Auto Immune Hemolytic Anemia Flashcards
What is the autoantibody production theory?
Autoantibody production is usually prevented by feedback mechanism. Suppressor T-Cells induce tolerance to “self” antigens by inhibiting B-cell activity. If T-supressor cells lose function ten autoantibody production results
What is the diagnostic test for autoimmune hemolytic anemia?
Positive DAT (pos autocontrol and Rh control also)
Appearance of positive DATs for autoantibodies due to transfusion reactions
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Appearance of positive DATs for alloantibodies due to transfusion reactions
Recipient antibody attaches to transfused donor cells with corresponding
Appearance of alloantibodies due to HDFN
Mother’s IgG antibody crosses the placenta and attaches to the baby’s paternally derived antigens
Autoanti-I
- who makes the autoantibodies
Everyone?
Autoanti-I
- Expected test reactions with Adult vs Cord Cells
Reacts with adult cells
non reactive with Cord cells
Autoanti-H
- Who makes the autoantibodies
Seen in group A1 and A1B patients, whose cells have most of the “H” antigen covered up
Autoanti-H
- Expected test reactions with A1 vs O cell
Strongly reactive with group O cells and non-reactive with A1 or A1B
CHD
- Cause
Caused by anti-I (very high titers and greater thermal amplitude)
CHD
- Symptoms
- Acrocyanosis
- Numbness in extremities
- Raynaud’s syndrome
- hemoglobinurea (in some)
- auto agglutination of blood at RT
CHD
- Lab results
- hemoglobinurea (in some)
- high titer anti-I at 4C
CHD
- Reactivity Temperature
Cold! 4C
CHD
- Treatment
Keep warm/avoid the cold
- if needs transfusion, transfuse blood through blood warmer
CHD
- DAT
complement only
CHD
- D-L test result (pos/neg)
Negative
CHD
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CHD
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CHD
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PCH
- What does it stand for?
Paroxysmal Cold Hemoglobinurea
PCH
- Antibody specificity
IgG antibody (Anti-P; Biphasic autohemolysin) binds to patients cells at low temps, fixes complement, which leads to intravascular hemolysis of cells at 37C
PCH
- Diagnostic test and what is the result?
Donath-Landsteiner Test
- Positive
PCH
- symptoms
symptoms often follow viral illnesses such as measles, chicken pox, flu, infectious mono
- fever
- shaking chills
- malaise
- abdominal cramps
- back pain
PCH
- Lab findings
- hemoglobinurea
- hemoglobinemia
PCH
- Treatment
Avoid cold exposure
- supportive treatment
PCH
- DAT
Complement only
WAIHA
- what does it stand for
Warm Auto Immune Hemolytic Anemia
WAIHA
- What temperature does it react
37C
WAIHA
- DAT
positive coated with IgG (some may also have complement coating cells
WAIHA
- majority of patients will have _____ and need _____
anemia; transfusions
WAIHA
- Symptoms
pallor, weakness, dizziness, dyspnea, jaundice, unexplained fever
WAIHA
- onset is usually _______
chronic
WAIHA
- DAT
positive (IgG OR IgG and complement)
WAIHA
- Treatment
Patient cells can’t be AHG-antigen typed by routine methods - must treat patient cells with EGA or Chloroquine diphosphate (CDP) to remove the AB coating cells
WAIHA
- Eluate Reactivity
panreactive
WAIHA
- The MOST important part of a work up is what?
is to identify any underlying alloantibodies
- adsorption procedures often needed
WAIHA
- treatment
- cortosteroids
- splenectomy
- Immunosuppressive drugs
- transfusion as a last resort
WAIHA
- IAT
may be positive or negative
- id positive adsorption must be performed to look for underlying alloantibodies
Drug-Induced Autoimmune Hemolytic Anemia
- DAT
positive
Drug-Induced Autoimmune Hemolytic Anemia
- treatment
discontinue drug
Drug-Induced Autoimmune Hemolytic Anemia
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Drug-Induced Autoimmune Hemolytic Anemia
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