Professional Documents
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Autoimmune Hemolytic Anemia
Autoimmune Hemolytic Anemia
Autoimmune Hemolytic Anemia
Hemolytic Anemias
Donald R. Branch, Ph.D.
Scientist
Research & Development
Canadian Blood Services
Toronto, Ontario CANADA
don.branch@utoronto.ca
Lecture Outline
No Need to be Terrified
DAT!
Agglutinated Cells
Spherocytosis
Neutrophil Erythrophagocytosis
DIRECT
ANTIGLOBULIN
TEST
Test for IgG and/or complement coating the patients red blood cells
Classification of Immune
Hemolytic Anemias
Alloimmune
HTR
DHTR
HDFN
Autoimmune hemolytic anemias (AIHAs)
DAT-positive
DAT-negative
Drug-induced
Autoimmune
Drug-adsorption
Immune-complex
Bystander Immune Cytolysis
Sickle cell hemolytic transfusion reaction syndrome
Reactive hemolysis
Classification of
AIHAs
2.
3.
Mixed AIHA
5.
6.
7.
(Shulman IA, Branch DR, et al. Autoimmune hemolytic anemia with both cold
and warm autoantibodies. JAMA 253:1746-1748, 1985)
Erythrophagocytosis in PCH
The Donath-Landsteiner
Test
Characteristics of Typical D-L Antibodies
Biphasic Hemolysin
IgG Immunoglobulin Class
Anti-P Specificity
May also react by IAT
P + complement
DAT-negative AIHA
Rare
Often severe - fatal
Sometimes anti-IgA/anti-IgM useful
Mononuclear phagocyte assay may be
useful use patient monocytes
Often responds to steroid treatment
2.
3.
4.
Specificity of Warm
Autoantibodies
Not Usually Necessary Academic Exercise
Anti-Rh-like specificity
Anti-e or anti-E (SIMPLE SPECIFICITY)
Anti-pdl does not react with D- or Rh null
Anti-dl does not react with Rhnull
Relative Rh specificity titration studies
Type I or Type II Autoantibodies
Other blood groups Kell, Gerbich (high
frequency antigens)
Relative Specificity
A Guide to Transfusion
of Patients with
Autoimmune
Hemolytic Anemia
Important Principles
Blood Transfusion in
Autoimmune Hemolytic
Anemia
Blood should never be denied a
patient with a justifiable need,
even though the compatibility test
may be strongly positive.
Probably the most common
mistake is reluctance to
transfuse even those patients
with severe anemia.
Initiate communication.
Indicate extent of compatibility testing
performed, e.g., auto- or alloadsorption.
Clinician should be assured that, after
appropriate compatibility testing, an acute HTR
is unlikely.
Indicate that RBCs will provide temporary
benefit even if they do not survive normally
because of the patients autoantibody.
situation.
Understand principles of compatibility
testing.
Seek assurance that appropriate
compatibility testing is to be
performed.
ABO and Rh
Spontaneous Agglutination
IgG auto - Treat cells with ZZAP to remove autoantibody and retype
IgM auto warm everything to 37C and retype or ZZAP treat and retype
(Branch DR, Petz LD. A new reagent (ZZAP) having multiple applications in
immunohematology. Am J Clin Pathol. 78:161-167, 1982)
TOTALS:
209/ 647
% OF SERA
WITH ALLOABS
40
36
15
38
32
38
12
40 _______
32%
(Branch DR, Petz LD: Detecting alloantibodies in patients with autoantibodies. Transfusion 39:6-
Adsorption Procedures
ZZAP!!!!!!!
Allogeneic Adsorption
1.
2.
R1R1
R2R2
rr
One cell Jk(a-)
Another cell Jk(b-)
Use ZZAP to denature other important
antigens
Allogeneic Adsorption
Mixed AIHA
PCH
Larry Petz
Don Branch
Acknowledgements
Larry Petz
Phyllis Morel
George Garratty
Jean-Michel Turc
Canadian Blood Services
don.branch@utoronto.ca