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Autoimmune Hemolytic
Anemia (AIHA)
-:Shivam patel
What is AIHA?
 When the increased destruction of erythrocytes due to the
presence of anti-bodies against them,with or without
complement on RBCs membrane.
 As premature cell destruction will lead to anemia 2ndry to
the hemolysed RBCs, and the bone marrow will not be able
to compensate the destructed RBCs by another healthy
RBCs.
Symptoms.
 Onset typically show
 In secondary AIHA, symptoms of
primary illness may overshadow
 Jaundice
 Splenomegaly ~20%
Classification of AIHA.
 Primary Secondary
(RBCs auto antibodies are present (AIHA occurring in association
And cause HA. with another systemic illness
But no evidence often called secondary AIHA.
Underlying systemic illness.
AIHA Subtypes
 Classification based on optimal RBC-autoantibody reactivity
temperatures.
 Warm and Cold AIHA more predominant.
 Warm is IgG (Opsinization) autoantibodies that bind optimally at 37°C.
 Cold is IgM (Complement) that strongly agglutinates at 4°C.
Warm AIHA (wAIHA)
 1 in 80,000
 Predominant type found in children.
 wAIHA – mononucleosis, HIV, and chronic
lymphocytic lymphoma.
 Optimal temperature of 37°C.
 Usually associated with IgG (Also IgA and
IgM).
 IgG bind to the RBCs at 37’C,lead to
extravascular hemolysis in the spleen
 IL-10 and IL-12 play a role in this process.
Cold AIHA (cAIHA)
 Maximal activity of in vitro RBC binding at temp.
between 0’C and 30’C.
 Almost always caused by IgM antibody with
specificity for antigen of system on RBCs.
 Antibodies are usually found in infection
mononucleosis.
 This hemolysis is complement-dependent
manner.
Diagnosis.
 CBC(decrease HB level)
 Jaundice and abnormally dark urine
 Direct antiglobulin test (DAT) or Coombs test (positive)
 Heptoglobin will be decrease
 Indirect bilirubin will increase
 Bone marrow aspiration if you suspect AIHA secondary to leukemia or
lymphoma
 Increased sensitivity fcRBC-bound Ig detection
Treatment.
1. Immunotherapy – the treatment of disease by inducing, enhancing, or
suppressing an immune response
Classified as suppression immunotherapy if the immunotherapy reduces or
suppresses the immune response
2. Corticosteroid in IgG mediated AIHA, Corticosteroid will interfere with Fc
receptor which are responsible for RBC destruction…
3. pulse dose dexamethasone can be effective in warm AIHA…
THANK YOU

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Autoimmune Hemolytic Anemia (AIHA) Guide

  • 2. What is AIHA?  When the increased destruction of erythrocytes due to the presence of anti-bodies against them,with or without complement on RBCs membrane.  As premature cell destruction will lead to anemia 2ndry to the hemolysed RBCs, and the bone marrow will not be able to compensate the destructed RBCs by another healthy RBCs.
  • 3. Symptoms.  Onset typically show  In secondary AIHA, symptoms of primary illness may overshadow  Jaundice  Splenomegaly ~20%
  • 4. Classification of AIHA.  Primary Secondary (RBCs auto antibodies are present (AIHA occurring in association And cause HA. with another systemic illness But no evidence often called secondary AIHA. Underlying systemic illness.
  • 5. AIHA Subtypes  Classification based on optimal RBC-autoantibody reactivity temperatures.  Warm and Cold AIHA more predominant.  Warm is IgG (Opsinization) autoantibodies that bind optimally at 37°C.  Cold is IgM (Complement) that strongly agglutinates at 4°C.
  • 6.
  • 7. Warm AIHA (wAIHA)  1 in 80,000  Predominant type found in children.  wAIHA – mononucleosis, HIV, and chronic lymphocytic lymphoma.  Optimal temperature of 37°C.  Usually associated with IgG (Also IgA and IgM).  IgG bind to the RBCs at 37’C,lead to extravascular hemolysis in the spleen  IL-10 and IL-12 play a role in this process.
  • 8. Cold AIHA (cAIHA)  Maximal activity of in vitro RBC binding at temp. between 0’C and 30’C.  Almost always caused by IgM antibody with specificity for antigen of system on RBCs.  Antibodies are usually found in infection mononucleosis.  This hemolysis is complement-dependent manner.
  • 9. Diagnosis.  CBC(decrease HB level)  Jaundice and abnormally dark urine  Direct antiglobulin test (DAT) or Coombs test (positive)  Heptoglobin will be decrease  Indirect bilirubin will increase  Bone marrow aspiration if you suspect AIHA secondary to leukemia or lymphoma  Increased sensitivity fcRBC-bound Ig detection
  • 10. Treatment. 1. Immunotherapy – the treatment of disease by inducing, enhancing, or suppressing an immune response Classified as suppression immunotherapy if the immunotherapy reduces or suppresses the immune response 2. Corticosteroid in IgG mediated AIHA, Corticosteroid will interfere with Fc receptor which are responsible for RBC destruction… 3. pulse dose dexamethasone can be effective in warm AIHA…

Editor's Notes

  1. Decrease RBCs production so decrease blood flow and increase RBCs destruction
  2. Splenomegaly= enlargement of the spleen
  3. igG Inhibition of B cell activation Mononucleosis= enlarged lymphnodes and swollen spleen may last for a few weeks longer CLL= type of bloodcancer
  4. In chronic cold agglutinin disease, the patient is more symptomatic during the colder months.
  5. Cbc= complete blood count