EM-CCM Conference on 6/18/14 Presented by Dr. Bonnie Brown
Special Thanks to Dr. Gillette for his insightful comments and attendance.
The Case
41 yo F pmhx Sickle Cell Disease presents 15 hours after onset of right face numbness, right arm numbness and weakness, and difficulty speaking. Stroke work-up performed including sickle cell pertinent labs (LDH, retic, HPLC) and hematology consult. Neurology and hematology recommend exchange transfusion – not something we see frequently in the ED!
CVA in Sickle Cell Disease Pearls
Occurs in young population – 35 yo to 65yo.
The pathophysiology with CVA in SC dx is increased blood viscosity due to sickling. A simple blood transfusion, while increasing the oxygen carrying capacity of the blood, will not decrease viscosity.
What’s needed is an exchange transfusion! In an exchange transfusion, the patient’s RBC’s are removed and replaced by donor RBC’s free of sickle cell disease. This increases the oxygen carrying capacity while DECREASING viscosity. Goal is HgbS < 30% on HPLC nearing but not above Hgb of 10 or Hct of 30. No RCT’s, but all three major expert panels recommend exchange transfusion in acute CVA in sickle cell disease. The timing of exchange transfusion is less clear. Some theorize that immediate exchange transfusion can worsen stroke as it can lead to hypotension during the transfusion.
In sickle cell children at high risk after transcranial Doppler, the STOP trial showed a decrease in cva over 30 months from 30% to 3% with blood transfusions with goal HgbS<30%.
TPA is NOT recommended in this population by these expert panels. They are theorized to have worsening bleeding potential with TPA AND the pathophysiology of their CVA is not embolic/thrombolic where TPA can theoretically help. SC patients have strokes secondary to sickling, decreased oxygen delivery, and increased viscosity in small venules.
Other indications for Exchange Transfusion:
– Acute CVA
– Severe Acute Chest syndrome
– Multi-organ Failure Syndrome
o Also used for preop transfusion, transfusion in volume sensitive patient, hyperosmolar contrast agents (uncertain), refractory priapism (uncertain)
Acute Chest Syndrome Pearls
Few definitions exist, in short, difficulty breathing/sob/cough/hypoxia with fever/infiltrates
Treat: antibiotics (impossible to distinguish between pna and acs), oxygen if hypoxic, pain control, blood transfusion, incentive spirometry, Exchange transfusion if severe (PaO2<70% on RA or worsening respiratory distress).
Multi-organ Failure Syndrome Pearls
Definition: acute pain crisis, leading to severe hemolysis resulting in multi-organ failure (liver, lung, kidney). Frequently associated with AMS.
Treatment: exchange transfusion
RUQ Syndrome Pearls
Definition: bili> 50, ALT elevated
Tx: exchange transfusion (limited data)
Different from hepatic crisis where bili <20 and less severe ALT elevation
Exchange Transfusion in Priapism Pearls
Initially concern for ASPEN syndrome with exchange transfusion (SC patients with priapism develop neurological complications after exchange transfusion), but newer reports suggest not from exchange transfusion, but from hyper-viscosity due to post exchange transfusion hgb >11.
References
â– Best practices for transfusion for patients with sickle cell disease
Wun and Hassell. Hematol Rev. Jul 1, 2009; 1(2): e22.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3222256/#!po=30.0000
â– The epidemiology, evaluation and treatment of stroke in adults with sickle cell disease.
Strouse J, Lanzkron, Urratia V. Expert Rev Hematol. Dec 2011; 4(6): 597–606. http://
www.ncbi.nlm.nih.gov/pmc/articles/PMC3267235/
â– Red Cell Exchange in Sickle Cell Disease. Swerdlow P. American Society of Hematology
Education Program. http://asheducationbook.hematologylibrary.org/content/2006/1/48.full
â– Standards for the Clinical Care of Adults with Sickle Cell Disease in UK Care Book. 2008.
Sickle cell society.org/NHS. http://sct.screening.nhs.uk/getdata.php?id=10991
Summary by Dr. Andrew Grock
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