions due to IgA deficiency are rare according to French hemovigilance data. The Journal of allergy and clinical immunology. 2017 Sep:140(3):884-885. doi: 10.1016/j.jaci.2017.03.029. Epub 2017 Apr 13 [PubMed PMID: 28414063]
Lilic D, Sewell WA. IgA deficiency: what we should-or should not-be doing. Journal of clinical pathology. 2001 May:54(5):337-8 [PubMed PMID: 11328829]
Collet A, de Chambure DP, Moitrot E, Breyne G, Mirgot F, Rogeau S, Tronchon M, Nicolas A, Sanges S, Stabler S, Ledoult E, Terriou L, Launay D, Hachulla E, Labalette M, Dubucquoi S, Lefèvre G. Non-allergic Hypersensitivity Reactions to Immunoglobulin Preparations in Antibody Deficiencies: What Role for Anti-IgA IgG and Complement Activation? Clinical reviews in allergy & immunology. 2024 Dec:67(1-3):47-57. doi: 10.1007/s12016-024-09007-0. Epub 2024 Oct 22 [PubMed PMID: 39436576]
Morawska I, Kurkowska S, Bębnowska D, Hrynkiewicz R, Becht R, Michalski A, Piwowarska-Bilska H, Birkenfeld B, Załuska-Ogryzek K, Grywalska E, Roliński J, Niedźwiedzka-Rystwej P. The Epidemiology and Clinical Presentations of Atopic Diseases in Selective IgA Deficiency. Journal of clinical medicine. 2021 Aug 25:10(17):. doi: 10.3390/jcm10173809. Epub 2021 Aug 25 [PubMed PMID: 34501259]
Hammarström L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clinical and experimental immunology. 2000 May:120(2):225-31 [PubMed PMID: 10792368]
Level 3 (low-level) evidenceFeng ML, Zhao YL, Shen T, Huang H, Yin B, Liu RZ, Qian KC, Liu DZ. Prevalence of immunoglobulin A deficiency in Chinese blood donors and evaluation of anaphylactic transfusion reaction risk. Transfusion medicine (Oxford, England). 2011 Oct:21(5):338-43. doi: 10.1111/j.1365-3148.2011.01082.x. Epub 2011 Jun 9 [PubMed PMID: 21658139]
Level 2 (mid-level) evidenceLatiff AH, Kerr MA. The clinical significance of immunoglobulin A deficiency. Annals of clinical biochemistry. 2007 Mar:44(Pt 2):131-9 [PubMed PMID: 17362578]
Ma L, Danoff TM, Borish L. Case fatality and population mortality associated with anaphylaxis in the United States. The Journal of allergy and clinical immunology. 2014 Apr:133(4):1075-83. doi: 10.1016/j.jaci.2013.10.029. Epub 2013 Dec 14 [PubMed PMID: 24332862]
Level 3 (low-level) evidenceWarrington R, Watson W, Kim HL, Antonetti FR. An introduction to immunology and immunopathology. Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology. 2011 Nov 10:7 Suppl 1(Suppl 1):S1. doi: 10.1186/1710-1492-7-S1-S1. Epub 2011 Nov 10 [PubMed PMID: 22165815]
Kinn JW, Bache RJ. Effect of platelet activation on coronary collateral blood flow. Circulation. 1998 Oct 6:98(14):1431-7 [PubMed PMID: 9760298]
Kow ASF, Chik A, Soo KM, Khoo LW, Abas F, Tham CL. Identification of Soluble Mediators in IgG-Mediated Anaphylaxis via Fcγ Receptor: A Meta-Analysis. Frontiers in immunology. 2019:10():190. doi: 10.3389/fimmu.2019.00190. Epub 2019 Feb 12 [PubMed PMID: 30809224]
Level 1 (high-level) evidenceNúnez-Núñez ME, Monraz-Monteón D, Lona-Reyes JC, Pozos-Ochoa LI, Magallón-Picazo D, Bayardo-Gutierrez B. [Necrotizing pneumonia in a patient with selective IgA deficiency]. Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993). 2024 Sep 30:71(3):205-211. doi: 10.29262/ram.v71i3.1344. Epub 2024 Sep 30 [PubMed PMID: 39625802]
