HEAMOLYTIC ANAEMIAS

Prof.Huda M. Haroun
Prof.Huda M. Haroun
MBBS. U.of K
MD. U. of K.
Msc.MPE U.of K.
 Haemolytic anaemia
• Physiology of RBC
• Cardinal feature of anaemia
• Causes
• Types
• Investigation
• Management
• Prevention
 Physiology of RBC
• RBC diameter is 6.0-8.5 mm. Biconcave disk

Composition of Cell Membrane

➢ Double layer membrane with vertical &horizontal interactions

➢ Protein 50%
(Actin,Bspectrin,aspectrin,Ankyrin,protien 4.1,4.2,Band 3protien

➢ Fat,40% Phospholipid,Cholestrol

➢ CHO,10% Glycophorin A,B,C

 Physiology of RBC cont.
• Haem + globin
• Globin ------- chromosome 11,16
• Types of haemoglobin:
• A beta
• A2 delta
• F gama
• H 4 beta
• Barr 4 gama
• Polypetide chain ---- 150 AA
 Cardinal Features of Haemolytic
Anemia
➢ Bone marrow hyperplasia

➢ Extra modularly haemopoiesis

➢ Pallor
➢ Jaundice(uncongugated hyper bilirubinaemia)

➢ Haptoglobin

➢ IBC
 Causes of Haemolytic Anaemias
➢ RBC PROBLEMS
➢ RBC Membrane Defects
➢ Enzyme Deficiency
➢ Haemoglobinopathesis

Out side cell •

➢ Infection (Parasitic, Septic)
➢ Antibodies
➢ Drugs/Toxins
 Cell membrane defect
➢ Congenital spherocytosis
➢ Autosomal Dominant
➢ Defect cell membrane
(protein,Actin,Ankyrin,a/b Spectrin)
➢ Increased permeability haemolysis in
hypotonic solution
 Clinical Feature
➢ Pallor
➢ Fatigability
➢ Jaundice
➢ Spleen enlarge but not palpable
 Investigations
• CBC
• Fragility test
• Auto haemolysis test
• Gel electrophoresis
 Treatment
• Spleenectomy
 G6PD.Defeciency
• Sex –link recessive
• Decrease activity of the enzyme
• Black American,europian ,Chinese
• Haemolysis induced ,drugs, food
 Clinical Feature
• Black urine
• Pallor
 Diagnosis
• CBC
• Enzyme assay
 Treatment
• Prevention
• Aspirin,Sulpha,Fava bean
 Acquired Hemolytic Anemia
• Most common are responses to certain
medications and infections
• Antibodies are attached to RBC.wall
leading to its destruction at high or low
temp.
 Acquired Haemolytic Anaemia
cont.
• Cold-antibody hemolytic anemia
• In which the antibodies react with the
RBCS at a temp.below that of normal body
temp.of IgG type.
.
• Warm at body temp.of IgM.type
 Acquired Haemolytic Anaemia cont.

• mechanical damage as they circulate

through the :-
• Blood vessels.
• Aneurysms.
• Artificial heart valves.
• Very high blood pressure can cause
red cells to break up and release their
contents
 Acquired Haemolytic Anaemia
cont.
• hemolytic anemia may be caused by a
condition called hypersplenism, in which a
large, overactive spleen rapidly destroys
red blood cells.
 Treatment
• Corticosteroid medications, or adrenal
steroids, may be effective, especially in
hemolytic anemia due to antibodies.

• If the cause of the disorder is a

medication, the medication should be
stopped.
 Prognosis
• Hemolytic anemias are seldom fatal.
However, if left untreated, hemolytic
anemia can lead to heart failure or liver
complications.
 HAEMOGLOBIN OPATHIESES

• Haem + globin
• Globin ------- chromosome 11,16
• Types of haemoglobin:
• A beta
• A2 delta
• F gama
• H 4 beta
• Barr 4 gama
• Polypetide chain ---- 150 AA
 DISEASES

• Loss of chain – partial or complete

• Exchange of amino acid

• TYPES:
• Sickle cell anaemia
• Thalassaemia
SICKLE CELL DISEASE:
• AR
• Amino acid exchange
• Glutamic acid by valine
• Position 6 beta chain
• TYPES:
• SS disease
• AS trait
PRESENTATION:
• Hyperhaemolytic crisis
• Vaso-occlusive
• Sequestration
• Aplastic
 Vaso occlusive crisis

PAIN
VERBAL REPORT OUCHER
SEVERIT COMMENTS
SCALE (0-10) SCORE
Y

Mild 1-3 10-30 − may not appear uncomfortable

− complains of pain
− +/- crying, neutral expression, at
rest
or shifting in chair
 Cont.

Moderate 4-6 40-60 − may show facial grimacing,

unhappiness, irritability, poor
appetite
− may be unable to carry out
normal daily activities,
uninterested in social
interaction
Severe >7 >70 − facial grimacing, unhappiness,
irritability, poor appetite
− crying very uncomfortable to
point of agony
 Treatment
1-Mild to moderate pain: acetaminophen (15
mg/kg/dose) with codeine(1 mg/kg/dose)
separately, or together.
2-Ibuprofen may be helpful (6 mos-12 yrs: 5-10
mg/kg/dose
3-Encourage patients to drink. If within 30-60
minutes pain relief is inadequate and there are no
other acute complications, the patient can be
discharged on oral analgesics.
• .
CONT.
▪ Moderate to severe pain: IV bolus of
morphine 0.1-0.15 mg/kg/dose (max. 7.5
mg/dose). It can be repeated once, 60
minutes later, if pain is inadequate
controlled
▪ Administer IV fluid to all patients in
moderate to severe pain: a 10mL/kg bolus
of saline, followed by1.5 times the
maintenance as dextrose 5% in sodium
chloride 0.45% solution.
 ACUTE SPLENIC
SEQUESTRATION
• A child with an acute splenic sequestration
crisis presents with symptoms of
• Acute anaemia (pallor, tachycardia, frank
cardiovascular collapse)
• Splenomegaly (pain in the left upper
quadrant, thrombocytopenia); and
• Evidence of an active bone marrow
response (increased reticulocytes)
• .
 CONT.
• On physical examination, patients show
signs of anaemia, hypovolemia, and an
enlarged spleen (larger than baseline),
sometimes massively so. Mild cases may
be asymptomatic
 Management
• Patients should be on a cardiac or O2
monitor.
• 2. To raise the hemoglobin and maintain
cardiovascular stability, transfuse the
patient with 10-15 mL/kg of packed RBCs
if there are any signs of cardiovascular
collapse or if Hb levels are less than 40-50
g/L (repeat if necessary..
 APLASTIC CRISIS
• The condition is characterized by a rapid
fall in hemoglobin (the retic count falls to
<0.1%; Hb falls often to 30-50 g/L) from a
direct effect of parvovirus B19 or (rarely)
other infectious agents on erythroid
progenitors in the marrow.
 Management
Start transfusion immediately if the
patient is unstable (e.g., has postural BP
instability, enlarged liver, cardiac gallop). If
the patient is stable, transfusion can be
delayed a few hours if necessary.
 THALATHAEMIA

• Defect - messenger RNA

• 1- Complete or partial deletion
Beta thalathaemia: •
• Major
• Intermediate
• Minor
Beta thalathaemia major (Cooley’s): •
– Homozygosis
• Progressive anemia
 Clinical Features:
– Severe anaemia
– Bone marrow hyperplasia
– Extramedellary haemopoysis
– Haemosedrosis
– Growth failure
 INVESTIGATIONS:
– FBC
– Haemoglobin electrophoresis – HbF 70% and
A2 3%