Nomenclature for factors of the HLA system, 2010

Abstract

The WHO Nomenclature Committee for Factors of the HLA System has met several times since the publication of the last major report in 2010.1 It met most recently in September 2023, to discuss additions to the serological defined HLA antigens. This report documents the additions and revisions to the nomenclature of HLA specificities following the principles established in previous reports.1-19 Links to these reports and details of HLA Nomenclature can be found on the website: hla.alleles.org The HLA-OLI pseudogene has been reported and officially named HLA-R. A full list of all recognised HLA genes is given in Table 1.20 Although at present it is only a recommendation that full-length sequences of the coding region of novel alleles be submitted, it was widely felt that in the future this should become a requirement for submission. Such requirement would remove many of the currently encountered ambiguities in the assignment of names to alleles for which partial sequences have been submitted and should not be burdensome as sequencing techniques have improved substantially since the submission conditions were first devised. In cases where novel mutations or polymorphisms are detected in non-coding regions of the gene, it will be a requirement that full-length sequences be submitted of both the novel allele and its most closely related allele. It should be noted with some caution that cells from which only partial sequences have been obtained may later be shown to have different or novel alleles when further sequencing is performed. This is of particular importance in cases where partial sequences of what appears to be the same allele have been obtained from several different cells. In such cases, all cells studied have been listed in this report. The list of those genes in the HLA region considered by the WHO Nomenclature Committee for Factors of the HLA System is given in Table 1. Current practice is that official designations will be promptly assigned to newly described alleles in periods between Nomenclature Committee meetings, provided that the submitted data and its accompanying description meet the criteria outlined above. A list of the newly reported alleles is published every three months in nomenclature updates in the journals HLA, Human Immunology and the International Journal of Immunogenetics. The listing of references to new sequences does not imply priority of publication. The use of numbers or names for alleles, genes or specificities which pre-empt assignment of official designations by the Nomenclature Committee is strongly discouraged. A total of 43,758 HLA alleles have been named as of December 2025. A complete listing of the numbers of alleles assigned for each HLA genes is given in Table 2. In September 2023, the WHO Nomenclature Committee for Factors of the HLA System met at the Stanford Blood Center located at Stanford University following the 18th International HLA and Immunogenetics Workshop held in Noordwijkerhout, the Netherlands in May 2022. The committee met to evaluate a proposal for the definition of additional and novel antigens defined in silico21. The in silico definition of HLA antigens was achieved by the systematic examination and cataloguing the amino acid (AA) replacements at specific residues determining epitopes (DEP) in all common HLA alleles at the classical HLA class I and class II loci [Common and Well Documented (CWD2.0) for alleles of HLA-DRB3, -DRB4, -DRB5, -DQA1 and -DPA1 and Common, Intermediate and Well Documented (CIWD3.0) for alleles of HLA-A, -B, -C, -DRB1, -DQB1 and -DPB1].21-23 The committee voted to accept the proposed serologic nomenclature update provided that a validation study was conducted. The manuscript aiming to confirm these computationally predicted antigens defined by DEP residues for the 11 HLA loci was designed to assess and compare the antibody reactivity of patients' sera in solid phase assays with Single Antigen Bead (SAB) preparations from various HLA proteins24. The differences in correlation confirmed the distinctions between proposed associated antigens and identified a few additional antigens.21, 25 A full listing of all the serological specificities for HLA-A, -B, -C, -DR, -DQ, and -DP and cellular defined specificities for HLA-Dw and HLA-DPw are given in Table 3. The concept of an HLA Associated Antigen was introduced in 1991, with the understanding that serological types would be more closely associated with the allele sequence defining them. Thirteen associated antigens were named at this time: A203, A210, A2403, B703, B3901, B3902, B4005, B5102, B5103, B7801, DR103, DR1403, and DR1404.12 In 1996 