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...

Longitudinally extensive transverse myelitis (LETM) is an unusual manifestation of systemic malignancy. It has been mainly reported with lung cancers and lymphoproliferative malignancy. LETM in systemic malignancy can be caused by either intramedullary metastases or paraneoplastic syndrome. We report an unusual case of small-cell carcinoma lung, who presented with LETM without having any cardin...

Autoimmune lymphoproliferative syndrome (ALPS) is a rare childhood disorder characterized by chronic non-malignant lymphoproliferation and autoimmunity. Patients with ALPS frequently exhibit episodic and intermittent, severe autoimmune- induced hemolytic anemia, thrombocytopenia or combined cytopenias. The co-occurrence of immune-mediated cytopenias, autoimmune thrombocytopenia and autoimmune h...

INTRODUCTION Systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is an extremely rare disorder and classically arises following primary acute or chronic active Epstein-Barr virus infection. It is characterized by clonal proliferation of Epstein-Barr virus-infected T-cells with an activated cytotoxic phenotype. This disease has a rapid clinical course and is more fr...

To the Editor: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disease due to cytokine overproduction from excessively activated lymphocytes and macrophages [1]. It primarily affects children and has a varied genetic background [1]. Rigaud et al. [2] reported for the first time that mutations in the X-linked inhibitor of apoptosis (XIAP) gene affect the risk for...

The term disappearing HDL syndrome refers to development of severe high density lipoprotein cholesterol (HDL-C) deficiency in noncritically ill patients with previously normal HDL-C and triglyceride levels. Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of the immune system due to an inability to regulate lymphocyte homeostasis resulting in lymphadenopathy and hepatosplenomegaly. ...

background: defects in b cell class switch recombination (csr) are a heterogeneous and yet very uncommon group of disorders which all have a genetic basis uniformly leading to hyper igm (higm) syndrome. due to the rare frequency of these conditions, a very small number of case series have been conducted on the affected patients. objective: to shed some light on the morbidity and mortality regar...

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome primarily affects males and is caused by problems with the immune system. The immune system normally protects the body from foreign invaders, such as bacteria and viruses, by recognizing and attacking these invaders and clearing them from the body. However, the immune system can malfunction and attack the body's own...

post-transplant lymphoproliferative disorder is a lymphocyte proliferating disease, usually of b cell origin, and rarely of t cell. involvement of liver itself in liver transplant recipients as the primary organ is not common. herein we report our experience in two patients who primarily presented in the allografted liver, both of whom were promptly diagnosed after liver biopsy and treated succ...