Data Registry of Auto Immune Hemolytic Anemia

Summary

In autoimmune hemolytic anemia (AIHA) auto-antibodies directed against red blood cells (RBCs) lead to increased RBC clearance (hemolysis). This can result in a potentially life-threatening anemia. AIHA is a rare disease with an incidence of 1-3 per 100,000 individuals. An unsolved difficulty in diagnosis of AIHA is the laboratory test accuracy. The current 'golden standard' for AIHA is the direct antiglobulin test (DAT). The DAT detects autoantibody- and/or complement-opsonized RBCs. The DAT has insufficient test characteristics since it remains falsely negative in approximate 5-10% of patients with AIHA, whereas a falsely positive DAT can be found in 8% of hospitalized individuals. Also apparently healthy blood donors can have a positive DAT. The consequences of DAT positivity are not well known and may point to early, asymptomatic disease, or to another disease associated with formation of RBC autoantibodies, such as a malignancy or (systemic) autoimmune disease. Currently, there are no guidelines to follow-up DAT positive donors.

A second unsolved difficulty is the choice of treatment in AIHA. Hemolysis can be stopped or at least attenuated with corticosteroids, aiming to inhibit autoantibody production and/or RBC destruction. Many patients do not respond adequately to corticosteroid treatment or develop severe side effects.

Currently, it is advised to avoid RBC transfusions since these may lead to aggravation of hemolysis and RBC alloantibody formation. But in case symptomatic anemia occurs, RBC transfusions need to be given. An evidence-based transfusion strategy for AIHA patients is needed to warrant safe transfusion in this complex patient group.

To design optimal diagnostic testing and (supportive) treatment algorithms, the investigators will study a group well-characterized patients with AIHA and blood donors without AIHA, via a prospective centralized clinical data collection and evaluation of new laboratory tests. With this data the knowledge of the AIHA pathophysiology and to evaluate diagnostic testing in correlation with clinical features and treatment outcome can be improved.

Conditions

• Autoimmune Hemolytic Anemia

Sponsors & Collaborators

• Leiden University Medical Center

  collaborator OTHER

• Radboud University Medical Center

  collaborator OTHER

• UMC Utrecht

  collaborator OTHER

• Maastricht University Medical Center

  collaborator OTHER

• Erasmus Medical Center

  collaborator OTHER

• Haga Hospital

  collaborator OTHER

• Isala

  collaborator OTHER

• Jeroen Bosch Ziekenhuis

  collaborator OTHER

• St. Antonius Hospital

  collaborator OTHER

• Onze Lieve Vrouwe Gasthuis

  collaborator OTHER

• Spaarne Gasthuis

  collaborator OTHER

• Amsterdam University Medical Center

  collaborator OTHER

• Prothya Biosolutions

  collaborator INDUSTRY

• Sanquin Research & Blood Bank Divisions

  lead OTHER

Principal Investigators

• M. De Haas, Prof. MD PhD · Center for Clinical Transfusion Research (CCTR), Sanquin, The Netherlands

• S.S Zeerleder, Prof. MD PhD · University Hospital, University of Bern, Switzerland and Department for BioMedical Research, University of Bern, Switzerland

Eligibility

Min Age

3 Months

Sex

ALL

Healthy Volunteers

Yes

Timeline & Regulatory

Start

2019-07-12

Primary Completion

2024-12-01

Completion

2024-12-01

Countries

• Netherlands

Study Locations