the B2708 associated antigen was named and it was decided to shorten the B7801 antigen name to B78.15 Following the definition of novel associated antigens documented below, it has been necessary to update the names of four of these: A203, A210, B703, and DR103, have been updated to A0203, A0210, B0703, and DR0103. A full listing of all the Associated Antigens for HLA-A, -B, -C, −and -DR are given in Table 4. This report includes nomenclature for the serological specificities encoded by both the HLA-DQA1 and HLA-DQB1 genes. As such it is now possible to define a nomenclature for the paired combination of both subunits of HLA-DQ molecules. Table 5 lists those proteins encoded in cis by common DQ haplotype blocks. The specificities resulting from trans-encoded heterodimers should be presented in the same format. The newly assigned HLA antigens and associated antigens will be implemented in April 2026 and will be made available through the IPD-IMGT/HLA Database (www.ebi.ac.uk/ipd/imgt/hla) with the April 2026 release of the database.26-28 The IPD-IMGT/HLA Database continues to act as the official repository for HLA sequences named by the WHO Nomenclature Committee for Factors of the HLA System.26-28 The database contains sequences for all HLA alleles officially recognised by the WHO Nomenclature Committee for Factors of the HLA System and provides users with online tools and facilities for their retrieval and analysis. These include allele reports, alignment tools, and detailed descriptions of the source cells. The online IPD-IMGT/HLA Database submission tool allows both new and confirmatory sequences to be submitted directly to the WHO Nomenclature Committee. New releases of the database are made every three months, in January, April, July and October, with the latest version (release 3.63.0 January 2026) containing 43,758 HLA alleles. The database may be accessed via the worldwide web at www.ebi.ac.uk/ipd/imgt/hla. The IPD-IMGT/HLA Database is currently supported by the following organisations: NMDP, TxMiller Foundation, CareDx, DKMS, Gift of Life, Werfen, Scisco Genetics, the European Federation for Immunogenetics (EFI), GenDx, Pirche, ThermoFisher, the American Society for Histocompatibility and Immunogenetics (ASHI), LabCorp, Histogenetics, the Asia-Pacific Histocompatibility and Immunogenetics Association (APHIA), BAG Diagnostics, Protrans, Inno-train, and Anthony Nolan. The Committee would like to thank Dominic Barker, Michael Cooper, Sebastian Hopper and Surayia Akter for their work with the IPD-IMGT/HLA Database. Also thanked is Andy Yates and the staff at the European Bioinformatics Institute for their continued support of the IPD-IMGT/HLA Database. We would also like to thank the many organisations that provide financial support for the IPD-IMGT/HLA Database and Benjamin Hester of the ‘NMDP Foundation’ for his work in soliciting and coordinating the funding of this project. We are grateful to Stanford Blood Center for covering the cost for providing open access for this article. SGE Marsh, University College London, London, UK (Chairman) WF Bodmer, Oncology Department, Oxford University, Oxford, UK MN Carrington, Basic Science Program, Frederick National Laboratory for Cancer Research, National Cancer Institute, Frederick, MD, USA and Laboratory of Integrative Cancer Immunology, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA & Ragon Institute of MGH, MIT, and Harvard, Cambridge, MA, USA HA Erlich, Benioff UCSF Children's Hospital Oakland Research Institute, Oakland, USA M Fernández-Viña, Stanford Blood Center, Palo Alto, USA & Department of Pathology, Stanford University School of Medicine, Stanford, USA S Heidt, Erasmus Medical Center, Rotterdam, & Leiden University Medical Center, Leiden, The Netherlands R Holdsworth, University of Melbourne, Melbourne, Australia WR Mayr, University of Vienna, Vienna, Austria M Maiers, Center for International Blood and Marrow Transplant Research, (CIBMTR), NMDP, Minneapolis, USA P Parham, Stanford University School of Medicine, Stanford, USA EW Petersdorf, Fred Hutchinson Cancer Center, Seattle, USA J Robinson, Anthony Nolan Research Institute, & University College London, London, UK J Trowsdale, Cambridge University, Cambridge, UK RE Bontrop, Biomedical Primate Research Centre, Rijswijk, The Netherlands K Osoegawa, Stanford Blood Center, Palo Alto, USA New sequences should be communicated to the WHO Nomenclature Committee for Factors of the HLA System via the sequence submission tool of the IPD-IMGT/HLA Database to receive official names, www.ebi.ac.uk/ipd/imgt/hla. The data that support the findings of this study are available from the corresponding author upon reasonable request.

