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Is it safe for
people with sickle
cell trait to donate
blood?
Reem Mohammed Alsaedi
Saudi Diploma Blood Transfusion
K.A.U Hospital
Table of Contents
sickle cell disease sickle cell trait
SCT and Blood
donation Counclosion
Sickle cell
disease
Sickle cell disease (SCD) is a widespread disease
caused by a mutation in the beta-globin gene that
leads to the production of abnormal hemoglobin
called hemoglobin S. The inheritance of the
mutation could be homozygous or heterozygous
combined with another hemoglobin mutation.
Introduction
● Sickle cell affects quality of
haemoglobin
 Red blood cells can take on sickle
shape when they give up oxygen
in small blood vessels
 They become stiff and can’t
circulate properly
 They can also become stuck in
small blood vessels and cause
blockages
Normal cell // Sickle
Cell
Sickled red blood cells can get stuck
and usually only circulate for 20 – 30
days
Normal red blood cells move freely in
the circulation for 120 days
Sickle cell is genetic disease
● Genes are the code which pass
information to us from our parents
● They are what makes us look more like
our mother or father
● Determining whether we are tall or
short, black or white
How sickle cell is inherited
?
Carriers have
the disorder
gene. They
usually have no
symptoms and
may not even
know they carry
the gene
If only one
parent were a
carrier: The child
cannot inherit
the disorder. But
they could still be
a carrier
If one parent has
a disorder and the
other is a carrier
the child is more
likely to inherit the
disorder
Healthy
person
A + A = =
From one
parent
From
one
parent
Healthy
person
Healthy red
blood cells
Sickle cell
carrier
A + S = =
From one
parent
From
one
parent
Sickle cell
carrier
Healthy red blood
cells (may be a few
sickled cells)
This person is healthy, but
can pass the unusual gene
on to their children
Person has sickle cell
disease
S + S = =
From one
parent
From
one
parent
Person has sickle
cell disease
Sickle red
blood cells
This person will have
the disease for their
whole life
● The sickle cell trait is a
genetic mutation
characterized by the
presence of hemoglobin S
(HbS) in heterozygosis in
which patients are
asymptomatic, which
makes them suitable for
blood donation
The sickle
cell trait
Sickle Cell
Trait (Hereditary Condition)
• Red blood cells (RBC’s) are normally round, circular or disc shaped.
• RBC’s have hemoglobin component (helps carry oxygen) and platelets (helps in
clotting)
• Person gets one gene for “sickling” hemoglobin and one gene for normal
hemoglobin.
• People with sickle cell trait rarely have symptoms – because they have normal
hemoglobin along with abnormal hemoglobin
• With INTENSE or EXTENSIVE exercise the hemoglobin can take a sickle shape or
crescent shape.
• This effects oxygen carrying capacity to the cells!
Sickle Cell
Trait (Hereditary Condition)
• 10 deaths reported in collegiate football alone from Sickle Cell since 2000
during performance enhancement/conditioning sessions!
• 1 death reported due to exertional sickling in pre-season to a track student-
athlete in 2010!
• Approximately 8-9% of African Americans have sickle cell trait!
• Hispanic Americans estimated at 4%
• Brazilians estimated at 2-8%!
• Most common among people whose ancestry come from Africa, the Middle East,
the Mediterranean, Caribbean and India!
• Sickle Cell Trait Does Not disqualify athletes from participation in intercollegiate
athletics!
SICKLE CELL
EVENT
(CASCADE-LIKE)
When athletes experience dehydration, infection
and low oxygen supply – The fragile RBC’s assume
a crescent shape causing RBC breakdown, poor
flow of RBC’s through blood vessels with poor
oxygenation to the tissues!
Symptoms of sickle cell anemia
Accelerated
Fatigue
Distracted
Dizziness
Swollen hand
Irritable
Prevalence of sickle cell
anemia
• 300,000 births annually
with Hb SS
• In many African
countries, 10-40% of
population carries Sickle
Trait
• Malaria endemic
countries – Sub-Saharan
Africa, Middle East, India
Sickle cell and thalassaemia: a brief introduction
PHE gateway number: 2014540
Number of Newborns per Country in
2015
From The New England Journal of Medicine, Piel, Frédéric, et al, Sickle Cell Disease, 376,
Apr 20.
