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HEMOLYTIC ANEMIA
8th
12 March 2014
Approach to hemolytic diseases
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Learning objectives
✓ To be able to define haemolysis and haemolytic anaemia
✓ To be able to classify haemolytic anaemias
✓ To understand the difference between intravascular and extravascular haemolysis
✓ to recognize the clinical & laboratory features of hemolytic anemia
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Approach to hemolytic diseases
HEMOLYTIC ANEMIA
anemia which result from an increase in the rate of red cell destruction
(decreased red cell lifespan)
RBCs are destroyed faster than the bone marrow can produce them.
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Hemolytic anemia
Manifestation
Features of Increased Red Cell Destruction &
Features of Increased Red Cell Production : compensatory vigorous blood regeneration.
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Pathophsiology
1. Increased RBC destruction-anemia
2. Release of RBC contents- breakdown of Hb. –catabolism to bile pigment- jaundice.
3. Unconjugated is water insoluble (indirect hyperbiliurbinemia) - not cross GFR-acholuric jaundice
4. Increase conjugation in liver –pigmented stones
5. Increase marrow erythroid activity- reticulocytosis.
6. marrow expansion &hyperplasia- bone &skeletal changes
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Classification
On clinical presentation;
Acute (favism)
Chronic (spherocytosis)
Acute attack over chronic disorders
On site of hemolysis
Intravscular
(incompatibility, PNH, favism)
Extravascular:
(HS, thalassemia,immune)
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Extravascular haemolysis
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Extravascular haemolysis
It is the major pathogenetic mechanism of hemolysis in a variety of hemolytic anemias
Anemia
Jaundice
Splenomegaly:
No evidence of hemoglobinemia and hemoglobinuria
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Intravascular haemolysis
is the minor pathway of red cell destruction Hemoglobinemia
Decreased serum haptoglobin
Hemoglobinuria
Hemosiderinuria
splenomegaly is not found
Anemia
Jaundice10waseem al tameemi
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Classification
On defect site
Intrinsic-intracorpusclar-
structural or functional defect within the red cell
sickle cell anemia and Glucose-6-Phosphate Dehydrogenase deficiency. (G6PD)
Extrinsic- extracorpuscular-
abnormality in the red cell environment(Marche hemoglobinuria,burn,infect)
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Classification
On etiology (most useful)
Inherited disorders
I. Genetic defect of hemoglobin ( SCD,Thalas. Unstable Hb.)
II. Abnormal RBC membrane (elliptocytosis,HS)
III.Abnormal RBC metabolism-enzyme deficiency- (G6PDdeficiency, pyruvate
kinase deficiency)
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Classification
Acqiured disorders
I. Immune HA (incompatibility, HDN, AIHA)
II. Traumatic µangiopathic hemolysis (prosthetic valves,HUS,TTP,DIC)
III.Infection (malaria,clostridial)
IV. Chemicals,drugs,venoms
V. Physical injury,thermal ,marche hemoglobinuria
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Clinical features of HA
1. Anemia; chronic congenital- mild ,moderate. Acute attack – severe
2. Jaundice; mild, if severe : risk in neonates-kerinkterus. No bilirubin in urine (acholuric jaundice).
3. Splenomegaly; extravascular destruction.
4. Cholethiasis; black multiple small bile stones. Biliary colic ,cholangitis or Asymptomatic
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Clinical features of HA
5. Aplstic crisis; chronic congenital hemolysis. Transient arrest in RBC production due to parvo virus B19 infection
6. Leg ulcers; chronic congenital hemolysis, sluggish flow in capillaries. HS&SCD.
7. Skeletal changes; skull bossing, zygomatic prominence, maxillary ,dental abnormalities. Enlarging metacarpals bones
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Laboratory features
1. Excess RBC destruction
1. Increase S.bilirubin (indirect) <5mg/dl
2. Increase S.LDH
3. Low S. haptoglobin.
4. Increase urobilinogen in urine, no bilirubin in urine.
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Laboratory features
2. Intravascular hemolysis1. Hemoglobinemia;red color plasma
2. Hemoglobinuria; pink –dark color urine ( no microscopic RBC)
3. Haemosiderinuria; proximal tubules re absorption
4. Met-hemoglobinemia & Met-hemalbuminemia; coffee brown plasma
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Laboratory features
3. Accelerated erythropoiesis
1. Reticulocytosis.
2. Polychromasia,macrocytosis, nucleated RBC,
3. Thrombocytosis,leucocytosis.
4. BM erythroid hyperplasia
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Laboratory features
4. Radiological featuresWidening of marrow cavity,
thinning of cortex separation between cortices
Prominent trabecualae
Hair on end appearance
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Erythrocyte membrane structure
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Erythrocyte membrane defect
Disturbances (quantities or function); RBC destruction (extravascular) in spleen –hemolysis or loss of part of membrane in relation to volume (abnormal shape) & reduced survival
Classification according to
shape; (spherocyte , elleptocyte, stomatocyte)
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Hereditary spherocytosis
most common hereditary membrane defect
Deficiency in β spectrin or anykrin
Autosomal dominant,75%,+ve FH
Presentation1. At childhood, commonly manifested, as chronic mild –
moderate HA
Anemia, jaundice, gall stone, splenomegaly,bone changes
2. At neonatal ,prolonged neonatal jaundice (1st wk)
3. Delayed presentation at adulthood (6th decade)
4. Asymptomatic until-----------pregnancy
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Hereditary spherocytosis
It is chronic mild compensated hemolysis, may exacerbated by attack of
haemolytic crisis: acute severe (infection, vaccination).
Aplastic crisis
Megaloblastic crisis
Leg ulcers
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investigation
Features of extravascular hemolytsis
CBC; anemia ,spherocytes, polychromasia, reticulocytosis
Osmotic fragility test; increased sensitivity to lysis if incubated in hypotonic saline. (diagnosis & screen)
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spherocytosis
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Osmotic fragility test
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Differential diagnosis
Any cause of sperocytosis
1. Immune hemolytic anemia; +ve Coomb’s
2. HDN (neonatal jaundice)
Treatment:1. Folic acid ;1mg/day -5mg/week. Life long.
Alleviate anemia & prevent megloblastic anemia
2. Blood transfusion in acute hemolytic complication.
3. Exchange transfusion in neonate
4. splenectomy .31waseem al tameemi
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splenectomy
o Correct anemia, improve RBC survival, relieve jaundice
Indications:
1. very severe chronic hemolysis
2. recurrent acute hemolytic crisis or acute aplastic crisis.
3. cholecystitis or biliary colic.
4. death in affected family member.
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Preparation & complications
o Delayed after 9-10 yrs.
o preoperative vaccine against encapsulated mo Streptococcus pneumonia
Hemophilius influenza.
Neisseria meningitidies
& influenza
o Followed by long term prophylaxis.
Surgical complications
Risk of infection, sepsis, malaria,
Thrombocytosis ; risk of thrombosis.
Pulmonary hypertension
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