Age-related Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorders: comparison with EBV-positive classic Hodgkin lymphoma in elderly patients.
Age-related Epstein-Barr virus-associated B-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV-associated large B-cell lymphoma in elderly without predisposing immunodeficiency. In nearly one- third of cases, aEBVLPD occurs as a polymorphous subtype with reactive cell-rich components, bearing a morphologic similarity to classic Hodgkin lymphoma (cHL). The aim of this study was to clarify clinicopathologic differences between the polymorphic subtype of aEBVLPD (n = 34) and EBV(+) cHL (n = 108) in patients aged 50 years or older. Results showed that aEBVLPD was more closely associated with aggressive clinical parameters than cHL, with a higher age at onset (71 vs 63 years); lower male predominance (male-female ratio, 1.4 vs 3.3); and a higher rate of involvement of the skin (18% vs 2%), gastrointestinal tract (15% vs 4%), and lung (12% vs 2%). aEBVLPD was histopathologically characterized by a higher ratio of geographic necrosis, greater increase (> 30%) in cytotoxic T cells among background lymphocytes, higher positivity for CD20 and EBNA2, and absence of CD15 expression. As predicted by the clinical profile, aEBVLPD had a significantly poorer prognosis than EBV(+) cHL (P < .001). The polymorphous subtype of aEBVLPD constitutes an aggressive group with an immune response distinct from EBV(+) cHL, and requires the development of innovative therapeutic strategies.
Asano
Naoko
N
Department of Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan. naasano@shinshu-u.ac.jp
Yamamoto
Kazuhito
K
Tamaru
Jun-Ichi
J
Oyama
Takashi
T
Ishida
Fumihiro
F
Ohshima
Koichi
K
Yoshino
Tadashi
T
Nakamura
Naoya
N
Mori
Shigeo
S
Yoshie
Osamu
O
Shimoyama
Yoshie
Y
Morishima
Yasuo
Y
Kinoshita
Tomohiro
T
Nakamura
Shigeo
S
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000276:immunology*; D018951:Antigens, CD20 / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016022:Case-Control Studies; D003043:Cocarcinogenesis; D020031:Epstein-Barr Virus Infections / Q000453:epidemiology* / Q000276:immunology; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006689:Hodgkin Disease / Q000145:classification / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology / Q000821:virology; D006801:Humans; D007157:Immunologic Surveillance; D016130:Immunophenotyping; D053208:Kaplan-Meier Estimate; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D009928:Organ Specificity; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012737:Sex Factors; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology
D018951:Antigens, CD20