Immunohematology JOURNAL of BLOOD GROUP SEROLOGY and EDUCATION
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Immunohematology JOURNAL OF BLOOD GROUP SEROLOGY AND EDUCATION V OLUME 22, NUMBER 3, 2006 This Issue of Immunohematology Is Supported by a Contribution From Dedicated to Education in the Field of Blood Banking Immunohematology JOURNAL OF BLOOD GROUP SEROLOGY AND EDUCATION VOLUME 22, NUMBER 3, 2006 CONTENTS 101 Prophylactic phenotype matching of donors for the transfusion of nonalloimmunized patients with sickle cell disease I.A. SHULMAN 103 Transfusion practices for patients with sickle cell disease at a major academic medical center A.AFENYI-ANNAN AND N. BANDARENKO 108 Partners for Life: the transfusion program for patients with sickle cell disease offered at the American Red Cross Blood Services, Southern Region,Atlanta, Georgia K.L. HILLYER,V.W.HARE, C.D. JOSEPHSON, S.B. HARRIS,AND C.D. HILLYER 112 The Charles Drew Program in Missouri: a description of a partnership among a blood center and several hospitals to address the care of patients with sickle cell disease E.J. ISAAK, B. LECHIEN,T. LINDSEY,AND M.R. DEBAUN 117 Children’s National Medical Center’s transfusion protocol for sickle hemoglobinopathies C.P. MINNITI,T. KRATOVIL,AND N.L.C. LUBAN 121 Transfusion support of patients with sickle cell disease at the Children’s Hospital of Philadelphia D.A. SESOK-PIZZINI,D.F.FRIEDMAN,K.SMITH-WHITLEY,AND S.J. NANCE 126 Review: clinical transfusion management in sickle cell disease Z.R. ROGERS 132 Serologic and molecular genetic management of a pregnancy complicated by anti-Rh18 R.L. HASPEL,S.VEGE,D.MICHELLE, R.M. KAUFMAN,AND C.M.WESTHOFF 136 In search of red blood cells for alloimmunized patients with sickle cell disease C. FLICKINGER 143 Molecular characterization of GYPB and RH of donors in the American Rare Donor Program S.VEGE AND C.M.WESTHOFF 148 150 151 IN MEMORIAM CLASSIFIED AD ERRATUM Robin Coombs 152 154 155 ANNOUNCEMENTS INSTRUCTIONS FOR AUTHORS ADVERTISEMENTS EDITORS-IN-CHIEF MANAGING EDITOR Sandra Nance, MS, MT(ASCP)SBB Cynthia Flickinger, MT(ASCP)SBB Philadelphia, Pennsylvania Philadelphia, Pennsylvania Connie M.Westhoff, SBB, PhD Philadelphia, Pennsylvania TECHNICAL EDITORS SENIOR MEDICAL EDITOR Christine Lomas-Francis, MSc Geralyn M. Meny, MD New York City, New York Philadelphia, Pennsylvania Dawn M. Rumsey,ART(CSMLT) Glen Allen, Virginia ASSOCIATE MEDICAL EDITORS David Moolton, MD Ralph R.Vassallo, MD Philadelphia, Pennsylvania Philadelphia, Pennsylvania EDITORIAL BOARD Patricia Arndt, MT(ASCP)SBB W.John Judd, FIBMS, MIBiol Mark Popovsky, MD Pomona, California Ann Arbor, Michigan Braintree, Massachusetts James P.AuBuchon, MD Christine Lomas-Francis, MSc Marion E. Reid, PhD, FIBMS Lebanon, New Hampshire New York City, New York New York City, New York Geoffrey Daniels, PhD Gary Moroff, PhD Susan Rolih, MS, MT(ASCP)SBB Bristol, United Kingdom Rockville, Maryland Cincinnati, Ohio Richard Davey, MD Ruth Mougey, MT(ASCP)SBB S. Gerald Sandler, MD Washington, District of Columbia Carrollton, Kentucky Washington, District of Columbia Sandra Ellisor, MS, MT(ASCP)SBB John J. Moulds, MT(ASCP)SBB David F.Stroncek, MD Anaheim, California Shreveport, Louisiana Bethesda, Maryland George Garratty, PhD, FRCPath Marilyn K. Moulds, MT(ASCP)SBB Marilyn J.Telen, MD Pomona, California Houston, Texas Durham, North Carolina Brenda J. Grossman, MD Paul M. Ness, MD St. Louis, Missouri Baltimore, Maryland EDITORIAL ASSISTANT PRODUCTION ASSISTANT Judith Abrams Marge Manigly COPY EDITORS ELECTRONIC PUBLISHER Lucy Oppenheim Paul Duquette Dawn M. Rumsey,ART(CSMLT) Glen Allen, Virginia Immunohematology is published quarterly (March, June, September, and December) by the American Red Cross, National Headquarters,Washington, DC 20006. Immunohematology is indexed and included in Index Medicus and MEDLINE on the MEDLARS system. The contents are also cited in the EBASE/Excerpta Medica and Elsevier BIOBASE/Current Awareness in Biological Sciences (CABS) databases. The subscription price is $30.00 (U.S.) and $35.00 (foreign) per year. Subscriptions, Change of Address, and Extra Copies: Immunohematology, P.O. Box 40325 Philadelphia, PA 19106 Or call (215) 451-4902 Web site: www.redcross.org/pubs/immuno Copyright 2006 by The American National Red Cross ISSN 0894-203X Prophylactic phenotype matching of donors for the transfusion of nonalloimmunized patients with sickle cell disease I.A. SHULMAN Sickle cell disease (SCD) affects approximately addition, hemolytic transfusion reactions can be 72,000 people in the United States (approximately 1 in serious and potentially life-threatening complications 500 African Americans and 1 in 1000 to 1400 in their own right. Studies have shown that the Hispanics).1 During the past three decades, the transfusion of ABO- and D-compatible donor RBCs that survival of patients with SCD has improved lack C, E, or K when the same antigens are absent from significantly, partly because transfusion therapy2,3 has the recipient’s RBCs can reduce the rate of alloimmuni- played a role as a disease-modifying strategy for zation in patients with SCD as well as the occurrence several manifestations