haemolytic anaemias - wordpress.com •to describe the mechanism of anemia. •to identify the...
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Haemolytic Anaemias
Dr. MUBARAK ABDELRAHMAN
MD PEDIATRICS AND CHILD HEALTH
Assistant Professor
FACULTY OF MEDICINE -JAZAN
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Objectives:
• To describe the mechanism of anemia.
• To identify the common causes and classification of hemolytic anemia.
• To recognize the clinical presentation, management and long term complications of common hemolytic anemia.
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Mechanisms of Anemia
Production Disorders: Factor Deficiency (RBC Size)
-Iron, Vit. B12, Folate Hematopoietic Cell Damage
Survival Disorders: Blood Loss Red Blood Cell Destruction (Shape)
-Hemolytic Anemias
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Clasification of haemolytic anaemias
According to cause:
Intrinsic(intra-corpuscular)
Extrinsic(extra-corpuscular)
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Intrinsic RBC Abnormalities:
Hereditary Membrane Proteins – Spherocytosis. Enzymes - G6PD Deficiency. Hemoglobin Synthesis - Sickle Cell Disorders, Thalassaemia.
Acquired:
• Paroxysmal nocturnal haemoglobinuria (PNH)
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Extrinsic causes
• Immune mediated.
• Mechanical.
• Hypersplenism.
• Infections, toxins, etc
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Haemolysis
• Extravascular
• Intravascular
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Extravascular Haemolysis
Clinical indicators:• Pallor
• Jaundice • Splenomegaly
Laboratory Findings:• Low Hb.• High Reticulocyte count.
• Polychromasia in peripheral film.
• Raised unconjugated serum bilirubin.
• Increased urine urobilinogen
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Intravascular Haemolysis
Clinical indicators: Pallor No jaundice initially , later Usually no splenomegaly Red urine.
Laboratory Findings: Low Hb High Reticulocyte count Polychromasia Haemoglobinuria Haemosiderinuria in chronic intravascular hamolysis
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Complications of chronic haemolytic anaemia:
• Depressed immunity-infections
• Chronic non–healing ulcers
• Gallstone formation---cholecystitis
• Hypersplenism if huge splenomegaly
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RBC MembraneHereditary Spherocytosis
Autosomal Dominant - Most Common Autosomal recessive - More Severe Disorder of RBC Membrane Proteins Cytoskeletal Proteins - Maintain RBC Shape, Strength,
Flexibility Chronic Hemolytic Anemia
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Hereditary Spherocytosis
Shear forces
in circulation
membrane
stabilitymembrane
loss
M
spherocytesplenomegaly
Chronic
hemolytic
anemia
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Hereditary Spherocytosis
Clinical manefistation:Asymptomatic
Fluctuating hemolysis
Splenomegaly
Pigmented gall stones- 50%
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Hereditary Spherocytosis
Laboratory Findings:• Mild, moderate or severe anaemia.• Spherocytes• Reticulocytes.• Osmotic fragility test :Increased susceptibility of RBC to lysis
in hypotonic solution
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Spherocytes
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Osmotic Fragility
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Management:
Folic Acid 5mg weekly, prophylaxis life long
Splenectomy
Blood transfusion in Ac, severe hemolytic crisis
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Hereditary Elliptocytosis
• Equatorial Africa, SE Asia
• AD / AR
• Functional abnormality in one or more anchor proteins in RBC membrane- Alpha spectrin , Protein 4.1
• Usually asymptomatic
• Mx: Similar to H. spherocytosis
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Elliptocytosis
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Red Cell Enzymopathies
Physiology:
HMP (Hexose monophosphate) shunt pathway:
NADPH & Glutathione production (G6PD def.).
EM (Embden Meyerhof) pathway:
ATP production (Pyruvate kinase def.).
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Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
Pivotal enzyme in HMP Shunt & produces NADPH to protect RBC against oxidative stress
Most common enzymopathy -10% world’s population
Protection against Malaria
X-linked
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Clinical Features:
• Acute drug induced hemolysis:
Aspirin, primaquine, chloroquine, dapsone, cotrimoxazole,
nalidixic acid, nitrofurantoin, ciprofloxacin,
chloramphenicol, …
• Chronic compensated hemolysis.
• Infection/acute illness.
• Neonatal jaundice.
• Favism.
• RED URINE
• It is a classical example of intravascular haemolysis
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Investigations: P. Smear: Bite cells, blister cells, irregular small cells, Heinz
bodies, polychromasia G-6-PD level
Treatment: Stop the precipitating drug or treat the infection Acute transfusions if required
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Bite Cell - G6PD Deficiency
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Paroxysmal Nocturnal Hemoglobinuria Acquired Disorder 25% Paroxysmal and NocturnalMutation of Stem Cells - No Anchor Protein
(Chronic Hemolysis)
Complement-
Induced Lysis
(Intravascular
- Hgb in Urine)C
CC
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Hemolytic Anaemias
Extrinsic Abnormalities - Acquired:
Autoimmune Hemolytic Anemia
Antibody Mediated (Spherocytes)
Non-Immune Acquired Hemolytic Anemia
Mechanical Trauma (Schistocytes), Infection : malaria
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Autoimmune Hemolytic Anemia
• Result from RBC destruction due to RBC autoantibodies: Ig G, M, E, A
• Most commonly-idiopathic
• Classification:
– Warm AI hemolysis: Ab binds at 37ᵒ C (Ig G).
– Cold AI Hemolysis: Ab binds at 4ᵒ C (Ig M).
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Non-Immune Acquired Hemolytic Anemia
1. Mechanical Trauma:A) Mechanical heart valves, Arterial grafts: cause shear stress
damage
B) March hemoglobinuria: Red cell damage in capillaries of feet
C) Thermal injury: burns
D) Microangiopathic hemolytic anemia (MAHA): by passage of RBC through fibrin strands deposited in small vessels disruption of RBC eg: DIC, Malignant HTN,TTP,HUS
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TRAUMATIC HEMOLYSIS
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Non-Immune Acquired Hemolytic Anemia
2. Infection:• Falciparum malaria: severe intravascular hemolysis
(Blackwater fever).
• Colstridium perfringens septicemia.
3. Chemical/Drugs:Oxidant denaturation of hemoglobin e.g. Dapsone, sulphasalazine, Arsenic gas,…
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Malaria in RBCs