A 4-year-old boy presented with severe back pain and vomiting. Examination found pallor and spleen enlargement. Tests showed decreased hemoglobin, sickled red blood cells, and hair-on-end skull appearance consistent with sickle cell anemia. Sickle cell anemia is an inherited blood disorder caused by a mutation causing hemoglobin to sickle and break down red blood cells early, resulting in anemia. Common complications include pain crises, infections, and organ damage. Diagnosis is confirmed by hemoglobin electrophoresis showing elevated hemoglobin S.

1. ss
P.R. Jayawickrama
D.S. Kaluwaduge
K.K.G. Kandewaththa
M. Kanchanamala
UNIVERSITY of RUHUNA , FACULTY of MEDICINE.
23.11.2012
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2. A four years old boy was brought to the pediatric
hospital with a history of severe back pain and
uncontrolled vomiting since previous night. Further
His mother told that her child was complaining
about some attacks of back pain occurring during
the past months.
On examination patient was pale and spleen was
palpable below the costal margin……!!!!
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3. In investigation,
• Haemoglobin level - decreased
• Peripheral blood smear - sickled cells
- nucleated RBC
-features of microcytic
hypochromic anaemia
• X-ray of the skull – hair on end appearance of
frontal bone
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4. SICKLE CELL ANAEMIA……????
Inherited.
form of an anaemia as a result of mutation in Hb.
RBC sickled or crescent shaped.
NOT enough healthy RBC to carry adequate
oxygen.
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5. Normally, humans have
Of these, Haemoglobin A makes up around 96-97%
of the normal haemoglobin in humans.
Haemoglobin
A two alpha two beta
A2 two alpha two delta
F two alpha two gamma
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6. Common types of Sickle Cell
Disorders
Type of
anaemia
Hemoglobin
variation
comment
Sickle Cell
Anemia
Sickle
haemoglobin
(HbS) + Sickle
haemoglobin
Most Severe –
No HbA
Hemoglobin S-
Beta thalassemia
Sickle
haemoglobin
(HbS) + reduced
HbA
Mild form of
Sickle Cell
Disorder
Hemoglobin S-C Sickle Mild form of 6

7. 7

8. Pathophysiology
Is caused by
• point mutation in the β-globin chain of Hb
glutamic (hydrophobic amino acid)
valine( hydrophilic )
• at the 6th position
Life span
• RBC 90–120 days
• sickle cells 10–20 days.

9. OXY-STATE DEOXY-STATE

10. • exposure to P O2 < 40 mmHg for 2 to 4 minutes
• polymerization of Hb
• The initiation of polymerization may be
-incomplete and
-reversible , if re-oxygenation occurs early in
the process.
• Repetitive exposure to alternating de-
oxygenated and oxygenated states lead to
-membrane distortion,
-irreversible sickling.

12. Signs ,symptoms & complications
• Anaemia
• Episodes of pain
• Hand-foot syndrome

13. • Frequent infections
• Vision problems
• Delayed growth
• Any signs or symptoms of stroke
• Enlarged heart and systolic murmurs

14. Laboratory investigations
Complete blood count
 Level of Hb -: 6–8 g/dL
(Normal range-: Male=13.5-17.5g/dl Female=11.5-15.5g/dl)
 High reticulocyte count (10–20%).
Blood film

15.  The blood film is microcytic and hypocromic
Sickled cell anaemia Normal
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16. 16

17. Sickle solubility test
Mixture of Hb S in a reducing solution
Gives a turbid appearance
(Precipitation of Hb S)
 Normal Hb gives a clear solution
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18. 18

19. ■ Hb electrophoresis
• To confirm the diagnosis.
• There is,
no Hb A
80–95% Hb SS
2–20% Hb F
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20. 20

21. Results of laboratory examination in sickle cell anaemia
Laboratory
examinations
results Values in this
disease
Values in health
WBC count increased 10000 -30000 5000 -10000
RBC count decreased 1 -4 million/mm3 4 -6 million/mm3
Hb count decreased 6 -8g/100ml Male=13.5-17.5g/dl
Female=11.5-
15.5g/dl
Haematocrit reading decreased 10 -30% 45%
Reticulocyte count increased 10-40% 1-2%
Hb electrophoresis positive HbS & HbF HbA
Urine analysis Albumin casts positive negative
Serum bilirubin increased 1-3 mg/100ml 0.2-0.8mg/100ml
Platelet count increased 40000-50000/mm3 150000-
400000/mm3
Bone marrow
exanination
Increased red cells 40-70% 8-30%
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22.  Tests to detect sickle cell genes before birth
 Diagnosed in an unborn baby
 Sampling amniotic fluid
 Look for the sickle cell gene
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23. Hair‐on‐end appearance
X-RAYS
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24. Hand-foot syndrome
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25. Chest x-ray
Acute chest syndrome
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26. Bone deformity
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27. MRI - Osteonecrosis
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28. REFFERENCES
• Kumar & Clark’s clinical medicine, 7th eddition
• Ganong’s physiology, 23rd eddition
• Quinn CT, et al.: Minor elective surgical procedures using general
anesthesia in children with sickle cell anemia without pre-operative
blood transfusion. Pediatr Blood Cancer 2005; 45:43–7
• Marchant WA, Walker I: Anaesthetic management of the child with
sickle cell disease. Paediatr Anaesth 2003; 13:473–89.
Anesthesiology Problem-oriented Patient Management, 6th ed.
2008, pp 980–92.
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29. ACKNOWLEDGEMENT
we wish to thank our dear sir Dr.Mahinda
Kommalage for all the advices and
his valuable time in completing this
presentation.
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30. THANK YOU !!!!!!
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