Vel Blood Group System: a Review J.R
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Journal of Blood Group Serology and Molecular Genetics VOLUME 33, N UMBER 2, 2017 This issue of Immunohematology is supported by a contribution from Grifols Diagnostics Solutions, Inc. Dedicated to advancement and education in molecular and serologic immunohematology Immunohematology Journal of Blood Group Serology and Molecular Genetics Volume 33, Number 2, 2017 CONTENTS C ASE R EPO R T 51 Hematologic complications in a patient with Glycine soja polyagglutination following fresh frozen plasma transfusion R.P. Jajosky, L.O. Cook, E. Manaloor, J.F. Shikle, and R.J. Bollag R EVIEW 56 The Vel blood group system: a review J.R. Storry and T. Peyrard C ASE R EPO R T 60 Two cases of the variant RHD*DAU5 allele associated with maternal alloanti-D J.A. Duncan, S. Nahiriniak, R. Onell, and G. Clarke R EVIEW 64 The FORS awakens: review of a blood group system reborn A.K. Hult and M.L. Olsson C ASE R EPO R T 73 A suspected delayed hemolytic transfusion reaction mediated by anti-Joa R.P. Jajosky, W.C. Lumm, S.C. Wise, R.J. Bollag, and J.F. Shikle R EVIEW 76 Recognizing and resolving ABO discrepancies G.M. Meny B OOK R EVIEW 82 Bloody Brilliant: A History of Blood Groups and Blood Groupers S. Gerald Sandler 84 90 94 96 A NNOUN C EMENTS A DVE R TISEMENTS I NST R U C TIONS S U B S cr IPTION FO R A UTHO R S I NFO R M AT I O N E DITO R - IN -C HIEF E DITO R IAL B OA R D Sandra Nance, MS, MT(ASCP)SBB Philadelphia, Pennsylvania Patricia Arndt, MT(ASCP)SBB Geralyn M. Meny, MD Pomona, California San Antonio, Texas M ANAGING E DITO R Barbara J. Bryant, MD Paul M. Ness, MD Cynthia Flickinger, MT(ASCP)SBB Galveston, Texas Baltimore, Maryland Wilmington, Delaware Lilian M. Castilho, PhD Thierry Peyrard, PharmD, PhD Campinas, Brazil Paris, France TEC HNI C AL E DITO R S Christine Lomas-Francis, MSc Martha R. Combs, MT(ASCP)SBB S. Gerald Sandler, MD New York City, New York Durham, North Carolina Washington, District of Columbia Joyce Poole, FIBMS Geoffrey Daniels, PhD Ira A. Shulman, MD Bristol, United Kingdom Bristol, United Kingdom Los Angeles, California Dawn M. Rumsey, ART(CSMLT) Anne F. Eder, MD Jill R. Storry, PhD Washington, District of Columbia Lund, Sweden Norcross, Georgia Melissa R. George, DO, FCAP Nicole Thornton S ENIO R M EDI C AL E DITO R Hershey, Pennsylvania Bristol, United Kingdom David Moolten, MD Julie K. Karp, MD Philadelphia, Pennsylvania Philadelphia, Pennsylvania E ME R ITUS E DITO R S Jose Lima, MD Delores Mallory, MT(ASCP)SBB A SSO C I AT E M EDI C AL E DITO R S Douglassville, Georgia Supply, North Carolina P. Dayand Borge, MD Philadelphia, Pennsylvania Christine Lomas-Francis, MSc Marion E. Reid, PhD, FIBMS New York City, New York Bristol, United Kingdom Corinne L. Goldberg, MD Durham, North Carolina M OLEC ULA R E DITO R Margaret A. Keller, PhD Philadelphia, Pennsylvania Immunohematology is published quarterly (March, June, September, and December) by the E DITO R IAL A S S I S TA N T American Red Cross, National Headquarters, Washington, DC 20006. Linda Frazier 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 P R ODU C TION A S S I S TA N T BIOBASE/Current Awareness in Biological Sciences (CABS) databases. Marge Manigly The subscription price is $50 for individual, $100 for institution (U.S.), and $60 for individual, $100 for institution (foreign), per year. C OPY E DITO R Subscriptions, Change of Address, and Extra Copies: Frederique Courard-Houri Immunohematology, P.O. Box 40325 Philadelphia, PA 19106 P R OOF R EADE R Wendy Martin-Shuma Or call (215) 451-4902 Web site: www.redcrossblood.org/hospitals/immunohematology E LEC T R ONI C P U B LISHE R Copyright 2017 by The American National Red Cross Paul Duquette ISSN 0894-203X O N O U R C OVE R Girl with a Red Hat (1666) by Johannes Vermeer is an example of a tronie, derived from Dutch meaning “face” and referring to a stylized work common to the Dutch Golden Age that, distinct from formal portrait painting, featured an anonymous model with an exaggerated expression or depicted as an iconic character. Girl with a Red Hat employs bold, rich colors, and the girl’s eye-catching hat and robe and informal turn to face the viewer are striking and engaging. Vermeer created only two other panel works, and the painting’s flamboyance and use of wood as a support medium have caused some to question its attribution, though dendrological analysis as well as technique considerations favor Vermeer. One of Vermeer’s more famous works, at just 9 × 71/16 inches, Girl with a Red Hat is also one of his smallest. Small integral membrane protein 1 (SMIM1) is the subject of an article in this issue. David Moolten, MD CASE R EPO R T Hematologic complications in a patient with Glycine soja polyagglutination following fresh frozen plasma transfusion R.P. Jajosky, L.O. Cook, E. Manaloor, J.F. Shikle, and R.J. Bollag Polyagglutination is a rare and underdiagnosed