Shown for reading. Its publisher allows quoting it, not republishing it.

References 444

1. 1.

   Nomenclature Committee WHO. Nomenclature for Factors of the HL-A System. Bull Wld Hlth Org. 1968;39:483-6.

   Not in our library

2. 2.

   Terasaki PI. WHO Terminology Report. Histocompatibility Testing 1970. 1970;49.

   Not in our library

3. 3.

   WHO Terminology Report. Bull Wld Hlth Org. 1972;47:659-62.

   Not in our library

4. 4.

   Nomenclature for Factors of the HLA System. Bull Wld Hlth Org. 1975;52:261.

   Not in our library

5. 5.

   Nomenclature for Factors of the HLA System. Bull Wld Hlth Org 1977. 1978;56:461-5.

   Not in our library

6. 6.

   Terasaki PI. Nomenclature for Factors of the HLA System, 1980. Histocompatibility Testing 1980. 1980;18-20.

   Not in our library

7. 7.

   Albert ED, Baur MP, Mayr WR. Nomenclature for factors of the HLA system 1984. Histocompatibility Testing. 1985;4-8.

   Not in our library

8. 8.

   Bodmer WF, Albert E, Bodmer JG, Dupont B, et al. Nomenclature for factors of the HLA system, 1987. Immunobiology of HLA. 1989;72-9.

   Not in our library

9. 9.

   Bodmer JG, Marsh SGE, Parham P, et al. Nomenclature for factors of the HLA system, 1989. Tissue Antigens. 1990;35:1-8.

   Not in our library10.1111/j.1399-0039.1990.tb01749.x

10. 10.

    Bodmer JG, Marsh SGE, Albert E, et al. Nomenclature for factors of the HLA system. Tissue Antigens 1990. 1991;37:97-104.

    Not in our library10.1111/j.1399-0039.1991.tb01853.x

11. 11.

    Bodmer JG, Marsh SGE, Albert E, Tsuji T, Aizawa M, Sasazuki T, et al. Nomenclature for factors of the HLA system, 1991. HLA 1991. 1992;17-31.

    Not in our library

12. 12.

    Bodmer JG, Marsh SGE, Albert E, et al. Nomenclature for factors of the HLA system. Tissue Antigens 1994. 1994;44:1-18.

    Not in our library10.1111/j.1399-0039.1994.tb02351.x

13. 13.

    Bodmer JG, Marsh SGE, Albert E, et al. Nomenclature for factors of the HLA system. Tissue Antigens 1995. 1995;46:1-18.

    Not in our library10.1111/j.1399-0039.1995.tb02470.x

14. 14.

    Bodmer JG, Marsh SGE, Albert ED, et al. Nomenclature for factors of the HLA system. Tissue Antigens 1996. 1997;49:297-321.

    Not in our library10.1111/j.1399-0039.1997.tb02759.x

15. 15.

    Bodmer JG, Marsh SGE, Albert ED, et al. Nomenclature for factors of the HLA system, 1998. Tissue Antigens. 1999;53:407-46.

    Not in our library10.1034/j.1399-0039.1999.530421.x

16. 16.

    Marsh SGE, Bodmer JG, Albert ED, et al. Nomenclature for factors of the HLA system. Tissue Antigens 2000. 2001;57:236-83.

    Not in our library10.1034/j.1399-0039.2001.057003236.x

17. 17.

    Marsh SGE, Albert ED, Bodmer WF, et al. Nomenclature for factors of the HLA system, 2002. Tissue Antigens. 2002;60:407-64.

    Not in our library10.1034/j.1399-0039.2002.600509.x

18. 18.

    Marsh SGE, Albert ED, Bodmer WF, et al. Nomenclature for factors of the HLA system. Tissue Antigens 2004. 2005;65:301-69.

    Not in our library10.1111/j.1399-0039.2005.00379.x

19. 19.

    Geraghty DE, Koller BH, Hansen JA, Orr HT. The HLA class I gene family includes at least six genes and twelve pseudogenes and gene fragments. J Immunol. 1992;149:1934-46.

