TRANSFUSION THERAPY IN SICKLE CELL DISEASE
AIM FOR CHAPTER
PHYSIOLOGY
INDICATIONS
The aim of transfusion in Sickle Cell Anaemia is to relieve symptoms and to treat or avert
complications. Due to the risks of iron overload, exposure to HIV, hepatitis, and other infectious agents,
alloimmunization, induction of hyperviscosity, and limits on the resource transfusion therapy in the patient
with sickle cell disease must be judicious and the need for either “top-up” or exchange transfusion should
be carefully guided by the patient’s symptomatology and the clinical indications.
PHYSIOLOGY
Blood transfusion targets the underlying pathophysiology of SCD as this reduces the percentage
of erythrocytes containing abnormal Hb S. Sickled RBCs possess several physiological properties
which facilitate vaso-occlusion through increased blood viscosity as there are abnormal
interactions between of sickle RBCs with leukocytes, platelets, clotting factors and vascular
endothelium. Donated RBCs contain normal haemoglobin which reduces the percentage of
circulating RBCs with Hb S and therefore reduces the risks of vaso-occulsion.
*Many complications of transfusion are amplified in SCD
Present Illness

Symptoms of symptomatic anaemia- shortness of breath, palpitations, lethargy, pica,
exersional symptoms, symptoms of heart failure,
 infections, neurologic symptoms
Past Medical History

Steady state

Indication of Previous transfusions and number of units transfused

Facility transfusion performed and dates

Adverse reactions-early, late, history of alloantibodies, worsening jaundice particularly
over the past 3 weeks post transfusion

Treatment for iron overload,
 Past gynae/obstetric history
Vaccinations and HIV status
 Hepatitis B, C, last HIV test result
Social History

Religious beliefs about transfusions and personal fears must be discussed.
In women

Menstrual history
Physical Examination

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Vital signs-temperature, respiratory rate, blood pressure and pulse.
CVS- JVP, S3, increased murmur, hyperdynamic precordium
RS: respiratory rate, bibasilar crepitations.
Abdominal-Liver span and spleen, pelvic masses- uterine fibroids
Musculoskeletal- pedal oedema.
Neurological- Document focal deficits if present
Indications for Transfusion
When making the decision to transfuse clinicians should not only by the recommendations but
also by clinical judgement. The following tables serve as an evidence based guide to transfusion.
Graded Recommendations for transfusion:
Complications
Indication
Type of transfusion
recommended
ACUTE
Symptomatic Acute Chest
Syndrome combined with a
decrease of Hb of 1g/dL below
baseline
Simple
Symptomatic severe ACS (i.e.
an Oxygen saturation less than
90% despite supplemental
oxygen)
Exchange
Acute splenic sequestration in
the presence of severe anaemia
Simple
Acute neurological events
within 72 hours of the onset or
if there is a progression of
neurological signs
Simple or exchange
CHRONIC
Child with transcranial Doppler Simple or exchange
(TCD) reading >200cm/sec
Adult and children with
previously clinically overt
stroke
Simple or exchange
Consensus Recommendation for transfusion
ACUTE
Indication
Type of transfusion
recommended
Hepatic sequestration
Exchange or simple
Intrahepatic cholestasis
Exchange or simple
Multisystem organ failure
Exchange or simple
Aplastic crisis
Simple
Symptomatic anaemia
Simple
Cases in which Transfusion Therapy is not usually indicated:
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Stable chronic anaemia.
Recurrent splenic sequestration
Uncomplicated painful crisis
Priapism
Minor surgery under local or short general anaesthesia
Uncomplicated infections
Chronic bone disease
Organ failure including Acute Kidney injury without other indications
To admit or not to admit? That is the question
Criteria for Admission for Transfusion

All patients with absolute criteria for
transfusion MUST be admitted to initiate
transfusion therapy.

All patients requiring acute, manual
exchange.

Patients with heart failure or
uncontrolled hypertension.
SPECIAL CONSIDERATIONS
Criteria for Outpatient Transfusion

Patients being initiated on transfusion
therapy for symptoms of acute anaemia,
chronic stable complications, or pregnancy.

