HEMOLYTIC DESORDERSHEMOLYTIC DESORDERS

Red Cell Turnover and Life Span Red Cell Turnover and Life Span 2.5 million red cells are removed from 2.5 million red cells are removed from

the circulation every second. the circulation every second. BM produces 200 billion new red cells BM produces 200 billion new red cells

(reticulocytes) each day. These cell (reticulocytes) each day. These cell survived for 120 days before they are survived for 120 days before they are

removed by the RES ( BM, liver, removed by the RES ( BM, liver, spleen)spleen)..

CLASSIFICATIONCLASSIFICATION

1.Acute versus chronic .1.Acute versus chronic .

3.3.Intra-vascular versus extra-vascular.Intra-vascular versus extra-vascular.

4.Intra-corpuscular versus extra-corpuscular 4.Intra-corpuscular versus extra-corpuscular . .

•22..Acquired versus congenitalAcquired versus congenital . .

Definition Definition

HA is a decrease in the total number of circulating HA is a decrease in the total number of circulating eerythrocytes that is caused by the prematurerythrocytes that is caused by the premature ddestruction or removal of red cells from the estruction or removal of red cells from the circulation.circulation.

Anaemia will result only if the rate of RBC Anaemia will result only if the rate of RBCdestruction exceed the BM response (un compensation)destruction exceed the BM response (un compensation)..

• HEMOLYTIC ANAEMIA

Clinical featuresClinical features

Chronic congenital HA•Anaemia

Jaundice Crisis

SplenomegalyGall stonesLeg ulcers

Skeletal abnormalities

Acute (Acquired) HA•sudden pallor

JaundiceTachycardiaAching pain,headache,

malaise, vomiting

, shaking chills and fever.

Manifestation of the underlying disease.

Laboratory manifestationLaboratory manifestation•I. signs of excessive RBC destruction:

Decrease RBC life span Increase 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 Signs of intra-vascular hemolysishemolysis

•Hemoglobinaenemia. Hemoglobinaenemia. Hemoglobinuria. Hemoglobinuria.

Haemosiderinuria. Haemosiderinuria. Met-heme-albuminaemia. Met-heme-albuminaemia.

hemopexin hemopexin Decrease Decrease

•Decrease Hb levelDecrease Hb level..

II. signs of acceleratedII. signs of accelerated erythropoiesiserythropoiesis •Blood

Reticulocytosis (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. Differential diagnosis. TheThein DDin DD III.Lab tests usefulIII.Lab tests useful

•Morphology(blood film findingsMorphology(blood film findings)) : : (spherocytes, (spherocytes, elliptocytes, acanthocytes, stomatocytes, target cells, fragmented elliptocytes, acanthocytes, stomatocytes, target cells, fragmented

RBCs, Autoagglutination)RBCs, Autoagglutination) Direct coomb’s test (Direct anti-human globulin-DAT) Direct coomb’s test (Direct anti-human globulin-DAT) . .

Osmotic fragility testOsmotic fragility test

Auto-hemolysis test.Auto-hemolysis test.Hb-Hb-electorphoresis test .electorphoresis test .

Screening test for G6PD deficiencyScreening test for G6PD deficiencySickling testSickling test. .

DIRECT ANTIHUMAN GLOBULIN (DAT)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 test Indirect antiglobulin test

Differential Diagnosis Of Hemolytic Differential Diagnosis Of Hemolytic Anaemia 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.