Exeley Inc.

Shulman, Ira A.

I M M U N O H E M A T O L O G Y, V O L U M E  2 2 , N U M B E R  3 , 2 0 0 6 101
Sickle cell disease (SCD) affects approximately
72,000 people in the United States (approximately 1 in
500 African Americans and 1 in 1000 to 1400
Hispanics).1 During the past three decades, the
survival of patients with SCD has improved
significantly, partly because transfusion therapy2,3 has
played a role as a disease-modifying strategy for 
several manifestations of the disease, including acute
chest syndrome,acute intrahepatic sequestration,acute
multiorgan failure syndrome, acute splenic sequestra-
tion, acute symptomatic anemia, aplastic crisis,
bacterial and malarial infections, chronic organ failure,
complicated pregnancy, eye surgery (e.g., scleral
buckle procedure), frequent pain episodes, priapism,
primary and secondary prevention or treatment of
stroke, and surgery requiring general anesthesia.
Depending on clinical circumstances, patients with
one or more of the aforementioned manifestations may
receive simple or exchange RBC transfusions that are
administered episodically or chronically. However,
each transfusion of a unit of RBCs poses a risk of RBC
antigen alloimmunization. Studies show that 25
percent or more of chronically transfused SCD patients
may experience this complication unless precautions
are taken.4 When alloimmunization occurs, the most
common clinically significant antibodies to develop in
transfused SCD patients include antibodies to Rh and
Kell antigens.5–8
It is preferable to avoid alloimmunization to RBC
antigens because this complication makes obtaining
compatible units for future transfusions difficult and
places the patient at risk for development of hemolytic
transfusion reactions, the symptoms of which can
mimic or trigger serious complications of SCD. In
addition, hemolytic transfusion reactions can be
serious and potentially life-threatening complications
in their own right. Studies have shown that the
transfusion of ABO- and D-compatible donor RBCs that
lack C, E, or K when the same antigens are absent from
the recipient’s RBCs can reduce the rate of alloimmuni-
zation in patients with SCD as well as the occurrence
of hemolytic transfusion reactions.
According to a National Institutes of Health (NIH)
publication3 (pages 155–158) the antigenic phenotype of the
RBCs (at least the common antigens belonging to ABO,
Rh, Kell, Duffy, Kidd, Lewis, Lutheran, P, and MNS)
should be determined in all patients older than 6
months. A permanent record of the phenotype results
should be maintained in the blood bank to optimize
matching and a copy of the record should be given to
the patient or family. Furthermore, limited matching of
donor RBCs for E, C, and K is usually performed, unless
patients have antibodies. The transfusion of pheno-
typically matched RBCs may be accomplished by
typing the RBCs of the patient before transfusion, if not
previously performed, for Rh and Kell, in particular E,
C, and K and performing more extensive matching for
antigens in systems such as Duffy, Kidd, and MNS for
those patients who are already alloimmunized. Donor
RBCs lacking the antigens that the patient’s RBCs lack
would then be selected for transfusion.
Although NIH publication 02-2117 was not the
result of a formalized consensus process, it does
represent the efforts of those who have dedicated their
professional careers to the care of individuals with
SCD. In spite of this publication, transfusion practices
in SCD remain disparate in the United States, as
illustrated by two recent national surveys.
Prophylactic phenotype matching
of donors for the transfusion of
nonalloimmunized patients with
sickle cell disease
I.A. SHULMAN
102 I M M U N O H E M A T O L O G Y, V O L U M E  2 2 , N U M B E R  3 , 2 0 0 6
Afenyi-Annan and Brecher surveyed 50 academic
medical centers to determine the extent of the use of
phenotype-matched donor RBCs in the transfusion of
patients with SCD.9 Of 37 academic centers that
responded to a questionnaire, 27 (73%) reported that
they provide antigen-matched donor RBCs. Although
the most common antigens matched were C, E, and K
(24 of the 27 sites or 89%), there were multiple
