Polymorphous lymphoproliferative disorder: a clinicopathological analysis.
Lymphoproliferative disorder (LPD) with polymorphous composition of proliferation (polymorphous LPD), containing large lymphoid cells together with small lymphocytes, plasma cells, macrophages, and/or eosinophils, is found in individuals with immunodeficiency conditions. Clinicopathological findings in 19 cases of polymorphous LPD registered with the Osaka Lymphoma Study Group, Osaka, Japan, were analyzed; they represented 0.4% of the registered cases. In six cases, there was a history of rheumatoid arthritis; five of them had received immunosuppressive agents. There were no acquired immunodeficiency syndrome cases or organ transplant recipients. Southern blotting and/or polymerase chain reaction (PCR)-based clonality analysis revealed monoclonal B cell and T cell proliferation in eight and six cases (B- and T-LPD), respectively, and polyclonality in one. In B-LPD, there was polymorphous proliferation, containing large B-lymphoid cells, while medium-to-large T lymphoid cells with occasional eosinophilic infiltration were seen in T-LPD. Epstein-Barr virus (EBV) was detected in three of eight B-LPD, four of six T-LPD, and one of one polyclonal LPD. The prognosis was not favorable; the 3-year overall survival rate was 49.7 +/- 17.3%. Thus, polymorphous LPD is relatively rare in Japan and is a heterogeneous disease with monoclonal proliferation of B or T cells; additionally, it is occasionally EBV-associated, and behaves as an aggressive lymphoma.
Nakamichi
Naoto
N
Department of Pathology (C3), Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan.
Wada
Naoki
N
Kohara
Masaharu
M
Fukuhara
Shirou
S
Sugiyama
Haruo
H
Ogawa
Hiroyasu
H
Hino
Masayuki
M
Kanamaru
Akihisa
A
Kanakura
Yuzuru
Y
Morii
Eiichi
E
Aozasa
Katsuyuki
K
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001402:B-Lymphocytes / Q000473:pathology; D002999:Clone Cells / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000473:pathology; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000473:pathology*; D007564:Japan / Q000453:epidemiology; D008223:Lymphoma / Q000453:epidemiology / Q000473:pathology* / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000453:epidemiology / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013601:T-Lymphocytes / Q000473:pathology