of the disease, including acute of hemolytic transfusion reactions. chest syndrome,acute intrahepatic sequestration,acute According to a National Institutes of Health (NIH) multiorgan failure syndrome, acute splenic sequestra- publication3 (pages 155–158) the antigenic phenotype of the tion, acute symptomatic anemia, aplastic crisis, RBCs (at least the common antigens belonging to ABO, bacterial and malarial infections, chronic organ failure, Rh, Kell, Duffy, Kidd, Lewis, Lutheran, P, and MNS) complicated pregnancy, eye surgery (e.g., scleral should be determined in all patients older than 6 buckle procedure), frequent pain episodes, priapism, months. A permanent record of the phenotype results primary and secondary prevention or treatment of should be maintained in the blood bank to optimize stroke, and surgery requiring general anesthesia. matching and a copy of the record should be given to Depending on clinical circumstances, patients with the patient or family. Furthermore, limited matching of one or more of the aforementioned manifestations may donor RBCs for E, C, and K is usually performed, unless receive simple or exchange RBC transfusions that are patients have antibodies. The transfusion of pheno- administered episodically or chronically. However, typically matched RBCs may be accomplished by each transfusion of a unit of RBCs poses a risk of RBC typing the RBCs of the patient before transfusion,if not antigen alloimmunization. Studies show that 25 previously performed, for Rh and Kell, in particular E, percent or more of chronically transfused SCD patients C, and K and performing more extensive matching for may experience this complication unless precautions antigens in systems such as Duffy, Kidd, and MNS for are taken.4 When alloimmunization occurs, the most those patients who are already alloimmunized. Donor common clinically significant antibodies to develop in RBCs lacking the antigens that the patient’s RBCs lack transfused SCD patients include antibodies to Rh and would then be selected for transfusion. Kell antigens.5–8 Although NIH publication 02-2117 was not the It is preferable to avoid alloimmunization to RBC result of a formalized consensus process, it does antigens because this complication makes obtaining represent the efforts of those who have dedicated their compatible units for future transfusions difficult and professional careers to the care of individuals with places the patient at risk for development of hemolytic SCD. In spite of this publication, transfusion practices transfusion reactions, the symptoms of which can in SCD remain disparate in the United States, as mimic or trigger serious complications of SCD. In illustrated by two recent national surveys. IMMUNOHEMATOLOGY, VOLUME 22, NUMBER 3, 2006 101 I.A. SHULMAN Afenyi-Annan and Brecher surveyed 50 academic articles that follow with regard to alloimmunization medical centers to determine the extent of the use of and to the use of leukocyte-reduced, irradiated, storage phenotype-matched donor RBCs in the transfusion of duration limited, and HbS negative RBC components. patients with SCD.9 Of 37 academic centers that responded to a questionnaire, 27 (73%) reported that References they provide antigen-matched donor RBCs. Although 1. National Institutes of Health, National Heart, Lung, the most common antigens matched were C, E, and K and Blood Institute. Facts about sickle cell anemia. (24 of the 27 sites or 89%), there were multiple Washington, DC: National Institutes of Health; variances among those 27 centers as to which antigens 1996. NIH publication 96-4057. were selected for phenotype matching. The authors 2. Claster S, Vichinsky EP. Clinical review: managing concluded that, despite a common approach by some sickle cell disease. BMJ 2003;327:1151-5. of the academic centers, there was no single standard 3. National Institutes of Health, National Heart, Lung, of care with regard to RBC component selection. A survey by the College of American Pathologists and Blood Institute. Management of sickle cell (CAP) examined 1182 labs in North America to disease. 4th ed.Washington, DC: National Institutes determine the extent of RBC antigen testing of of Health; 2002. NIH publication 02-2117. nonalloimmunized patients with SCD as well as the use http://www.nhlbi.nih.gov/health/prof/blood/ of phenotype-matched donor RBCs in the transfusion sickle/sc_mngt.pdf. of these patients.10 The CAP survey, unlike the one by 4. Vichinsky EP, Luban N, Wright E, Olivieri N. Afenyi-Annan and Brecher, did not specifically target Prospective RBC phenotype matching in a stroke- academic medical centers. The CAP survey results prevention trial in sickle cell anemia: a multicenter indicated that approximately 63 percent of North transfusion trial.Transfusion 2001;41:1086-92. American laboratories did not routinely phenotype 5. Vichinsky EP, Earles A, Johnson RA, Hoag MS. patients with SCD for antigens other than ABO and D. Alloimmunization in sickle cell anemia and Of the 37 percent of the laboratories that did perform transfusion of racially unmatched blood. N Engl J additional antigen testing beyond ABO and D, only 75 Med 1990;322:1617-21.