This document discusses immune hemolytic anemia, including its causes and diagnostic tests. Key points include:
- Immune hemolytic anemia occurs when red blood cells are destroyed by antibodies or complement activation. Common causes are transfusion reactions, autoimmune hemolytic anemia, hemolytic disease of the newborn, and drug-induced hemolytic anemia.
- The Coombs test detects antibodies bound to red blood cells. The direct Coombs test detects in vivo sensitization while the indirect Coombs test detects antibodies in serum/plasma.
- Hemolytic disease of the newborn is caused by maternal antibodies crossing the placenta and destroying fetal red blood cells, commonly due to Rh
This document discusses immune hemolytic anemia, including its causes and diagnostic tests. Key points include:
- Immune hemolytic anemia occurs when red blood cells are destroyed by antibodies or complement activation. Common causes are transfusion reactions, autoimmune hemolytic anemia, hemolytic disease of the newborn, and drug-induced hemolytic anemia.
- The Coombs test detects antibodies bound to red blood cells. The direct Coombs test detects in vivo sensitization while the indirect Coombs test detects antibodies in serum/plasma.
- Hemolytic disease of the newborn is caused by maternal antibodies crossing the placenta and destroying fetal red blood cells, commonly due to Rh
This document discusses immune hemolytic anemia, including its causes and diagnostic tests. Key points include:
- Immune hemolytic anemia occurs when red blood cells are destroyed by antibodies or complement activation. Common causes are transfusion reactions, autoimmune hemolytic anemia, hemolytic disease of the newborn, and drug-induced hemolytic anemia.
- The Coombs test detects antibodies bound to red blood cells. The direct Coombs test detects in vivo sensitization while the indirect Coombs test detects antibodies in serum/plasma.
- Hemolytic disease of the newborn is caused by maternal antibodies crossing the placenta and destroying fetal red blood cells, commonly due to Rh
This document discusses immune hemolytic anemia, including its causes and diagnostic tests. Key points include:
- Immune hemolytic anemia occurs when red blood cells are destroyed by antibodies or complement activation. Common causes are transfusion reactions, autoimmune hemolytic anemia, hemolytic disease of the newborn, and drug-induced hemolytic anemia.
- The Coombs test detects antibodies bound to red blood cells. The direct Coombs test detects in vivo sensitization while the indirect Coombs test detects antibodies in serum/plasma.
- Hemolytic disease of the newborn is caused by maternal antibodies crossing the placenta and destroying fetal red blood cells, commonly due to Rh
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Anemia Hemolitik Autoimun
Dr. Efrida, dr., SpPK(K)., MKes
IMMUNE HEMOLYTIC ANEMIA Red cells react with antibody with or without complement activation and are consequently destroyed. IgG-coated red cells interact with the Fc receptors on macrophages and are either completely or partially phagocytosed. When the phagocytosis is partial, then the unphagocytosed part of the cell may detach from the macrophage and circulates as a spherocyte. Activation of complement proceeds as far as deposition of the membrane attack complex ( C5-C9) which results in intravascular hemolysis. The immune hemolytic anemias include transfusion reactions, auto-immune hemolytic anemia (AIHA), hemolytic disease of the newborn (HDN), and drug induced hemolytic anemia COOMBS’ TEST
Coombs’ test is used to detect red cells antibodies.