Pytrus T, Iwańczak B, Iwańczak F. [Nodular lymphoid hyperplasia--underestimated problem of gastrointestinal tract pathology in children]. Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego. 2008 May:24(143):449-52 [PubMed PMID: 18634394]
Asada Y, Isomoto H, Shikuwa S, Wen CY, Fukuda E, Miyazato M, Okamoto K, Nakamura T, Nishiyama H, Mizuta Y, Migita K, Ito M, Kohno S. Development of ulcerative colitis during the course of rheumatoid arthritis: Association with selective IgA deficiency. World journal of gastroenterology. 2006 Aug 28:12(32):5240-3 [PubMed PMID: 16937542]
Cataldo F, Marino V, Bottaro G, Greco P, Ventura A. Celiac disease and selective immunoglobulin A deficiency. The Journal of pediatrics. 1997 Aug:131(2):306-8 [PubMed PMID: 9290622]
Wang N, Shen N, Vyse TJ, Anand V, Gunnarson I, Sturfelt G, Rantapää-Dahlqvist S, Elvin K, Truedsson L, Andersson BA, Dahle C, Ortqvist E, Gregersen PK, Behrens TW, Hammarström L. Selective IgA deficiency in autoimmune diseases. Molecular medicine (Cambridge, Mass.). 2011:17(11-12):1383-96. doi: 10.2119/molmed.2011.00195. Epub 2011 Aug 4 [PubMed PMID: 21826374]
Khanmohammadi S, Shad TM, Delavari S, Shirmast P, Bagheri Y, Azizi G, Aghamohammadi A, Abolhassani H, Yazdani R, Rezaei N. Evaluation of Specific Antibody Responses in Patients with Selective IgA Deficiency and Ataxia Telangiectasia. Endocrine, metabolic & immune disorders drug targets. 2022:22(6):640-649. doi: 10.2174/1871530322666220208111837. Epub [PubMed PMID: 35135457]
Vassallo RR. Review: IgA anaphylactic transfusion reactions. Part I. Laboratory diagnosis, incidence, and supply of IgA-deficient products. Immunohematology. 2004:20(4):226-33 [PubMed PMID: 15679454]
Dubois C, Ducas É, Laforce-Lavoie A, Robidoux J, Delorme A, Live LS, Brouard D, Masson JF. A portable surface plasmon resonance (SPR) sensor for the detection of immunoglobulin A in plasma. Transfusion. 2024 May:64(5):881-892. doi: 10.1111/trf.17818. Epub 2024 Apr 9 [PubMed PMID: 38591151]
Lieberman P, Nicklas RA, Oppenheimer J, Kemp SF, Lang DM, Bernstein DI, Bernstein JA, Burks AW, Feldweg AM, Fink JN, Greenberger PA, Golden DB, James JM, Kemp SF, Ledford DK, Lieberman P, Sheffer AL, Bernstein DI, Blessing-Moore J, Cox L, Khan DA, Lang D, Nicklas RA, Oppenheimer J, Portnoy JM, Randolph C, Schuller DE, Spector SL, Tilles S, Wallace D. The diagnosis and management of anaphylaxis practice parameter: 2010 update. The Journal of allergy and clinical immunology. 2010 Sep:126(3):477-80.e1-42. doi: 10.1016/j.jaci.2010.06.022. Epub 2010 Aug 7 [PubMed PMID: 20692689]
