Senile EBV-associated B-cell lymphoproliferative disorder of indolent clinical phenotype with recurrence as aggressive lymphoma.
Senile EBV-associated B-cell lymphoproliferative disorder (LPD) was proposed as a new disease entity in 2003. This condition has a high incidence in elderly people without underlying immunodeficiencies, and is characterized by EBV-positive B-cell proliferation with a polymorphic composition. Histologically, the disease has two subtypes. The polymorphic LPD (PLPD) subtype has a preferable prognosis, whereas the large cell lymphoma (LCL) subtype involves aggressive disease progression. Reported herein is a case of senile EBV-BLPD with indolent clinical features and PLPD subtype in the initial phase that recurred as an aggressive lymphoma 3 years after the initial diagnosis. In the recurrent phase, Southern blotting confirmed monoclonal proliferation of large lymphoid B-cells. In both the initial and recurrent phases, polymerase chain reaction (PCR) yielded a single discrete band of a similar size due to an immunoglobulin heavy-chain gene rearrangement, indicating that the large lymphoid B-cells retained identical monoclonality throughout the histological progression and over the whole clinical course. These results suggest that the PLPD subtype is a histological finding in early phase senile EBV-BLPD and that the LCL subtype reflects the progressive phase of the disease.
Shiozawa
Eisuke
E
Second Department of Pathology, Showa Uinversity School of Medicine, Tokyo, Japan. eshiozawa@aol.com
Saito
Bungo
B
Yamochi-Onizuka
Toshiko
T
Makino
Reiko
R
Takimoto
Masafumi
M
Nakamaki
Tsuyoshi
T
Tomoyasu
Shigeru
S
Ota
Hidekazu
H
D000368:Aged; D001402:B-Lymphocytes / Q000473:pathology*; D002999:Clone Cells; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain / Q000235:genetics; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007124:Immunoenzyme Techniques; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D017403:In Situ Hybridization; D008206:Lymphatic Diseases / Q000473:pathology / Q000821:virology; D016393:Lymphoma, B-Cell / Q000473:pathology / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000378:metabolism / Q000473:pathology / Q000821:virology*; D008297:Male; D016133:Polymerase Chain Reaction; D012367:RNA, Viral / Q000032:analysis; D012008:Recurrence