1. Overview of Apheresis
and Sickle Cell Disease
Raúl H. Morales-Borges, MD
Medical Director
American Red Cross
Blood Services
Puerto Rico Region
2. What is Sickle Cell Disease?
• Incidence
• Pathophysiology
• Treatment
• Apheresis Considerations
– Patient History
– Amount and Type of blood
– Adverse Reactions
3.
4. Incidence
30 Years Ago
- Infectious Disease
- Stroke
- Average Life Span
Today
- Infectious Disease
- Multiple Organs
- Average Life Span
9. How does SCD cause problems?
• Increased blood viscosity
– Limits blood flow
– Promotes red cell sickling
– Promotes cell-cell interactions
– Activation of coagulation systems
• Increased cardiac output
• Expanded plasma volume
• Will likely have a lower hemaglobin/hematocrit
12. Velocity = pi (pressure) (radius of tube)4
8 (length of tube) (viscosity)
Classic Poiseuille equation for laminar
flow of a fluid through a straight tube
15. What does this mean to my patient?
• By doubling the viscosity, the velocity is cut in
half.
• Flow is slower in vessels of small radius.
– arterioles, venules, capillaries
• Flow is slower in vessels with lower pressures.
– Venules
• Vaso-occlusive events and Red Cell Destruction
16.
17. Treatment Options
• Prophylactic vaccines and antibiotics
• Hydroxyurea
• Aspirin
• Iron Chelation Therapy
• Simple Transfusions
• Red Cell Exchange
18. How do these issues impact my patient?
• Acute
– Vaso-occlusive stroke
– Acute chest syndrome
– Multiorgan failure syndrome
– Right upper quadrant syndrome
– Surgery/pregnancy
• Chronic
– Primary and secondary stroke prevention
– Multi-organ problems
19. What are the potential complications of
SCD that may be treated by apheresis?
• Acute infarctive stroke
• Acute chest syndrome
• Multi-organ failure syndrome
• Right upper quadrant syndrome
• ? Priapism
• ? Acute pain episode
• ? Pregnancy
20. How does a RBC exchange transfusion
help my patient?
• Removing sickle cells and replacing them with
normal red cells will….
– Improve the oxygen-carrying capacity of the blood
– Decrease blood viscosity
– Reduce vaso-occlusive events
– Reduce hemolytic complications
27. Antigen – Antibody Reactions
Y
SENSITIZATION
Y
Y
ABO
Y
Red Cell
Y
Y
Y
Y
Rh (D, C, c, E, e)
Y
Y Kell (K, k)
Y
Y
Y
Kidd (Jka, Jkb)
Y
Y
Y
Y
Y Duffy (Fya, Fyb)
Y
Other: S, U, V, hrb
Y
Antibody
Y
Y
Y
Y
Y
Y
28. Goal: Less than 30%
• Final Hematocrit
– Raise it no more than 3% (why?)
• Final sickle cell % of less than 30 (why?)
– Approximately 1.5 red cell volumes in an
untransfused patient.
– Approximately 1 red cell volume in a recently
transfused patient.
29.
30. What does this mean to my patient?
• By doubling the viscosity, the velocity is cut in
half.
• Flow is slower in vessels of small radius.
– arterioles, venules, capillaries
• Flow is slower in vessels with lower pressures.
– Venules
• Vaso-occlusive events and Red Cell Destruction
31. How much to exchange?
• Prior to the exchange, need:
– Starting hematocrit
– Sickle cell percentage
– Weight in kg
– Total red cell volume
32. Calculating the Red Cell Volume
Red cell volume = (*TBV) (hematocrit)
*TBV = estimated total blood volume
- adult male: 60 – 75 (70)cc per kg
- adult female: 55 – 70 (65)cc per kg
- infant/child: 80/70cc per kg
33. Procedural Considerations
• Removal of Normal Plasma Constituents
• Lowers Plasma Cations – Calcium
• Alteration of Pharmacodynamics
• May Alter Laboratory Test Results
34. Possible Complications
• Acute transfusion reactions
• Vascular access complications
• Procedural complications
• Delayed transfusion reactions
• Other
39. Issues of Exchange Transfusion in
SCD
• No clear cut guidelines for palliative chronic exchange
transfusion for painful vaso-occlusive crisis in adults
patients (Navaid M, Melvin T: Am J Hosp Palliat Care 2010 May;
27(3):215-218).
• Good results in the use of partial exchange transfusion
in the management of SCD in a perioperative setting
(Jaeckel R, Thieme M, Czezlick E, Sablotzki A: Journal of Medical Case
Reports 2010; 4:82).
• Not effective in SCD with coexisting warm
autoantibody haemolysis (Baron JM, Baron BW: Blood Transfus
2010; 8:303-306).
40. Summary
• What is SCD?
• What are some of the complications of SCD?
• What are some of the available treatment options?
• How do RBC exchange transfusion help?
• What are some of the indications for treatment?
42. Summary
• What are some of the factors to be considered prior to
beginning a RBC exchange procedure?
• What are the goals of a RBC exchange?
• What are some of the possible complications during
and following a RBC exchange?
• How do we recognize and manage these complications?
43. References
• Sharon, BI. Management of Congenital Hemolytic Anemias. In
Rossi’s Principles of Transfusion Medicine. 4th ed. AABB Press, 2009.
• Special Review Issue of the Journal of Clinical Apheresis: Clinical
Applications of Therapeutic Apheresis. 2010. ASFA website.
http://apheresis.org.
• McLeod BC, Triulzi DJ. Therapeutic Apheresis. A Physician’s
Handbook, Bethesda, MD: American Association of Blood
Banks, 2005.
• McLeod BC, Weinstein R, Winters, JL, Szczepiorkowski, ZM
eds. Apheresis: Principles and Practice. 3nd ed. Bethesda, MD:
AABB Press, 2010.
• Popovsky MA, ed. Transfusion Reactions, 3nd ed. Bethesda, MD:
AABB Press, 2007.
• The Food and Drug Administration/Center for Biologics
Evaluation and Research: Code of Federal Regulations, Title 21
– Food and Drugs. http://www.fda.gov.
44. References
• Technical manual, 16th ed. Bethesda, MD: American
Association of Blood Banks, 2008.
• Standards for blood banks and transfusion services, 26-27th ed.
Bethesda, MD: American Association of Blood Banks, 2010-11.
• Swerdlow P. Red Cell Exchange. Hematology. 2006:48-53.
http://asheducationbook.hematologylibrary.org/cgi/content/ful
l/2006/1/48
• Lottenberg R & Hassell K. An evidence-based approach to the
treatment of adults with sickle cell disease. Hematology. 2005: 58-
65.
• Gladwin M & Kato G. Sickle cell disease: Advances in
pathogenesis and management. Hematology. 2005:51-57.
• Immunohematology, American Red Cross.2006:22:101-148.
45. Dr. Raúl H. Morales Borges
Hematólogo/Oncólogo
• Cruz Roja Americana de • Ashford Medical Center
PR – Suite # 107
– Servicios de Sangre – Condado, San Juan
– Ubicados en el Centro – Tel. 787-722-0412
Medico de PR – Fax 787-723-0554
– Tel. 787-759-8100 – ihoa@coqui.net
– Ext. 3873 – www.ihoapr.com
– Cel. 787-505-5814
– MoralesBorgesR@usa.redcross.org