1. Hemolytic Anemias
Defined as those anemias result from an increased in the rate of red cell destruction.
The red cell destruction is usually removed extravascular by macrophages of reticuloendothelial system (RE) [BM, liver & spleen].
Acute destruction or chronic destruction
Extravascular or Intravascular hemolysis
Hereditary or Acquired hemolytic anemia
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2. Classification of hemolytic anemias
Hereditary
Acquired
Membrane
Hereditary spherocytosis, hereditary elliptocytosis
Immune
Autoimmune
Warm antibody type
Metabolism
Cold antibody type
G6PD deficiency, pyruvate kinase deficiency
Alloimmune
Hemolytic transfusion reactions
Hemoglobin
Thalassemia disorders
Hemolytic disease of the newborn
Allografts, especially marrow transplantation
Abnormal (Hb S, Hb C, unstable);
Drug associated
Red cell fragmentation syndromes
Arterial grafts, cardiac valves
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3. Thrombotic thrombocytopenic purpura Hemolytic uraemic syndrome
cont. of Classification of hemolytic anemias
Hereditary
Acquired
Microangiopathic
Thrombotic thrombocytopenic purpura
Hemolytic uraemic syndrome
Meningococcal sepsis
Pre-eclampsia
Disseminated intravascular coagulation
March hemoglobinuria
Infections
Malaria, clostridia
Chemical and physical agents
Especially drugs, industrial/domestic substances, burns
Secondary
Liver and renal disease
Paroxysmal nocturnal hemoglobinuria
G6PD, glucose-6-phosphate dehydrogenase; Hb, hemoglobin.
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4. Diagnosis History Clinical features Laboratory findings
CBC (Hb & MCV), Plt, (WBC & diff)
Retic
Blood film
Specific tests
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5. CBC WBC 8.3 10^3/L NE % 64.5 % LY % 27.6 % MO % 5.1 % EO % 2.6 %
BA % %
NRBC % %
NE # ^3/L
LY # ^3/L
MO # ^3/L
EO # ^3/L
BA # ^3/L
NRBC # ^3/L
RBC ^6/L
HGB g / dL
HCT %
MCV fL
MCH pg
MCHC g / dL
RDW %
PLT ^3/L
MPV fL
RET %
RET #
IRF
MRV
CBC
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6. Laboratory findings in Hemolytic Anemia
Features of increased red cell destruction:
S. bilirubin,  urine urobilinogen breakdown, fecal stercobilinogen, s. haptoglobins absent.
Feature of  red cell productions
Reticulocytosis
BM erythroid hyperplasia
Damaged red cell
Morphology
Osmotic fragility & autohemolysis
Red cell survival
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7. Autoimmune Hemolytic Anemia (AIHAs)
Caused by antibody production by the body against its own red cells.
Characterized by a positive direct antiglobulin test (DAT) called Coombs test
Divided into warm and cold according Abs react at 37oC or 4oC.
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8. Autoimmune Hemolytic Anemia
Warm Type
Cold Type
Idiopathic
Secondary: Systemic lupus
erythematosus, other
connective tissue
disorders
chronic lymphocytic
leukaemia
malignant lymphoma
ulcerative colitis
ovarian teratoma
drugs, e.g. methyldopa
Secondary: Mycoplasma
pneumonia
infectious
mononucleosis
paroxysmal cold
hemoglobinuria:
rare; may be primary
or associated with
infection
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9. Cold Autoimmune Hemolytic Anemia
In these syndromes the antibodies whether:
Monoclonal: Idiopathic cold agglutination lymphomas.
Polyclonal as infection: Infectious mononucleosis, Mycoplasma pneumonia
Patient may have a chronic hemolytic anemia aggravated by cold. Sometimes associated with intravascular hemolysis.
Laboratory:
Similar like warm AIHA
Less spherocyte, more red cell agglutination
DAT reveals complement (C3d)
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10. Causes of intravascular hemolysis
Mismatched blood transfusion (usually ABO)
G6PD deficiency with oxidant stress
Red cell fragmentation syndromes
Some autoimmune hemolytic anemias
Some drug- and infection-induced hemolytic anemias
Paroxysmal nocturnal hemoglobinuria
March hemoglobinuria
Unstable hemoglobin
G6PD, glucose-6-phosphate dehydrogenase.
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11. Hereditary Hemolytic Anemia
Causes of hereditary hemolytic anemia.
Hereditary Hemolytic Anemia
Hemoglobin defects
Metabolic defects
Membrane defects
defective synthesis,
deficiency of:
hereditary
e.g. thalassemia
spherocytosis
( or )
pyruvate kinase
abnormal variants,
triose phosphate
elliptocytosis
e.g. Hb S, Hb C,
isomerase
unstable
pyrimidine-5-
stomatocytosis
nucleotidase
glucose-6-phosphate
etc.
dehydrogenase
glutathione synthetase
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12. INHERITANCE ■ AUTOSOMAL ■ SEX-LINKED
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13. Sex-linked Inheritance
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14. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
Sex-linked hereditary hemolytic anemia
Acute intravascular hemolysis due to oxidant stress.
Episodes of acute hemolytic anemia
Most patients are asymptomatic
4 Clinical syndromes:
oxidative stress-induced hemolysis by certain drugs, infection and other illness.
Favism (fresh and uncooked beans are more dangerous than dried cooked ones.
Neonatal jaundice
Chronic hemolytic anemia
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