CREDITS: This presentation template was created by Slidesgo,
including icons by Flaticon, infographics & images by Freepik and
illustrations by Stories
SCD is highly prevalent in
Saudi Arabia.
1,572,140 SCD & SCT
The national premarital screening
program conducted over a 6-year period,
including 1,572,140 men and women
SCD & SCT
4.46%
NORMA
L
95.54%
4.2%
0.26
%
SCT SCD
Screening for SCT
PCR
Hemoglobin
electrophores
is
Turbidity
tests
Point-of-care
(POC) testing
Sickling Test Method
1) A sample of venous blood or capillary blood may be collected for
this test. *Venous blood from the arm.*Capillary blood from the
finger tips or ear lobes and in infants from the heel of the foot.
2) Mixing blood with the reducing agent, sodium metabisulphite, will
induce sickling in susceptible cells.
3) the results can be viewed under a microscope after 20 minutes.
Negative Test This test is simple and quick, used Positive Test HbA to
identify the presence of HbS. HbS Normal RBC Sickled RBC *Positive
sickling test associated with a normal haemoglobin is likely to indicate
a patient with sickle cell trait.
Sickle Solubility Test (SST)
A rapid and inexpensive technique used to screen for the presence of sickling
hemoglobins, can be used at home. •A positive result must be confirmed by another
method (HPLC or electrophoresis) to confirm the presence of Hb S and to distinguish Hb
AS (carrier state) from Hb SS (sickle cell disease).
•Disadvantage: Other insoluble hemoglobins, such as Hb C-Harlem, will also give a
positive result.
Method: Depend on phosphate solubility 1) Erythrocytes are lysed by saponin. 2) The
released hemoglobin is reduced by sodium hydrosulfite in a phosphate buffer. The
presence of HbA under The resulting tactoids of HbS 3) Reduced HbS is characterized
by its very these same conditions results causes the solution to remain low solubility
andred solution. of in a clear the formation turbid. neumatic liquid crystals (tactoids).
Hemoglobin Electrophoresis
test
Haemoglobin electrophoresis will differentiate between
homozygous and heterozygous conditions. * Hemoglobin types
have different electrical charges and move at different speeds.
*HbAS: Has both HbA and HbS. Shows 2 bands *HbSS: Is less
negative by 2 compared to HbA . Migrates slower than HbA
A point-of-care testing
technology (Sickle SCAN)
Steps
1. Collect a 5 µL blood
sample.
2. Mix blood with buffer
solution
3. Place 5 drops on test
strip and wait 5
minutes.
Steps
1. Collect a 5 µL blood
sample.
2. Mix blood with buffer
solution
3. Place 5 drops on test
strip and wait 5
minutes.
Steps
1. Collect a 5 µL blood
sample.
2. Mix blood with buffer
solution
3. Place 5 drops on test
strip and wait 5
minutes.
Steps
1
. Collect a 5
elpmas doolb Lµ
.
2
. Mix blood with buffer solution
3
. Place 5 drops on test strip and wait 5 minutes.
Results
FAQs
● Blood transfusion can be lifesaving in aplastic crisis, splenic
sequestration, and acute chest syndrome.
● Transfusion is the mainstay of management in ischemic
stroke, a devastating complication of SCD, and chronic
transfusion regimens prevent stroke recurrence in a
considerable proportion of patients.
A Boold Transfusion
● Based on the recommendations of the AABB, red blood cell (RBC)
units collected from donors with sickle cell trait (SCT) should not be
used for transfusion of patients with SCD,
● since the main goal of transfusion therapy in patients with SCD is
reduction of HbS level. RBC units prepared for intrauterine
transfusions should also be HbS negative.
● There are other potential benefits of screening RBCs for the
presence of HbS, as leukofiltration failure has been reported with
SCT blood.
A Sickle cell trait person
and blood donation
● The blood filtration may affect by the temperature, pH, osmolarity,
type of anticoagulant, time of storage and oxygen saturation of the
blood unit.
