kennedy disease is a rare x-linked neurodegenerative disorder that affects patients in 30-50 years of age. it is caused by cag-repeat in androgen receptor gen. there is no known effective treatment for kennedy disease. we report a 60-year-old man who had fasciculations and proximal and distal muscle weakness. physical examination showed involvement of the bulbar musculature accompanied by tongu...

x-linked agammaglobulinemia (xla) is an immunodeficiency caused by mutations in the bruton tyrosine kinase (btk) gene. in order to identify the mutations in btk gene in iranian patients with antibody deficiency, 13 male patients with an xla phenotype from 11 unrelated families were enrolled as the subjects of investigation for btk mutation analysis using pcr-sscp followed by sequencing. five di...

IL-6, a multifunctional cytokine produced by monocytes, fibroblasts, and endothelial cells, promotes the growth of EBV-immortalized B cells in vitro and renders these cells tumorigenic in athymic mice. In the present study, serum/plasma IL-6 bioactivity was found to be abnormally elevated, albeit transiently, in 17 of 18 solid organ transplant recipients with posttransplant lymphoproliferative ...

Kaposi's sarcoma-associated herpesvirus (KSHV) is tightly linked to at least two lymphoproliferative disorders, primary effusion lymphoma (PEL) and multicentric Castleman's disease (MCD). However, the development of KSHV-mediated lymphoproliferative disease is not fully understood. Here, we generated two recombinant KSHV viruses deleted for the first RBP-Jκ binding site (RTA(1st)) and all three...

Epstein-Barr virus (EBV) is a ubiquitous DNA virus that normally infects silently, establishing lifelong latency. Substantial empirical observations support the view that immunodeficiency is permissive in EBV-induced lymphoproliferative diseases (LPD). Primary immune deficient patients such as those with X-linked lymphoproliferative disease and individuals with acquired immune deficiency second...

Since its discovery in 1994, Kaposi's sarcoma-associated herpesvirus (KSHV) has been associated with lymphoproliferative disorders, particularly in patients infected with human immunodeficiency virus (HIV). The disorders most strongly linked to KSHV are multicentric Castleman's Disease (MCD), primary effusion lymphoma, and diffuse large B-cell lymphomas. We report an unusual case of KSHV-associ...

Post-transplant lymphoproliferative disorder (PTLD) is a rare but frequently fatal complication of iatrogenic immunosuppression. PTLD encompasses a spectrum of B cell lymphoproliferations ranging from reactive plasmacytic hyperplasia to monomorphic B cell lymphoma. The tumours are almost always associated with Epstein-Barr Virus (EBV), and similar lymphoproliferative disorders also occur in oth...

The practical importance of finding a morphologically benign lymphoid aggregate in the bone marrow of patients without known lymphoproliferative disease was assessed in 786 consecutive patients who had had 951 iliac crest bone marrow biopsies performed. Of these, 430 patients known to have lymphoproliferative disease at the time of biopsy were excluded. Of 356 patients, 86 (aggregate group) had...

Post-transplant lymphoproliferative disorder (PTLD) is a rare but frequently fatal complication of iatrogenic immunosuppression. PTLD encompasses a spectrum of B cell lymphoproliferations ranging from reactive plasmacytic hyperplasia to monomorphic B cell lymphoma. The tumours are almost always associated with Epstein-Barr Virus (EBV), and similar lymphoproliferative disorders also occur in oth...