Sickle Cell and Transfusions
Ashley Duckett, MD
MUSC
June 21, 2012
Transfusion
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Risks of transfusions
Indications for transfusion
Exchange transfusion
Standardize care at MUSC
• Largest users of US rare donor registry
• Different antigens than on donor RBCs
Transfusions
• The good
– Increase O2 carrying capacity & organ perfusion
– Decrease percentage of HbS
– Prevent organ damage
• The bad
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Infection
Volume overload
Transfusion reactions
Alloimmunization
Iron overload
Hyperviscosity
Transfusion
• If pt is new to MUSC, notify blood bank that pt
has SCD –
– leuko-reduced and phenotypically matched
RBCs
• Pts seen here from birth had a phenotype sent
at 1 year old or before 1st transfusion
Transfusion
• If pt stable, high retic and simple pain
crisis DO NOT need to transfuse
• There is NO DATA that transfusion
shortens the duration of a pain crisis
• Expert opinion: NIH guidelines, Red
Cross guidelines…
Transfusion Reactions
• Febrile nonhemolytic transfusion reactions
– IF h/o febrile transfusion reactions should be
premedicated (Benadryl, Tylenol) - ask the pt
• Acute hemolytic transfusion reactions
• Delayed hemolytic transfusion reactions
• TRALI (transfusion associated lung injury)
Alloantibodies
• Almost 30% have alloantibodies
– Need to give phenotypically matched RBCs (matched
for C, E, Kell)
– Ask pts if they have abs or if it takes a long time to find
compatible blood
• Anamnestic response delayed hemolytic
transfusion reaction (life threatening!)
– 5-20 days after a transfusion
Iron Overload
• Each unit of blood adds 200-250 mg Fe
– Can’t phlebotomize anemic patients
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End organ damage – heart, liver, endocrine;
Contributes to early death
Serum ferritin, MRI (heart, liver, panc, spleen)
Chelation therapy
Desferal – req IV or SQ infusion
Exjade (desferasirox)-only oral iron chelator
– Side effects: N/V/D/abdominal pain, inc cr
– Rare hepatic failure – need LFT monitoring
Hyperviscosity
• Blood from SS pt has higher viscosity than
normal blood at same Hb
• Deoxygenated sickle blood has 10x higher
viscosity than oxygenated sickle blood at
the same Hb
• Increased viscosity means slow flow (esp in
small vessels) and promotes sickling
• DO NOT transfuse a sickle cell pt to a Hb
of >10
Reasons to transfuse acutely
• Symptomatic anemia
– Severe fatigue, dyspnea, heart failure
– Usually not until Hb <5 (NIH guidelines, 2002)
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Acute Chest Syndrome
Acute CVA
Aplastic crisis (remember short RBC life span)
Preop
Hepatic or Splenic sequestration
Priapism (second line because of ASPEN)
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Exchange transfusion- (Erythrocytapharesis)
• VERY SICK pts – multiorgan failure,
impending respiratory failure, hepatic
sequestration
• Pts with Hb too high for simple transfusion
or need to rapidly decrease HbS fraction
Exchange transfusion
• Need to know pts HbS fraction
– Understand HbS fraction
– Indicated when HbS>80%, goal <30%
• Maybe with lower fraction if Hb is >8-9
– STAT Hb electrophoresis, call path and let
them know
• Call pheresis team and determine access
– Dialysis cath, now new ports?
Preoperative Transfusions
• Multicenter study of 604 surgical patients
• Randomized to aggressive (Hb S<%30) vs
conservative (Hb 10) transfusion regimen
• Serious complications were the same
(acute chest in 10%) except more
transfusion rxns in aggressive group
• ?SC or B+ thal pts with high baseline Hb
Vichinsky et al; NEJM 1995;333 206-13
Preop transfusions
• Cochrane review Jan 2012
– 2 studies (NEJM and study from Bahrain) and
observational data from 3rd world countries
– Is no tx = to simple transfusion?
“Conservative therapy appears to be as effective as aggressive
therapy in preparation for surgery in people with sickle cell disease,
further research is needed to examine the optimal regimen for
different surgical types, and to address whether preoperative
transfusion is needed in all surgical situations”.
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Chronic transfusions
• Benefits must outweigh known risks
• Primary and secondary prevention of CVA (STOPI and STOPII trials)
• Recurrent acute chest, debilitating pain
• Symptomatic anemia with renal failure
unresponsive to epo
• Pulmonary hypertension
• Heart failure
• ?Priapism, Leg ulcers
• Sickle cell progress note
• Transfusion criteria on OACIS
• Dr Greenberg, Dr Sherron Jackson
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References
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Inati, Adlette. Recent advances in improving the management of sickle cell disease.
Blood Reviews, 2009 Dec; 23 Suppl 1:S9-13.
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Yazdanbakhsh, K et al. Red blood cell alloimmunization in sickle cell disease:
pathophysiology, risk factors, and transfusion management. Blood, 2012. May 4.
[Epub ahead of print]
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Smith-Whitely, K and Thompson, AA. Indications and complications of transfusions in
sickle cell disease. Pediatr Blood Cancer. 2012 Aug;59(2):358-64. [Epub 2012 May 4.]
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American Red Cross. Practice Guidelines for Blood Transfusion, Second Edition, April
2007
The Management of Sickle Cell Disease, 4th ed. NIH Publication No. 02-2117.
Revised May 28, 2002 (Forth Edition) National Institutes of Health, National Heart,
Lung, and Blood Institute
Hirst C, Williamson L. Preoperative blood transfusions for sickle cell disease.
Cochrane Database of Systematic Reviews 2012, Issue 1. Art. No.: CD003149. DOI:
10.1002/14651858.CD003149.pub2
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