● Citrate anticoagulant and low oxygen saturation are at least
responsible in part for the HbS polymerization.
● When the red cells are partially deoxygenated, the formation of
intracellular polymerized sickle haemoglobin occur. The generated
polymer affects cells’ flexibility.
● The number of intracellular HbS polymer fractions formed can
predict loss and failure of filtration of AS RBCs.
A Sickle cell trait person
and blood donation
A Sickle cell trait person
and blood donation
● Studies showed that individuals with Hb AS have increased
coagulation activity and alterations in sensitivity to calcium and
enzymatic hydrolysis of their RBC
● A successful filtration of sickle trait donor blood components has
been obtained when SO 2 is increased RBCs from SCT become
rigid during the storage, suggesting that HbS might compromise the
cells’ elasticity. Indeed, studies showed an increased elasticity of
stored AS cells.
ADVERSE EFFECTS OF
SICKLE TRAIT
● An anaphylactic haemoglobinuric.
● Massive intravascular sickling.
● Multiple renal and splenic infarctions has been
described in a neonate.
● Jaundice.
● The transfusion of sickle trait RBCs should be avoided for
certain patients (i.e. neonates and patients with either
pathologic homozygous or heterozygous haemoglobin S) and
under certain conditions (hypoxemia).
● The other possibility could be to use these RBCs only to adult
sickle cell negative recipients who require the transfusion of
RBCs of a rare or a specific phenotype
CONCLUSION
References
Sickle Cell Disease Association of America: www.sicklecelldisease.org/index.cfm?page=sickle-cell-
trait-athletics
Alhamdan NA, Almazrou YY, Alswaidi FM, et al. Premarital screening for thalassemia and sickle cell
disease in Saudi Arabia. Genet Med 2007;9:372-7
Maha Badawi , 1,2 Sara Garoot, Mofida Tahlawi,2 Salwa Hindawi, et al. Evaluation of a point-of-care
method for screening blood donors for sickle cell status. 4 TRANSFUSION Volume 60, February
2020
AABB Technical Manual, 19th edition (Technical Manual of the American Assoc of Blood Banks) 19th
Edition
THANK YOU

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  • 1. Is it safe for people with sickle cell trait to donate blood? Reem Mohammed Alsaedi Saudi Diploma Blood Transfusion K.A.U Hospital
  • 2. Table of Contents sickle cell disease sickle cell trait SCT and Blood donation Counclosion
  • 4. Sickle cell disease (SCD) is a widespread disease caused by a mutation in the beta-globin gene that leads to the production of abnormal hemoglobin called hemoglobin S. The inheritance of the mutation could be homozygous or heterozygous combined with another hemoglobin mutation. Introduction
  • 5. ● Sickle cell affects quality of haemoglobin  Red blood cells can take on sickle shape when they give up oxygen in small blood vessels  They become stiff and can’t circulate properly  They can also become stuck in small blood vessels and cause blockages
  • 6. Normal cell // Sickle Cell Sickled red blood cells can get stuck and usually only circulate for 20 – 30 days Normal red blood cells move freely in the circulation for 120 days
  • 7. Sickle cell is genetic disease ● Genes are the code which pass information to us from our parents ● They are what makes us look more like our mother or father ● Determining whether we are tall or short, black or white
  • 8. How sickle cell is inherited ? Carriers have the disorder gene. They usually have no symptoms and may not even know they carry the gene If only one parent were a carrier: The child cannot inherit the disorder. But they could still be a carrier If one parent has a disorder and the other is a carrier the child is more likely to inherit the disorder
  • 9. Healthy person A + A = = From one parent From one parent Healthy person Healthy red blood cells
  • 10. Sickle cell carrier A + S = = From one parent From one parent Sickle cell carrier Healthy red blood cells (may be a few sickled cells) This person is healthy, but can pass the unusual gene on to their children
  • 11. Person has sickle cell disease S + S = = From one parent From one parent Person has sickle cell disease Sickle red blood cells This person will have the disease for their whole life
  • 12. ● The sickle cell trait is a genetic mutation characterized by the presence of hemoglobin S (HbS) in heterozygosis in which patients are asymptomatic, which makes them suitable for blood donation The sickle cell trait
  • 13. Sickle Cell Trait (Hereditary Condition) • Red blood cells (RBC’s) are normally round, circular or disc shaped. • RBC’s have hemoglobin component (helps carry oxygen) and platelets (helps in clotting) • Person gets one gene for “sickling” hemoglobin and one gene for normal hemoglobin. • People with sickle cell trait rarely have symptoms – because they have normal hemoglobin along with abnormal hemoglobin • With INTENSE or EXTENSIVE exercise the hemoglobin can take a sickle shape or crescent shape. • This effects oxygen carrying capacity to the cells!
