A diagnosis of autoimmune lymphoproliferative syndrome caused by FAS deficiency during adulthood is unusual. We analyzed 17 cases of autoimmune lymphoproliferative syndrome caused by FAS deficiency diagnosed during adulthood in French reference centers for hereditary immunodeficiencies and for immune cytopenias. Twelve of the 17 patients had developed their first symptoms during childhood. The ...

Tissues from patients thought to have Epstein-Barr virus (EBV)-induced lymphoproliferative diseases were probed for EBV genomes using 2 independent hybridization techniques. Tissues from six patients with the X-linked lymphoproliferative syndrome, all five renal allograft recipients with immunoblastic sarcoma, and eight patients with diverse types of immunodeficiency and lymphoproliferative dis...

AIMS To investigate the expression pattern of Epstein-Barr virus (EBV) latent genes at the single cell level in post-transplantation lymphoproliferative disorders and acquired immunodefiency syndrome (AIDS) related lymphomas, in relation to cellular morphology. METHODS Nine post-transplantation lymphoproliferative disorders and three AIDS related lymphomas were subjected to immunohistochemist...

epstein-barr virus (ebv) was discovered 50 years ago from an african burkitt lymphoma cell line. ebv-associated lymphoproliferative disorders (lpds) are life-threatening diseases, especially in children. in this article, we review ebv-associated lpds, especially in the area of primary immunodeficiency disease (pid). we searched pubmed for publications with key words including ebv infection, lym...

Splenic Lymphoma with villous lymphocytes (SLVL) is a B cell chronic lymphoproliferative disorder defined in the WHO classification as the leukemic form of splenic marginal zone lymphoma. Autoimmune diseases or second neoplasms in patients affected by B cell chronic lymphoproliferative disorders are well known conditions after fludarabine containing regimens (above all in the case of Chronic Ly...

Myasthenia gravis (MG), a neuromuscular junction autoimmune disease, sometimes complicates second malignancies; however, T-cell lymphoproliferative disorders have rarely been reported. A 55-year-old man, who received oral tacrolimus and prednisolone for MG for 16 years after thymectomy, presented with left abdominal pain, lymphadenopathy, and splenomegaly. A lymph node biopsy revealed periphera...