condition, to infection1,2 rather than to a congenital or somatic mutation. characterized by agglutination of red blood cells (RBCs) with T activation is the most common form of microbial-induced almost all ABO-compatible adult sera. Polyagglutination can polyagglutination and serves as a prototypical example occur when a cryptantigen is exposed on RBCs via microbial 3 enzyme activity. Because nearly all adults naturally produce (Fig. 1). Antibodies against cryptantigens are naturally antibodies against cryptantigens, transfusion of plasma can cause occurring and are usually IgM.3 The patient’s own antibodies unexpected hemolysis and hematologic complications, such as may mediate hemolysis.4 However, it is more common for thrombocytopenia and disseminated intravascular coagulation, in patients whose cryptantigens are exposed. We report a case hemolysis to occur after the transfusion of blood components of Glycine soja polyagglutination occurring in a 60-year-old that contain plasma.5–7 Thrombocytopenia may develop African-American man with disseminated methicillin-resistant because of the presence of the cryptantigen on platelets Staphylococcus aureus (MRSA) infection. Prior to transfusion, (PLTs).8,9 In addition, coagulopathies, such as disseminated the patient developed severe anemia of unknown etiology. 5,10 Following transfusion of 3 units of fresh frozen plasma (FFP), his intravascular coagulation (DIC), often occur. RBC count could not be determined for 24 days because of RBC agglutination in his blood sample. In addition, the FFP transfusion correlated with the rapid development of severe, transfusion- Sialidase refractory thrombocytopenia and anemia. The perplexed clinic- al team consulted the blood bank. A direct antiglobulin test demonstrated 1+ mixed-field reactivity with both monoclonal anti-IgG and anti-C3d. Lectin panel testing showed reactivity with only Glycine soja, confirming the condition. Subsequently, plasma components were avoided, and RBC and platelet (PLT) components were washed prior to transfusion. After a 44-day hospitalization involving the transfusion of 22 units of RBCs and 13 units of PLTs, the patient was discharged to a long-term care facility. The patient’s confounding hematologic complications can best be explained by polyagglutination, which developed Healthy RBC Membrane Abnormal RBC Membrane secondary to the severe MRSA infection. The FFP transfusion likely passively transferred antibodies that bound to the patient’s Fig. 1 Microbial enzymes such as sialidases (circle sector shape) RBC cryptantigens, leading to RBC agglutination and anemia. can enzymatically remove N-acetylneuraminic acid (triangles) from The development of severe thrombocytopenia may be related to RBC antigens. This action exposes normally hidden cryptantigens, cryptantigen exposure on the patient’s PLTs. Although difficult to such as T (shown on the right), which can subsequently bind anti-T. identify, polyagglutination needs to be recognized to appropriately This particular type of polyagglutination is known as T activation. manage hemotherapy. The purpose of this case study is to report This form is often described in pediatric patients with necrotizing hematologic complications following FFP transfusion in a patient enterocolitis or atypical hemolytic uremic syndrome caused by with Glycine soja polyagglutination, a rarely described condition. Streptococcus pneumoniae. T is also present on platelets and Immunohematology 2017;33:51–55. glomerular endothelial cells. The circle designates D-galactose. The square designates N-acetyl-D-galactosamine. R = Remainder of molecule; RBC = red blood cell. Key Words: unclassified polyagglutination, Glycine soja, Staphylococcus aureus, lectin, minor crossmatch, T activation In the past, polyagglutination was readily detected by blood Polyagglutination describes the agglutination of red blood centers when ABO typing resulted in ABO discrepancies.8,11 cells (RBCs) that occurs with nearly all compatible adult This finding occurred because human-source sera containing sera.1,2 It arises through an alteration of glycoprotein moieties antibodies against cryptantigens were used for blood typing. on the RBC membrane. This condition is usually secondary The transition to monoclonal antibodies for blood typing IMMUNOHEMATOLOGY, Volume 33, Number 2, 2017 51 R.P. Jajosky et al. eliminated these ABO discrepancies. In addition, most two incision and drainage (I&D) procedures of the septic blood centers do not routinely screen for polyagglutination.12 knee. Subsequently, his hemoglobin declined from 11.4 to Therefore, polyagglutination is becoming under-recognized. 7.1 g/dL (reference range: 14.0–18.0 g/dL) for unknown To identify polyagglutination, the condition must be suspected reasons (Fig. 2). The patient’s blood typed as group A, D+, by either clinicians or blood bank personnel, and additional and multiple RBC units were transfused.