title: B-Cell and Classical Hodgkin Lymphomas Associated With Immunodeficiency: 2015 SH/EAHP Workshop Report-Part 2.
abstract: OBJECTIVE
  The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology submitted small and large B-cell lymphomas (BCLs), including classical Hodgkin lymphoma (CHL), in the context of immunodeficiency.
  
  
  METHODS
  Clinicopathologic and molecular features were studied to explore unifying concepts in malignant B-cell proliferations across immunodeficiency settings.
  
  
  RESULTS
  Cases submitted to the workshop spanned small BCLs presenting as nodal or extranodal marginal zone lymphoma and lymphoplasmacytic lymphoma, Epstein-Barr virus (EBV) positive in 75% of cases. Submitted large BCLs formed a spectrum from diffuse large B-cell lymphoma (DLBCL) to CHL across immunodeficiency settings. Additional studies demonstrated overexpression of PD-L1 and molecular 9p24 alterations in the large BCL spectrum and across different immunodeficiency settings.
  
  
  CONCLUSIONS
  Small BCLs occur in all immunodeficiency settings, and EBV positivity is essential for their recognition as immunodeficiency related. Large BCLs include a spectrum from DLBCL to CHL across all immunodeficiency settings; immunohistochemical and molecular features are suggestive of shared pathogenetic mechanisms involving PD-L1 immune checkpoints.
authors.affiliation: From the VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Daphne
authors.initials: D
authors.lastname: de Jong
authors.affiliation: From the VU University Medical Center, Amsterdam, the Netherlands.|Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA.
authors.forename: Margaretha G M
authors.initials: MG
authors.lastname: Roemer
authors.affiliation: Queen Elizabeth Hospital, Kowloon, Hong Kong.
authors.forename: John K C
authors.initials: JK
authors.lastname: Chan
authors.affiliation: HMDS, St James's University Hospital, Leeds, United Kingdom.
authors.forename: John
authors.initials: J
authors.lastname: Goodlad
authors.affiliation: Stanford University School of Medicine, Stanford, CA.
authors.forename: Dita
authors.initials: D
authors.lastname: Gratzinger
authors.affiliation: Weill Medical College of Cornell University, New York.
authors.forename: Amy
authors.initials: A
authors.lastname: Chadburn
authors.affiliation: National Institutes of Health, Bethesda, MD.
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.affiliation: University of California Los Angeles Medical Center, Los Angeles.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Said
authors.affiliation: Stanford University School of Medicine, Stanford, CA.
authors.forename: Yasodha
authors.initials: Y
authors.lastname: Natkunam
mesh_terms: D004493:Education; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006689:Hodgkin Disease / Q000276:immunology / Q000473:pathology / Q000821:virology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000473:pathology / Q000821:virology; D016393:Lymphoma, B-Cell / Q000276:immunology / Q000473:pathology / Q000821:virology; D008297:Male
keywords: 9p24; Autoimmunity; Classical Hodgkin lymphoma; Iatrogenic immunodeficiency; Large B-cell lymphoma; Marginal zone lymphoma; PD-L1; Posttransplant lymphoproliferative disorder; Primary immunodeficiency; T-cell/histiocyte-rich B-cell lymphoma