  • 14. Sickle Cell Trait (Hereditary Condition) • 10 deaths reported in collegiate football alone from Sickle Cell since 2000 during performance enhancement/conditioning sessions! • 1 death reported due to exertional sickling in pre-season to a track student- athlete in 2010! • Approximately 8-9% of African Americans have sickle cell trait! • Hispanic Americans estimated at 4% • Brazilians estimated at 2-8%! • Most common among people whose ancestry come from Africa, the Middle East, the Mediterranean, Caribbean and India! • Sickle Cell Trait Does Not disqualify athletes from participation in intercollegiate athletics!
  • 15. SICKLE CELL EVENT (CASCADE-LIKE) When athletes experience dehydration, infection and low oxygen supply – The fragile RBC’s assume a crescent shape causing RBC breakdown, poor flow of RBC’s through blood vessels with poor oxygenation to the tissues!
  • 16. Symptoms of sickle cell anemia Accelerated Fatigue Distracted Dizziness Swollen hand Irritable
  • 17. Prevalence of sickle cell anemia • 300,000 births annually with Hb SS • In many African countries, 10-40% of population carries Sickle Trait • Malaria endemic countries – Sub-Saharan Africa, Middle East, India
  • 18. Sickle cell and thalassaemia: a brief introduction PHE gateway number: 2014540
  • 19. Number of Newborns per Country in 2015 From The New England Journal of Medicine, Piel, Frédéric, et al, Sickle Cell Disease, 376, Apr 20.
  • 20. CREDITS: This presentation template was created by Slidesgo, including icons by Flaticon, infographics & images by Freepik and illustrations by Stories
  • 21. SCD is highly prevalent in Saudi Arabia. 1,572,140 SCD & SCT The national premarital screening program conducted over a 6-year period, including 1,572,140 men and women SCD & SCT 4.46% NORMA L 95.54% 4.2% 0.26 % SCT SCD
  • 22.
  • 24. Sickling Test Method 1) A sample of venous blood or capillary blood may be collected for this test. *Venous blood from the arm.*Capillary blood from the finger tips or ear lobes and in infants from the heel of the foot. 2) Mixing blood with the reducing agent, sodium metabisulphite, will induce sickling in susceptible cells. 3) the results can be viewed under a microscope after 20 minutes. Negative Test This test is simple and quick, used Positive Test HbA to identify the presence of HbS. HbS Normal RBC Sickled RBC *Positive sickling test associated with a normal haemoglobin is likely to indicate a patient with sickle cell trait.
  • 25. Sickle Solubility Test (SST) A rapid and inexpensive technique used to screen for the presence of sickling hemoglobins, can be used at home. •A positive result must be confirmed by another method (HPLC or electrophoresis) to confirm the presence of Hb S and to distinguish Hb AS (carrier state) from Hb SS (sickle cell disease). •Disadvantage: Other insoluble hemoglobins, such as Hb C-Harlem, will also give a positive result. Method: Depend on phosphate solubility 1) Erythrocytes are lysed by saponin. 2) The released hemoglobin is reduced by sodium hydrosulfite in a phosphate buffer. The presence of HbA under The resulting tactoids of HbS 3) Reduced HbS is characterized by its very these same conditions results causes the solution to remain low solubility andred solution. of in a clear the formation turbid. neumatic liquid crystals (tactoids).
