The outlook depends on the underlying cause of the disease and whether symptoms are managed appropriately and in a timely manner. Death as a result of autoimmune hemolytic anemia is rare. Find a Specialist Find a Specialist. Healthcare Resources To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself.
You can also learn more about genetic consultations from MedlinePlus Genetics. Related Diseases Related Diseases. Conditions with similar signs and symptoms from Orphanet. Biological differential diagnoses include other non-autoimmune causes of hemolytic anemia. Visit the Orphanet disease page for more information.
Research Research. Clinical Research Resources ClinicalTrials. Click on the link to go to ClinicalTrials. Please note: Studies listed on the ClinicalTrials. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
Patient Registry The Autoimmune Registry supports research for Autoimmune hemolytic anemia by collecting information about patients with this and other autoimmune diseases.
You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries. Organizations Organizations. Organizations Supporting this Disease. Organizations Providing General Support. Do you know of an organization? Learn More Learn More. Where to Start MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them. In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers.
This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. PubMed is a searchable database of medical literature and lists journal articles that discuss Autoimmune hemolytic anemia.
Click on the link to view a sample search on this topic. Submit a new question What causes autoimmune hemolytic anemia? See answer What is the outlook for people with autoimmune hemolytic anemia? Patients with mild asymptomatic anemia can be monitored without specific treatment, which should be reserved for symptomatic anemia, severe circulatory symptoms, or transfusion dependence.
Because exacerbations can be transient, a period of support with transfusion may be useful before determining the need for pharmacological intervention. Unfortunately, the median response duration was only 11 months Table 3 , although 6 of 10 responding patients also responded to a second course.
Rituximab has been combined with bendamustine BR or fludarabine in 2 prospective uncontrolled studies Table 3. Although the studies are not directly comparable, the response criteria used were the same, and there appeared to be a higher rate of complete remission and fewer infections in patients receiving BR. Combination therapy provides more opportunity for a durable response but with a greater risk for toxicity.
BR is a suitable second-line option for fit patients or a first-line option in selected patients with severe disease. Response was sustained in 4 of 6 patients at a median of 16 months range, Although further studies are needed, therapeutic options for CAD are limited, and bortezomib may be a suitable second-line option in patients who are not fit for combination therapy, such as BR or rituximab plus fludarabine.
CAD patients usually take several months to respond to rituximab-based therapy Table 3 , whereas hemolysis can be severe and intravascular. In addition to blood transfusion, daily or alternative-day plasma exchange of 1 to 1. Although not recommended for routine treatment of CAD, steroids may have a limited role as rescue therapy in severe cases. Patients with acute severe intravascular hemolysis may also respond to the C5 inhibitor eculizumab, which blocks the terminal complement pathway.
Its activity in CAD is supported by case reports and a prospective study of 13 patients who received 6 months of treatment, with a reduction in transfusion requirements and LDH. Given the mechanism of hemolysis outlined previously, inhibition of the classical complement pathway is a rational and promising therapeutic approach for CAD.
Treatment would need to be maintained to have a continuing effect and is not expected to improve cold-associated symptoms, which result from agglutination of antibody-bound red cells upstream of complement activation.
On-going responses were observed in a named patient program using a fixed biweekly dose of 5. Many more complement inhibitors are under development for various clinical indications and have been recently reviewed.
Given the clonal nature of CAD, newer targeted therapies for B-cell malignancies, such as the BTK inhibitor ibrutinib and B-cell receptor pathway—modulating drugs eg, idelalisib and venetoclax might have activity in CAD, although the hypothesis awaits investigation.
National Center for Biotechnology Information , U. Anita Hill and Quentin A. Author information Copyright and License information Disclaimer. Corresponding author. Conflict-of-interest disclosure: A. All rights reserved. This article has been cited by other articles in PMC. Abstract The diagnosis of autoimmune hemolytic anemia AIHA can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis and then determine the immune nature of hemolysis with the direct anti-globulin test.
Learning Objectives Understand how AIHA and its subtypes can be diagnosed through a combination of clinical assessment and laboratory tests Describe the latest advances in treatment of primary warm AIHA and cold agglutinin disease.
Table 1. Investigations in patients presenting with AIHA. Open in a separate window. Figure 1. Table 2. Causes of intravascular hemolysis. Figure 2. CAD patients In CAD patients, intravascular hemolysis can be triggered by bacterial or other febrile illnesses and should be treated promptly. Primary warm AIHA Although most patients require treatment, watchful waiting may be appropriate in a minority of patients with mild asymptomatic anemia.
Future prospects Future prospects for primary warm AIHA include inhibition of the following: B cells with alternative anti-CD20 monoclonal antibodies mAbs eg, obinutuzumab Mammalian target of rapamycin eg, sirolimus Proteasomes eg, bortezomib Spleen tyrosine kinase Syk eg, fostamatinib Neonatal Fc receptor FcRn Bruton tyrosine kinase BTK Complement pathways Following the success of rituximab, additional anti-CD20 mAbs were developed, primarily to target B-cell malignancies.
Primary CAD Patients with mild asymptomatic anemia can be monitored without specific treatment, which should be reserved for symptomatic anemia, severe circulatory symptoms, or transfusion dependence. Comparison of Nordic studies of rituximab and rituximab combination therapies for CAD.
Response criteria for all 3 studies were the same. Emergency treatment CAD patients usually take several months to respond to rituximab-based therapy Table 3 , whereas hemolysis can be severe and intravascular. Future prospects Given the mechanism of hemolysis outlined previously, inhibition of the classical complement pathway is a rational and promising therapeutic approach for CAD. References 1. Variability of the erythropoietic response in autoimmune hemolytic anemia: analysis of cases.
Lessons from very severe, refractory, and fatal primary autoimmune hemolytic anemias. Am J Hematol. Free access to newly published articles. Purchase access. Rent article Rent this article from DeepDyve. Access to free article PDF downloads. Save your search. Customize your interests. Create a personal account or sign in to:. Privacy Policy. Try out PMC Labs and tell us what you think. Learn More. Warm antibodies capable of agglutinating and haemolysing enzyme-treated cells only are described.
It is shown that they form a separate antibody category among the red cell autoantibodies. Their serological behaviour and immunochemical characteristics as well as their significance in vivo are studied and discussed.
Full text is available as a scanned copy of the original print version. Get a printable copy PDF file of the complete article 1. Links to PubMed are also available for Selected References. These references are in PubMed.
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