Immune Hemolytic Anemia

Etiology of Immune Hemolytic Anemia

Auto Immune
Warm Auto Antibody Type Cold Auto Antibody Type Paroxysmal Cold Hemoglobinury Drug-Induced Hemolytic Anemia Alloimmune Hemolysis Hemolytic Transfusion Reaction Hemolytic Disease of The Newborn Idiophatic Secondary

Immune Hemolytic Anemia

Idiophatic Warm Auto Antibody Type Secondary

Lymphoproliferative Disease : Leukimia, lymphoma maligna, etc Drugs

Auto Immune
SLE Idiophatic Cold Auto Antibody Type Secondary Lymphoproliferative Disease Infection : Mycoplasma pneumonia, infectius mononucleus, EBV, etc Syphilis Secondary Pasca viral infection (self-limited)

Idiophatic Paroxysmal Cold Hemoglobinury

Epidemiology Of Immune Hemolytic Anemia

1. Auto Immune Hemolytic Anemia
In indonesia, there is no data specifically on the prevalence and incidence AIHA. Warm antibody type = 1-2/100.000 population Warm antibody type any age, prevalence = 70% Cold antibody type in all middle age and eldery, prevalence = less than warm antibody type

Epidemiology
2. Paroxysmal Cold Hemoglobinury Rare case 0.4 cases per 100.000 people The male-to-female ratio is 2:1 to 5:1 Acute affects mostly young children, commonly following an acute viral or upper respiratory illness. Chronic commonly seen in the elderly. Contributory secondary causes are generally neoplastic in origin, followed by infections.

3. Drug Related Hemolylisis Annual incidence is estimated at about 1/1,000,000 About 100 drugs have been implicated in drug-induced AIHA, including: cephalosporins (primarily cefotetan and ceftriaxone), levodopa, methyldopa, penicillin and its derivatives, quinidine and some nonsteroidal anti-inflammatory drugs

Pathogenesis of Auto Immune Hemolytic Disease (AIHA)

Warm : IgG

Erythrocyte

Antibody
Cold : IgM Ag-Ab complex Classical pathway Alternative pathway Ekstravascular Hemolysis Membran attacking complex

Activate complement system

Intravascular Hemolysis

Immune Adherence (cellular Activation)

Pathogenesis of Paroxysmal Cold Hemoglobinury

Extreme Condition (Cold Temperature)

Donath-Landsteiner Autoantibody (IgG spesific)

Erythrocyte

Ag-Ab Complex Temperature back to normal (370C)

Lysis of Erytrocyte

Pathogenesis of Drug-Induced immune Hemolytic Anemia (DIIHA)

Drugs

Hapten

Drug coated erythrocytes strongly

formed antibodies to the drug

reaction to the drug on the surface of erythrocytes Erythrocyte are destroy by the spleen

Absorp in GIT

Form Ternery complex

Drug-Erythrocyte complex are weak

Antibody bind to that complex Bond become strong

Drug induce formation of antibody to erythrocyte

Activate complement

Drug cause oxidative hemolysis of erythrocyte

Hemolysis

Clinical Manifestations
1. AIHA Warm Auto Antibody Type anemia occurs slowly + icterus + fever attack occurs suddenly, abdominal pain and severe anemia 40 % icterus Hemoglobinury 25 % not accompanied by organomegaly

Cold Auto Antibody Type In a cold temperature aglutination Chronic hemolysis Mild anemia = Hb 9-12 g/dL Acrocyanosis persistent blue or cyanotic discoloration of the extremities, most commonly occurring in the hands Splenomegaly

2. Paroxysmal Cold Hemoglobinury AIHA = 2-5% Myalgia Fever Hemoglobinury for a few hours Urticaria

3. Drug-Induced immune Hemolytic Anemia (DIIHA) History of drug use (+) Occur mildmoderate anemia Hapten / autoantibody Severe anemia ternery complex When previously exposed to the drug, a single dose can occur hemolysis immediately