variances among those 27 centers as to which antigens
were selected for phenotype matching. The authors
concluded that, despite a common approach by some
of the academic centers, there was no single standard
of care with regard to RBC component selection.
A survey by the College of American Pathologists
(CAP) examined 1182 labs in North America to
determine the extent of RBC antigen testing of
nonalloimmunized patients with SCD as well as the use
of phenotype-matched donor RBCs in the transfusion
of these patients.10 The CAP survey, unlike the one by
Afenyi-Annan and Brecher, did not specifically target
academic medical centers. The CAP survey results
indicated that approximately 63 percent of North
American laboratories did not routinely phenotype
patients with SCD for antigens other than ABO and D.
Of the 37 percent of the laboratories that did perform
additional antigen testing beyond ABO and D, only 75
percent actually selected phenotype-matched RBCs for
patients with SCD. On the other hand,more than three-
quarters of the respondent laboratories reported that
for patients with SCD they use RBCs that are leukocyte
reduced and nearly two-thirds of these laboratories
reported that they use RBCs that test negative in a
sickle cell screening test. The authors of the CAP
survey concluded that the majority of North American
hospitals do not determine the RBC phenotype of
nonalloimmunized patients with SCD (beyond ABO
and D) and the laboratories that do perform RBC
antigen testing of these patients beyond ABO and D
most commonly match for C, E, and K when
phenotype-similar RBCs are transfused.
The authors in this issue of Immunohematology
describe various transfusion management protocols
used for patients with SCD for whom they care in their
institutions: those designated as comprehensive sickle
cell centers and others around the country. All of these
centers actively treat patients with SCD. Given the lack
of consensus in the United States regarding the need to
perform RBC phenotyping of patients with SCD, and
the use of phenotypically-matched donor RBCs for
their transfusions, it will be interesting to read the
articles that follow with regard to alloimmunization
and to the use of leukocyte-reduced, irradiated, storage
duration limited, and HbS negative RBC components.
References
1. National Institutes of Health, National Heart, Lung,
and Blood Institute. Facts about sickle cell anemia.
Washington, DC: National Institutes of Health;
1996. NIH publication 96-4057.
2. Claster S, Vichinsky EP. Clinical review: managing
sickle cell disease. BMJ 2003;327:1151-5.
3. National Institutes of Health, National Heart, Lung,
and Blood Institute. Management of sickle cell
disease. 4th ed.Washington, DC: National Institutes
of Health; 2002. NIH publication 02-2117.
http://www.nhlbi.nih.gov/health/prof/blood/
sickle/sc_mngt.pdf.
4. Vichinsky EP, Luban N, Wright E, Olivieri N.
Prospective RBC phenotype matching in a stroke-
prevention trial in sickle cell anemia: a multicenter
transfusion trial.Transfusion 2001;41:1086-92.
5. Vichinsky EP, Earles A, Johnson RA, Hoag MS.
Alloimmunization in sickle cell anemia and
transfusion of racially unmatched blood. N Engl J
Med 1990;322:1617-21.
6. Rosse WF, Gallagher D, Kinney TR, Castro O. The
cooperative study of sickle cell disease.Transfusion
and alloimmunization in sickle cell disease. Blood
1990;76:1431-7.
7. Davies SC, McWilliam AC, Hewitt PE, et al. Red cell
alloimmunization in sickle cell disease. Br J
Haematol 1986;63:241-5.
8. Orlina AR, Unger PJ, Koshy M. Post-transfusion
alloimmunization in patients with sickle cell
disease.Am J Hematol 1978;5:101-6.
9. Afenyi-Annan A, Brecher M: Pre-transfusion
phenotype matching for sickle cell disease
patients.Transfusion 2004;44:619-20.
10. Osby M, Shulman I. Phenotype matching of donor
red blood cell units for nonalloimmunized sickle
cell disease patients: a survey of 1182 North Amer-
ican laboratories. Arch Path Lab Med 2005; 129:
190-3.
Ira A. Shulman, MD, Professor and Vice Chair of
Pathology/Director of Transfusion Medicine Keck
School of Medicine at the University of Southern Cali-
fornia Department of Pathology, LAC+USC Medical
Center, 1200 N. State Street, Los Angeles, CA 90033.
I.A. SHULMAN


