LEC.4 Dr.Khaled
MEDICINE
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 .
2.Acquired versus congenital.
3.Intra-vascular versus extra-vascular.
4.Intra-corpuscular versus extra-corpuscular
•HEMOLYTIC ANAEMIA
Definition
HA is a decrease in the total number of circulating
erythrocytes that is caused by the premature
destruction or removal of red cells from the
circulation.
Anaemia will result only if the rate of RBC
destruction exceed the BM response (un compensation).
Clinical features
Chronic congenital HA Acute (Acquired) HA
Anaemia sudden pallor
Jaundice Jaundice
Crisis Tachycardia
Splenomegaly Aching pain,
Gall stones headache,
Leg ulcers malaise
Skeletal abnormalities , vomiting
, shaking chills and fever
Manifestation of the underlying disease.
Laboratory manifestation
I. signs of excessive RBC destruction:
Decrease RBC life span
Increase catabolism of heme.
indirecthyperbilirubinaemia
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.
Decrease hemopexin
Decrease Hb level.
II. signs of accelerated erythropoiesis
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. 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 testHb-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 test Indirectantiglobulin 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 alcoholcosumption.
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.