Ina AIHA 3.57.51 PM

Ina AIHA 3.57.51 PM - AUTOIMMUNE HAEMOLYTIC ANAEMIA ANAEMIA...

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AUTOIMMUNE HAEMOLYTIC AUTOIMMUNE HAEMOLYTIC ANAEMIA ANAEMIA American Journal of Hematology 69:258-271 (2002) Bradley C. Gehrs and Richard C. Friedberg University of Alabama, Birmingham
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INTRODUCTION Ig G and/or Ig M bind to rbc surface ag – initiate rbc destruction via C system and RES IHA – classification – autoimmune, alloimmune, drug induced AIHA – a/b directed against self rbc
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cont AIHA – incidence 1-3 cases/100,000 per year Auto a/b usually reacted against high frequency ag – exhibit reactivity against allogeneic rbc as well
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Degree of haemolysis depends on : 1. Characteristic of antibody- quantity specificity T ability to fix C ability to bind to tissue machrophage . PATHOGENESIS
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2. target ag – density expression age of pt
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cont cont Ig G a poor activator for classical C p/way Generally Ig G sensitized rbc are eliminated by phagocyte of RES RES also have receptor for C3b and iC3b – can potentiate e/v hemolysis Ig M -- sensitized rbc ass with e/v and i/v hemolysis
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cont cont I/v – Ig M readily activated by classical C p/way Regulatory rbc protein DAF and MIRL – overwhelming C activation is req. to produce clinically evident i/v hemolysis More common Ig M sensitized rbc u/go e/v hemolysis
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cont cont REC have receptor for rbc bound C3b and iC3b resulting fr C activation Spleen – site for Ig G ass e/v hemolysis Liver (kupper cell) – site for Ig M ass e/v
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CLASSIFICATION OF AIHA 1. Warm AIHA Idiopathic Secondary (LPD, A/ Immune) 1. Cold AIHA Cold Agglutinin syndrome PCH 1. Mixed AIHA Idiopathic secondary 1. Drug induced Autoimmune type Drug adsorption type Neoantigen type
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cont cont Classification according to T reactivity of a/b Warm react strongly near 37 c Cold bind rbc strongly near 0-4 c Lymphoproliferative d/o – about half of cases of secondary cold and warm AIHA Idiopathic more in female, peak at 4 and 5 decade
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LAB EVALUATION LAB EVALUATION 2 criteria to dx AIHA – serologic evidence and clinical or lab evidence
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WARM AIHA Intro
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Ina AIHA 3.57.51 PM - AUTOIMMUNE HAEMOLYTIC ANAEMIA ANAEMIA...

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