Lapac MLS 3-A AUTOIMMUNE HEMOLYTIC ANEMIA (AHA) It is a rare disorder characterized by premature RBC destruction and anemia caused by autoantibodies that bind the RBC surface with or without complement activation. (Etiology or Cause) Autoantibodies may arise as a result of immune system dysregulation and loss of immune tolerance, exposure to antigen similar to autoantigen, B lymphocyte neoplasm and others. Characteristics of AHA Warm AHA Cold Paroxysma Mixed Agglutinin l Cold Type AHA Dse Hemoglobi nria Ig class IgG (rarely IgM IgG IgG, IgM IgM, IgA) Optimum 37C 4C, 4C 4-37 C reactivity reactivity temperature extends to > of 30 C autoantibod y Sensitizatio IgG or IgG + C3d C3d IgG and C3d n detected C3d, only by direct C3d antiglobin uncommon test Warm AHA Cold Paroxysma Mixed Agglutinin l Cold Type AHA Dse Hemoglobi nria
Complement Variable Yes Yes Yes
Activation Hemolysis Extravascul Extravascul Intravascula Extravascul ar primarily ar, rarely r ar and intravascula Intravascula r r Autoantibod Panreactive I (most), i P Panreactive, y specificity or Rh (some), Pr unclear complex;rare (rare) specificity ly specific Rh or other antigen WARM AUTOIMMUNE HEMOLYTIC ANEMIA Warm Autoimmune Hemolytic Anemia -most commonly found in adults older than 40 years of age and in children younger than 4 years of age
Classification
Idiopathic- etiology is unknown
Secondary- found in conditions such as lymphoproliferative diseases, nonlymphoid neoplasms, autoimmune disorders, immunodeficiency disorders, viral infections COLD AGGLUTININ DISEASE Cold Agglutinin Disease
Chronic CAD-occurs in middle-aged and
elderly individuals
Acute CAD-occurs secondary to
Mycoplasma pneumoniae infection, infectious mononucleosis and other viral infections PAROXYSMAL COLD HEMOGLOBINURIA Paroxysmal Cold Hemoglobinuria
-commonly seen in young children after a
respiratory infection -rare in adults -anti-P autoantibody also called as Donath- Landsteiner antibody is a complement- binding IgG hemolysin with specificity for the P antigen on RBCs Mixed-Type AHA -occurs very infrequently