Shaker MS, Wallace DV, Golden DBK, Oppenheimer J, Bernstein JA, Campbell RL, Dinakar C, Ellis A, Greenhawt M, Khan DA, Lang DM, Lang ES, Lieberman JA, Portnoy J, Rank MA, Stukus DR, Wang J, Collaborators, Riblet N, Bobrownicki AMP, Bontrager T, Dusin J, Foley J, Frederick B, Fregene E, Hellerstedt S, Hassan F, Hess K, Horner C, Huntington K, Kasireddy P, Keeler D, Kim B, Lieberman P, Lindhorst E, McEnany F, Milbank J, Murphy H, Pando O, Patel AK, Ratliff N, Rhodes R, Robertson K, Scott H, Snell A, Sullivan R, Trivedi V, Wickham A, Chief Editors, Shaker MS, Wallace DV, Workgroup Contributors, Shaker MS, Wallace DV, Bernstein JA, Campbell RL, Dinakar C, Ellis A, Golden DBK, Greenhawt M, Lieberman JA, Rank MA, Stukus DR, Wang J, Joint Task Force on Practice Parameters Reviewers, Shaker MS, Wallace DV, Golden DBK, Bernstein JA, Dinakar C, Ellis A, Greenhawt M, Horner C, Khan DA, Lieberman JA, Oppenheimer J, Rank MA, Shaker MS, Stukus DR, Wang J. Anaphylaxis-a 2020 practice parameter update, systematic review, and Grading of Recommendations, Assessment, Development and Evaluation (GRADE) analysis. The Journal of allergy and clinical immunology. 2020 Apr:145(4):1082-1123. doi: 10.1016/j.jaci.2020.01.017. Epub 2020 Jan 28 [PubMed PMID: 32001253]
Level 1 (high-level) evidencePourmand A, Robinson C, Syed W, Mazer-Amirshahi M. Biphasic anaphylaxis: A review of the literature and implications for emergency management. The American journal of emergency medicine. 2018 Aug:36(8):1480-1485. doi: 10.1016/j.ajem.2018.05.009. Epub 2018 May 9 [PubMed PMID: 29759531]
Lee J, Rodio B, Lavelle J, Lewis MO, English R, Hadley S, Molnar J, Jacobstein C, Cianferoni A, Spergel J, Zielinski L, Tsarouhas N, Brown-Whitehorn T. Improving Anaphylaxis Care: The Impact of a Clinical Pathway. Pediatrics. 2018 May:141(5):. pii: e20171616. doi: 10.1542/peds.2017-1616. Epub 2018 Apr 3 [PubMed PMID: 29615480]
Davenport RD, Burnie KL, Barr RM. Transfusion management of patients with IgA deficiency and anti-IgA during liver transplantation. Vox sanguinis. 1992:63(4):247-50 [PubMed PMID: 1481472]
Sandler SG. How I manage patients suspected of having had an IgA anaphylactic transfusion reaction. Transfusion. 2006 Jan:46(1):10-3 [PubMed PMID: 16398725]
Eijkhout HW, van den Broek PJ, van der Meer JW. Substitution therapy in immunodeficient patients with anti-IgA antibodies or severe adverse reactions to previous immunoglobulin therapy. The Netherlands journal of medicine. 2003 Jun:61(6):213-7 [PubMed PMID: 12948165]
Level 2 (mid-level) evidenceLieberman P, Rachid R. Administration of intravenous immunoglobulin to a patient with hypogammaglobulinemia and anti-IgA antibodies. The journal of allergy and clinical immunology. In practice. 2013 Nov-Dec:1(6):704. doi: 10.1016/j.jaip.2013.05.014. Epub 2013 Jun 22 [PubMed PMID: 24565726]
Kiani-Alikhan S, Yong PF, Grosse-Kreul D, Height SE, Mijovic A, Suddle AR, Ibrahim MA. Successful desensitization to immunoglobulin A in a case of transfusion-related anaphylaxis. Transfusion. 2010 Sep:50(9):1897-901. doi: 10.1111/j.1537-2995.2010.02662.x. Epub [PubMed PMID: 20456696]
Level 3 (low-level) evidenceAmeratunga R, Leung E, Woon ST, Lea E, Allan C, Chan L, Steele R, Lehnert K, Longhurst H. Selective IgA Deficiency May Be an Underrecognized Risk Factor for Severe COVID-19. The journal of allergy and clinical immunology. In practice. 2023 Jan:11(1):181-186. doi: 10.1016/j.jaip.2022.10.002. Epub 2022 Oct 12 [PubMed PMID: 36241155]