    Not in our library

20. 20.

    Williams F, Curran MD, Middleton D. Characterisation of a novel HLA-A pseudogene, HLA-BEL, with significant sequence identity with a gorilla MHC class I gene. Tissue Antigens. 1999;54:360-9.

    Not in our library10.1034/j.1399-0039.1999.540405.x

21. 21.

    Coquillard G, Lau M, Kletzel M, Rodriguez-Marino SG. Identification of two pseudogenes with sequence homology to human and gorilla MHC class IA genes: ancestral haplotype in the Filipino population. Hum Immunol. 2004;65:665-73.

    Not in our library10.1016/j.humimm.2004.02.025

22. 22.

    Hurley CK, Schreuder GMT, Marsh SGE, Lau M, Middleton D, Noreen H. The search for HLA-matched donors: a summary of HLA-A*, -B*, -DRB1/3/4/5* alleles and their association with serologically defined HLA-A, -B, -DR antigens. Tissue Antigens. 1997;50:401-18.

    Not in our library10.1111/j.1399-0039.1997.tb02894.x

23. 23.

    Schreuder GMT, Hurley CK, Marsh SGE, et al. The HLA dictionary 1999: A summary of HLA-A, -B, -C, -DRB1/3/4/5, -DQB1 alleles and their association with serologically defined HLA-A, -B, -C, -DR and -DQ antigens. Tissue Antigens. 1999;54:409-37.

    Not in our library10.1034/j.1399-0039.1999.540412.x

24. 24.

    Schreuder GMT, Hurley CK, Marsh SGE, et al. The HLA dictionary 2001: A summary of HLA-A, -B, -C, -DRB1/3/4/5, -DQB1 alleles and their association with serologically defined HLA-A, -B, -C, -DR and -DQ antigens. Tissue Antigens. 2001;58:109-40.

    Not in our library10.1034/j.1399-0039.2001.580210.x

25. 25.

    Schreuder GMT, Hurley CK, Marsh SGE, et al. The HLA dictionary 2004: A summary of HLA-A, -B, -C, -DRB1/3/4/5 and -DQB1 alleles and their association with serologically defined HLA-A, -B, -C, -DR and -DQ antigens. Tissue Antigens. 2005;65:1-55.

    Not in our library10.1111/j.1399-0039.2005.00350.x

26. 26.

    Holdsworth R, Hurley CK, Marsh SGE, et al. The HLA dictionary 2008: A summary of HLA-A, -B, -C, -DRB1/3/4/5, and -DQB1 alleles and their association with serologically defined HLA-A, -B, -C, -DR, and -DQ antigens. Tissue Antigens. 2009;73:95-170.

    Not in our library10.1111/j.1399-0039.2008.01183.x

27. 27.

    Robinson J, Malik A, Parham P, Bodmer JG, Marsh SGE. IMGT/HLA database–a sequence database for the human major histocompatibility complex. Tissue Antigens. 2000;55:280-7.

    Not in our library10.1034/j.1399-0039.2000.550314.x

28. 28.

    Robinson J, Waller MJ, Fail SC, et al. The IMGT/HLA database. Nucleic Acids Res. 2009;37:D1013-7.

    Not in our library10.1093/nar/gkn662

29. 29.

    Stewart CA, Horton R, Allcock RJ, et al. Complete MHC haplotype sequencing for common disease gene mapping. Genome Res. 2004;14:1176-87.

    Not in our library10.1101/gr.2188104

30. 30.

    Zhu F, He Y, Zhang W, He J, Xu X, Yan L. Analysis of the complete genomic sequence of HLA-A alleles in the Chinese Han population. Int J Immunogenet. 2009;36:351-60.

    Not in our library10.1111/j.1744-313x.2009.00874.x

31. 31.

    Yu M, Hall JE, Hartman K, et al. A novel human leukocyte antigen-A*01 allele, A*010104, identified by polymerase chain reaction-sequence-based typing in a Caucasian hematopoietic stem cell donor. Tissue Antigens. 2006;68:263.

    Not in our library10.1111/j.1399-0039.2006.00642.x

32. 32.

