CD3-negative lymphoproliferative disease of granular lymphocytes in a girl with an unusual pattern of anti-Epstein-Barr virus antibodies.
We describe an 11-year-old girl who initially had mild hepatosplenomegaly and then presented with abnormal expansion of CD3-negative granular lymphocytes in peripheral blood and Epstein-Barr virus (EBV) genome in the DNA obtained from the peripheral blood mononuclear cells (PBMNC). After approximately 3 years, she developed oedema, ascites, marked hepatosplenomegaly, and pancytopenia, and showed both a profile of anti-EBV antibodies of reactivated infection and a high titre of anti-cytomegalovirus antibody. Although she was treated with antibiotics, ganciclovir, and prednisolone, she died of hepatic failure.


CONCLUSIONS
Careful clinical observation, periodic examination of anti-EBV antibodies, and the analysis of EBV genome from PBMNC are needed in young patients with CD3-negative lymphoproliferative disease of granular lymphocytes.
Department of Paediatrics, Nippon Medical School, Tokyo, Japan.
Y
Y
Fukunaga
T
T
Asano
J
J
Takehana
K
K
Ambo
K
K
Matsuoka
M
M
Yamamoto
D017252:CD3 Complex / Q000276:immunology*; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006098:Granulocytes / Q000276:immunology*; D006529:Hepatomegaly / Q000209:etiology; D004854:Herpesvirus 4, Human / Q000276:immunology*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000276:immunology*; D013163:Splenomegaly / Q000209:etiology
D017252:CD3 Complex