Prophylactic phenotype matching of donors for the transfusion of nonalloimmunized patients with sickle cell disease

Flickinger, Cynthia

In search of red blood cells for alloimmunized patients with sickle cell disease

Nance, Sandra

Westhoff, Connie M.

Flickinger, Cindy

Thank you to contributors to the 2006 issues

Changes in the journal

ERRATUM: Vol.21, No.4, 2005, p.60

Norton, Alice

Allen, David L.

Murphy, Michael F.

Review: platelet alloantigens and antibodies and their clinical significance

Harmening, Denise

124 I M M U N O H E M A T O L O G Y, V O L U M E  2 0 , N U M B E R  2 , 2 0 0 4
C O M M U N I C A T I O N S  C O N T ’ D
Letter to the Editor-in-Chief
Looking back at the history of the journal
It’s hard to believe that 20 years have passed since
you asked me to write the first article for the very first
issue of your new journal, Vol. 1, No. 1, 1984,
Immunohematology. Each year, you and your staff
have managed to produce a prodigious series of
journals at a price which was, and still is, affordable to
all!  The journal has covered a wide range of topics, all
practical, timely, and important to the field of
immunohematology. In addition, the journal has
attracted an editorial board composed of individuals,
well known in the field, who have donated their time
and expertise in making it a quality peer-reviewed
publication. As the needs of blood bank technologists,
physicians, and educators have changed to reflect the
advances and redesign of transfusion medicine, so has
the focus of the journal kept pace over the past 20
years.
Immunohematology has come a long way and has
become a favorite journal of all blood bankers!  It was
the first of its kind to provide current and timely
articles on practical issues for blood bank serologists
and educators. In the beginning, and now, the journal
continues to provide wonderful review articles,
original scientific studies, unusual serologic cases, and
literature reviews.
The straightforward presentations of serologic case
studies make it an invaluable resource!  The actual
serologic characteristics of certain antibodies reported
in your journal has made it a convenient resource for
blood bank technologists to recognize unusual
antibodies. In addition, your willingness to guide and
nurture first-time authors has made the journal a great
success! 
Even after 20 years, Immunohematology remains a
well-balanced, timeless journal, designed for easy
reading,and infused with a richness of tradition!  Thank
you for a wonderful journal under your leadership!
The commitment and dedication of all those who have
contributed to this journal have made this publication
well acknowledged and admired by everyone in the
field. I would like to personally thank you and applaud
the journal for its many years of service to the
profession.
Denise Harmening, PhD, MT(ASCP)
Department of Medical & Research Technology
University of Maryland School of Medicine
Baltimore, MD 21201
IMPORTANT NOTICE ABOUT MANUSCRIPTS
FOR
IMMUNOHEMATOLOGY
Mail all manuscripts (original and 2 copies) to Mary H. McGinniss, Managing Editor, 10262 Arizona Circle,
Bethesda, MD  20817. In addition, please e-mail a copy to Marge Manigly at mmanigly@usa.redcross.org


Letter to the Editor-in-Chief: Looking back at the history of the journal

&nbsp;

Vassallo, Ralph R.

Review: IgA anaphylactic transfusion reactions. Part I. Laboratory diagnosis, incidence,and supply of IgAdeficient products

Greenwalt, Tibor J.

Letter to the Editor-in-Chief: My reminiscence of the early days of the American Red Cross Rare Donor Registry

González, Carlos A.

Guzmán, Liliana

Nocetti, Gabriela

Drug-dependent antibodies with immune hemolytic anemia in AIDS patients

Rinder, Henry M.

BOOK REVIEW: Platelets in Thrombotic and Non-thrombotic Disorders: Pathophysiology, Pharmacology, and Therapeutics

Not a member yet

Prophylactic phenotype matching of donors for the transfusion of nonalloimmunized patients with sickle cell disease

In search of red blood cells for alloimmunized patients with sickle cell disease

Thank you to contributors to the 2006 issues Changes in the journal

ERRATUM: Vol.21, No.4, 2005, p.60

Review: platelet alloantigens and antibodies and their clinical significance

Letter to the Editor-in-Chief: Looking back at the history of the journal

Review: IgA anaphylactic transfusion reactions. Part I. Laboratory diagnosis, incidence,and supply of IgAdeficient products

Letter to the Editor-in-Chief: My reminiscence of the early days of the American Red Cross Rare Donor Registry

Drug-dependent antibodies with immune hemolytic anemia in AIDS patients

BOOK REVIEW: Platelets in Thrombotic and Non-thrombotic Disorders: Pathophysiology, Pharmacology, and Therapeutics

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