    Reinders J, Rozemuller EH, Otten HG, et al. Identification of HLA-A*0111N: a synonymous substitution, introducing an alternative splice site in exon 3, silenced the expression of an HLA-A allele. Hum Immunol. 2005;66:912-20.

    Not in our library10.1016/j.humimm.2005.06.010

33. 33.

    Lazaro AM, Xiao Y, Cao K, et al. Thirty-two novel HLA-A alleles identified during intermediate resolution testing. Tissue Antigens. 2008;71:165-8.

    Not in our library10.1111/j.1399-0039.2007.00977.x

34. 34.

    Crowley J, Dunne C, Cox ST, Little AM. An unlikely rare result of HLA-A*0236, *3601 masking the presence of a novel allele A*0114. Tissue Antigens. 2007;69:200-1.

    Not in our library10.1111/j.1399-0039.2006.00748.x

35. 35.

    Bera O, Dubois V, Assaqa A, Belon P, Favre-Victoire I, Gebuhrer L. A deletion in exon 3 results in a new HLA-A*01 null allele (A*0115N) in a Martinican woman. Tissue Antigens. 2006;67:61-3.

    Not in our library10.1111/j.1399-0039.2005.00528.x

36. 36.

    Lazaro AM, Xiao Y, Regenscheid A, Ng J, Hurley CK, Posch PE. Characterization of 104 novel alleles at the HLA-A, -B, and -DRB1 loci from National Marrow Donor Program volunteer donors. Tissue Antigens. 2009;73:364-72.

    Not in our library10.1111/j.1399-0039.2009.01226.x

37. 37.

    Henry JB, Hubbell CA, Davis MC, Fernandez-Vina MA, Yunis EJ, Shrimpton AE. A new HLA-A1 mutation: a novel, null variant allele. Am J Clin Pathol. 2004;122:185-92.

    Not in our library10.1309/8de1-2yu6-5bkh-hqw6

38. 38.

    Schlaf G, Wahle A, Seliger B, Altermann WW. A novel HLA-A*0119 allele of a Caucasian family containing a mutation in a highly conserved region. Int J Immunogenet. 2006;33:247-8.

    Not in our library10.1111/j.1744-313x.2006.00606.x

39. 39.

    Longhi E, Frison S, Andreini E, Karpasitou K, Serafini M, Poli F. Description of HLA-A*0127N, a novel nonexpressed allele identified by SBT. Hum Immunol. 2007;68:913-4.

    Not in our library10.1016/j.humimm.2007.08.261

40. 40.

    Shuang C, Shan X, Zhang Z. A novel allele, HLA-A*0128, identified by sequence-based typing in a Chinese individual. Tissue Antigens. 2008;72:599-600.

    Not in our library10.1111/j.1399-0039.2008.01128.x

Cited by papers we hold 11

• HLA‐associated adverse drug reactions ‐ scoping review · 2021
  Not checked
• Common, intermediate and well‐documented HLA alleles in world populations: CIWD version 3.0.0 · 2020
  No notice
• IPD-IMGT/HLA Database · 2019
  No notice
• The Immune Epitope Database and Analysis Resource in Epitope Discovery and Synthetic Vaccine Design · 2017
  No notice
• Distinguishing functional polymorphism from random variation in the sequences of >10,000 HLA-A, -B and -C alleles · 2017
  No notice
• HLA Typing for the Next Generation · 2015
  No notice
• Mapping Bias Overestimates Reference Allele Frequencies at the HLA Genes in the 1000 Genomes Project Phase I Data · 2015
  No notice
• Determination of HLA‐A, ‐C, ‐B, ‐DRB1 allele and haplotype frequency in Japanese population based on family study · 2015
  No notice
• The IPD and IMGT/HLA database: allele variant databases · 2014
  No notice
• Cost-efficient high-throughput HLA typing by MiSeq amplicon sequencing · 2014
  No notice
• Derivation of HLA types from shotgun sequence datasets · 2012
  No notice

Reads

A person counts once a day per paper. We keep no cookie and no address: a reader is a hash that changes every day. Any month under five reads shows no number.