Journal of Clinical Oncology, Vol 8, 378-384, Copyright © 1990 by American Society of Clinical Oncology
Epstein-Barr virus polymorphic B-cell lymphoma associated with leukemia and with congenital immunodeficiencies
JH Joncas, P Russo, P Brochu, P Simard, J Brisebois, J Dube, D Marton, JM Leclerc, H Hume and GE Rivard
Department of Microbiology, Hopital Sainte-Justine, Montreal, Quebec, Canada.
Polymorphic B-cell lymphoma seen in four patients with congenital
immunodeficiencies and in two patients with leukemia receiving chemotherapy
was associated with the Epstein-Barr virus (EBV). The tumors had
characteristic histologic features: they were polymorphic consisting of a
mixture of lymphoblasts and differentiated cells including plasma cells,
and areas of hemorrhagic necrosis were prominent. The tumors were either
polyclonal, monoclonal, or multiclonal. Patients with congenital
immunodeficiencies who developed these tumors died despite radiotherapy,
corticosteroids plus acyclovir, or a combination of intravenous (IV)
immunoglobulins and alpha 2 interferon. Patients with leukemia recovered
when immunosuppressive drugs were discontinued and leukemia has not
recurred over a period of 2 and 4 years, respectively, in the two patients.
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