Data Registry of Auto Immune Hemolytic Anemia
Data Registry of Auto Immune Hemolytic Anemia
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.
Inclusion Criteria:

 -  Sufficient comprehension of the Dutch language

 -  Signed informed consent by patient and/or parent/caretaker or donor

 -  Patients older than 3 months

 -  Patients with a positive DAT, a positive eluate and signs of hemolysis*

 -  Patients with a positive DAT with complement only, negative eluate, but with signs of
 hemolysis

 -  Donors with a (repeatedly) positive DAT and a positive eluate and/or clinically
 relevant cold auto-antibodies

 Exclusion Criteria:

 -  Prior inclusion in the DRAIHA study