  • 26. Hemoglobin Electrophoresis test Haemoglobin electrophoresis will differentiate between homozygous and heterozygous conditions. * Hemoglobin types have different electrical charges and move at different speeds. *HbAS: Has both HbA and HbS. Shows 2 bands *HbSS: Is less negative by 2 compared to HbA . Migrates slower than HbA
  • 27.
  • 28.
  • 29. A point-of-care testing technology (Sickle SCAN) Steps 1. Collect a 5 µL blood sample. 2. Mix blood with buffer solution 3. Place 5 drops on test strip and wait 5 minutes.
  • 30. Steps 1. Collect a 5 µL blood sample. 2. Mix blood with buffer solution 3. Place 5 drops on test strip and wait 5 minutes.
  • 31. Steps 1. Collect a 5 µL blood sample. 2. Mix blood with buffer solution 3. Place 5 drops on test strip and wait 5 minutes.
  • 32. Steps 1 . Collect a 5 elpmas doolb Lµ . 2 . Mix blood with buffer solution 3 . Place 5 drops on test strip and wait 5 minutes. Results FAQs
  • 33. ● Blood transfusion can be lifesaving in aplastic crisis, splenic sequestration, and acute chest syndrome. ● Transfusion is the mainstay of management in ischemic stroke, a devastating complication of SCD, and chronic transfusion regimens prevent stroke recurrence in a considerable proportion of patients. A Boold Transfusion
  • 34. ● Based on the recommendations of the AABB, red blood cell (RBC) units collected from donors with sickle cell trait (SCT) should not be used for transfusion of patients with SCD, ● since the main goal of transfusion therapy in patients with SCD is reduction of HbS level. RBC units prepared for intrauterine transfusions should also be HbS negative. ● There are other potential benefits of screening RBCs for the presence of HbS, as leukofiltration failure has been reported with SCT blood. A Sickle cell trait person and blood donation
  • 35. ● The blood filtration may affect by the temperature, pH, osmolarity, type of anticoagulant, time of storage and oxygen saturation of the blood unit. ● Citrate anticoagulant and low oxygen saturation are at least responsible in part for the HbS polymerization. ● When the red cells are partially deoxygenated, the formation of intracellular polymerized sickle haemoglobin occur. The generated polymer affects cells’ flexibility. ● The number of intracellular HbS polymer fractions formed can predict loss and failure of filtration of AS RBCs. A Sickle cell trait person and blood donation
  • 36. A Sickle cell trait person and blood donation ● Studies showed that individuals with Hb AS have increased coagulation activity and alterations in sensitivity to calcium and enzymatic hydrolysis of their RBC ● A successful filtration of sickle trait donor blood components has been obtained when SO 2 is increased RBCs from SCT become rigid during the storage, suggesting that HbS might compromise the cells’ elasticity. Indeed, studies showed an increased elasticity of stored AS cells.
  • 37. ADVERSE EFFECTS OF SICKLE TRAIT ● An anaphylactic haemoglobinuric. ● Massive intravascular sickling. ● Multiple renal and splenic infarctions has been described in a neonate. ● Jaundice.
  • 38. ● The transfusion of sickle trait RBCs should be avoided for certain patients (i.e. neonates and patients with either pathologic homozygous or heterozygous haemoglobin S) and under certain conditions (hypoxemia). ● The other possibility could be to use these RBCs only to adult sickle cell negative recipients who require the transfusion of RBCs of a rare or a specific phenotype CONCLUSION
  • 39. References Sickle Cell Disease Association of America: www.sicklecelldisease.org/index.cfm?page=sickle-cell- trait-athletics Alhamdan NA, Almazrou YY, Alswaidi FM, et al. Premarital screening for thalassemia and sickle cell disease in Saudi Arabia. Genet Med 2007;9:372-7 Maha Badawi , 1,2 Sara Garoot, Mofida Tahlawi,2 Salwa Hindawi, et al. Evaluation of a point-of-care method for screening blood donors for sickle cell status. 4 TRANSFUSION Volume 60, February 2020 AABB Technical Manual, 19th edition (Technical Manual of the American Assoc of Blood Banks) 19th Edition