Coombs’ reagent is Anti Human Globulin (AHG) from the serum of horses or rabbits which are injected with human globulin. DIRECT COOMBS’ TEST: Detects antibodies or complement on the surface of red cells which are sensitized in-vivo. Red cells suspension 40-60% are directly mixed with Coombs’ reagent. The test is positive if agglutination of red cells occurs. Positive test in AIHA, HDN, Drug induced HA, transfusion reaction due to ABO incompatibility. INDIRECT COOMBS’ TEST Detects red cells antibodies in the serum or plasma. The test is conducted in 2 phases: * Normal red cells suspension 2% is incubated with patient’s serum for 2 hours at 370 C. Then the red cells are washed 3 times with NaCL 0.9% solution. * Washed red cells suspension 40-60% are mixed with Coombs’ reagent. The test is positive if agglutination occurs. Positive test is found in Pregnant women with incompatibility of fetus blood group Incompatibility of donor’s red cells in cross match. HEMOLYTIC DISEASE OF THE NEWBORN HDN is a consequence of the passage of fetal red cells across the placenta resulting in the immunization of the mother to blood group antigens of the fetus. The iso-antibodies produced in the mother are transferred back across the placenta (only IgG are transferred) to the fetus causing red cells destruction of the fetus. Transplacental hemorrhage most commonly occures at the time of labor. Almost all cases of HDN (93%) are caused by the anti-D of the Rh system, 3% due to other antibodies within the Rh system and only 1% due to incompatibility of the ABO blood group. HDN due to ANTI-D Small numbers of fetal red cells (Rh positive) can be found in the maternal circulation (Rh negative) especially during the third trimester, but the main transplacental passage occurs at the time of labor. It is very unusual for the first-born child to be affected with HDN (incidence < 1%). Sensitation may be due to previous abortion or transfusion with Rh positive red cells. Neonatus may be born with mild anemia or severe conditions. The baby could be with severe anemia, icterus, pallor, tachy- cardia, edema, and hepato-splenomegaly. If the serum bilirubin exceeds 30 mg/dl, the pigment will be deposited in the cells of basal ganglia resulting in KERN ICTERUS. In extreme conditions the baby will be born with hydrops fetalis or stillbirth (IUFD) occurs. Laboratory findings of Rh HDN: Decreased Hb (normal Hb in newborns 21-27 g/dl) Reticulocytosis > 6% Normoblastosis in blood film 10.000-100.000/mm3 (normally 200-2000/mm3 , or < 6 in 100 leucocytes ) Negative spherocyte in blood film Leucocytosis > 20.000 - 30.000 / mm3 (normally 15.000-20.000/mm3 ) Increased indirect bilirubin Positive direct Coombs’ test of cord blood
Therapy of newborns: Exchange transfusion and photo-therapy.
ABO – HDN Occurs in newborns with A or B blood group from mothers of O blood group. IgM antibodies occurs in mothers with other blood groups, these antibodies are not transferred by the placenta. Mild anemia occurs in the newborn Spherocytosis in blood film Increased indirect bilirubin Negative kern icterus AUTO-IMMUNE HEMOLYTIC ANEMIA (AIHA)
Antibodies against antigens on the membrane of red cells.
WARM AIHA Reaction of Ag-Ab occurs optimally at 37o C The red cells are mostly coated with IgG or IgG + complement, only a small part of the antibodies are IgM or IgA. Warm AIHA occures in Primary – idiopathic Secondary in Auto-immune disease (e.g. SLE), and CLL Drug induced HA COLD AIHA Reaction of Ag-Ab occurs optimally at < 310 C The red cells are mostly IgM antibodies, involved complement Cold AIHA occures in Primary idiopathic Secondary in Lymphoma, Infectious mononucleosis, and Mycoplasma pneumonia Paroxysmal Cold Hemoglobinuria (PCH) Laboratory findings in AIHA: * Positive Coombs’ test * Evidence of intra- or extra- vascular hemolysis * Morphology of blood film: normochrom normocytair spherocyte schistocyte (fragmentocyte) erythro-phagocytosis in monocyte * In PCH: positive Donath Landsteiner antibody DRUG INDUCED HEMOLYTIC ANEMIA 1. Antigens as a drug-membrane complex on the red cells IgG antibodies Extravascular hemolysis with positive direct Coombs’ test Occurs in - high dose penicillin therapy - cephalotin therapy 2. Antigens as drug-protein complex, needs a complement. IgM antibodies Intravascular hemolysis with positive direct Coombs’ test Occurs in : stibophen, phenacetin, or quinine therapy 3. Therapy with high dose methyldopa. Mechanism of HA unknown. PAROXYSMAL NOCTURNAL Hb-URIA (PNH) Defect of membrane of red cells i.e. deficiency of Glycosyl Phosphatidyl Inositol (GPI). Red cells become sensitive to lowered pH, usually occurs during sleep and hemolysis occurs intravascularly. Signs of Hb-uria, hemosiderinuria and methemalbuminemia. Specific test in PNH: * Sugar water test * Acid serum test = Ham test Complications: - Aplastic anemia - Intravascular thrombosis (can be fatal) - Iron deficiency anemia Pemeriksaan Laboratorium (deteksi dan etiologi) • CBC termasuk retikulosit • Urinalisis lengkap • Bilirubin total, direk, indirek • LDH • Haptoglobin • Tes Coomb Direk, Indirek Cont… • Ig (+) dan perlekatan Ab pada permukaan eritrositpenanda anemia hemolitik autoimun • Aglutinasi spontan sel yg dilapisi IgM (Ab komplit) • Sel yg dilapisi IgG tdk beraglutinasi spontan dalam larutan saline ( Ab inkomplit)