Patients being optimized for elective
surgery or intravascular contrast studies.

Patients on stable chronic transfusion
programs
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Chronic renal failure
Patients uterine fibroids
Heart Failure
Religious groups-Jehovah Witness
PROCEDURE

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Pre-transfusion Counselling
The request
o Ensure the request form is completed accurately including vital information such
as
 Previous transfusion/reaction
 Previous pregnancies
 Specific requirements based on accurate calculations
 Sickle negative and leukocyte reduced blood should be requested on the
order form
Methods of transfusion
o SIMPLE
 Symptomatic anaemia
 All other indications if Hb =<6g/dl
Giving 10ml/kg by 10 points (vol %) or haemoglobin by about 3 g/dL
o EXCHANGE
 Acute neurological events
 Acute chest syndrome with respiratory decompensation
 Ophthalmological surgery1
EXCHANGE TECHNIQUE FOR ADULTS
Using whole blood or packed cells reconstituted to whole blood equivalent (PCV 0.30-0.40)
NOTE: if whole blood is unavailable alternate infusion of equal volumes of packed cells and
normal saline, in appropriate aliquots.
1. Bleed one unit (500ml) of blood from the patient, infuse 500 ml of saline
2. Bleed a second unit from the patient and infuse two units of blood (or 500ml packed cell
and 500ml Normal saline in appropriate e.g. 100ml. Care must be taken not to cause too
much fluctuation in haematocrit level).
3. Repeat steps 1 and 2; if the patient has a large red blood cell mass, repeat once more
RAPID MANUAL PARTIAL EXCHANGE TRANSFUSION
(Modified from Charache et al. 1989)
Packed red cells are initially transfused while whole blood is removed to reduce the percentage
of Hb S without further increasing the haemoglobin levels. This technique is applicable to adults
and children and is based on the initial haematocrit and body weight in kilograms. The exchange
can be done using one or two IV sites by removing and transfusing aliquots adjusted to the blood
volume. This is particular importance in children.

Monitoring
o Before
 Use of Lasix
 Informed consent and witnessing
 Pre-transfusion vitals
o During

o After- post transfusion reaction
Transfusion Goals
ALTERNATIVES TO BLOOD TRANSFUSION
REFERENCES
https://scinfo.org/guidelines/transfusion-therapy
Furosemide supplemented blood transfusion in cases of chronic
severe anemia.
Nand N, Gupta MS, Sharma M.
Abstract
Pulmonary capillary 'wedge' pressures (PCWP) were measured in 20 adult cases of chronic severe
anemia (CSA) before and after transfusion of 700 ml of whole blood at a rate of 5 ml/min. The cases were
randomly divided into 2 groups of 10 cases each. Group II also received 40 mg of furosemide
immediately before the start of transfusion. The majority of the cases had hemoglobin values less than 4
g% and serum albumin values less than 2.5 g%. Pretransfusion intracardiac pressures were normal in all
the cases. Following blood transfusion (BT), appreciable rises (p less than 0.001) in hemoglobin and
arterial and venous oxygen saturation were observed. PCWP increased significantly after BT in Group I
(p less than 0.001). Although it decreased by 3.75% in Group II, this was not statistically significant (p
greater than 0.05). This study implies that a blood transfusion of 700 ml, given at a speed of 5 ml/min in
patients with CSA, results in sufficient hemodynamic stress to cause a significant rise in PCWP, and that
this is completely prevented by simultaneous administration of 40 mg of furosemide.
Furosemide Treatment Before Blood Transfusion in Patients With
Systolic Dysfunction
The recruitment status of this study is unknown because the information has not been
verified recently.
Verified October 2012 by Tel-Aviv Sourasky Medical Center.
Date of scheduled completion October 2014
Sponsors:
Tel-Aviv Sourasky Medical Center
Information provided by (Responsible Party):
Tel-Aviv Sourasky Medical Center