HEMOLYTIC DESORDERS Red Cell Turnover and Life Span 2.5 million red cells are removed from the circulation every second. BM produces 200 billion new red.

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Presentation transcript:

HEMOLYTIC DESORDERS Red Cell Turnover and Life Span 2.5 million red cells are removed from the circulation every second. BM produces 200 billion new red cells (reticulocytes) each day. These cell survived for 120 days before they are removed by the RES ( BM, liver, spleen).

CLASSIFICATION 1.Acute versus chronic. 3. Intra-vascular versus extra-vascular. 4.Intra-corpuscular versus extra-corpuscular. 2.Acquired versus congenital.2.Acquired versus congenital.

Definition HA is a decrease in the total number of circulating e rythrocytes that is caused by the premature d estruction or removal of red cells from the circulation. Anaemia will result only if the rate of RBC destruction exceed the BM response (un compensation). HEMOLYTIC ANAEMIA

Clinical features Chronic congenital HA Anaemia Jaundice Crisis Splenomegaly Gall stones Leg ulcers Skeletal abnormalities Acute (Acquired) HA sudden pallor Jaundice Tachycardia Aching pain, headache, malaise, vomiting, shaking chills and fever. Manifestation of the underlying disease.

Laboratory manifestation I. signs of excessive RBC destruction: Decrease RBC life span I ncrease catabolism of heme. indirect hyperbilirubinaemia. increase rate of bilirubin production. increase rate of urobilinogen production increase LDH activity. Absence of serum haptoglobin

Signs of intra-vascular hemolysis Hemoglobinaenemia. Hemoglobinuria. Haemosiderinuria. Met-heme-albuminaemia. hemopexin DecreaseHemoglobinaenemia. Hemoglobinuria. Haemosiderinuria. Met-heme-albuminaemia. hemopexin Decrease Decrease Hb level.Decrease Hb level.

II. signs of accelerated erythropoiesis II. signs of accelerated erythropoiesis Blood R eticulocytosis (polychromasia in the blood film). Macrosytosis. Normoblastaemia. Leukocytosis and thrombocytosis. Bone marrow. Erythroid hyperplasia. Ferrokinetics: increase plasma iron turnover. increase erythrocyte iron turnover

Differential diagnosis. The in DD III.Lab tests useful Morphology(blood film findings) : (spherocytes, elliptocytes, acanthocytes, stomatocytes, target cells, fragmented RBCs, Autoagglutination) Direct coomb’s test (Direct anti-human globulin-DAT). Osmotic fragility test Auto-hemolysis test. Hb-electorphoresis test. Screening test for G6PD deficiency Sickling test.Morphology(blood film findings) : (spherocytes, elliptocytes, acanthocytes, stomatocytes, target cells, fragmented RBCs, Autoagglutination) Direct coomb’s test (Direct anti-human globulin-DAT). Osmotic fragility test Auto-hemolysis test. Hb-electorphoresis test. Screening test for G6PD deficiency Sickling test.

DIRECT ANTIHUMAN GLOBULIN (DAT) Testing the patient RBC for their invivo sensitization. It is used in ; 1.Transfusion reaction, 2. Hemolytic disease of the newborn. 3. Auto immune hemolytic anaemia(AIHA) 4.Drug-induced hemolytic anaemia. INDIRECT ANTI-HUMAN GLOULIN TEST (IAT) Testing the patient serum for the presence of irregular antibodies (Allo); 1.Part of cross matching. 2.Antibody screening & identification. 3.Titration of antibodies.

Direct antiglobulin testIndirect antiglobulin test

Differential Diagnosis Of Hemolytic Anaemia 1.Anaemia with increase Reticulocytes: a. Haemorrage b.Recovery from deficiency of iron, B12, folate. c. Recovery from marrow failure as in cessation of alcohol cosumption. 2.Anaemia with acholuric jaundice; a.Ineffective erythropoiesis. b. Loss of blood in to body cavity. 3.Acholuric jaundice without anaemia. 4.Marrow invasion. 5.myoglobulinuria.