نتایج جستجو برای: linked lymphoproliferative syndrome

تعداد نتایج: 843487  

Journal: :Pediatric Hematology/Oncology and Immunopathology 2020

Journal: :Cancer research 1981
A K Saemundsen D T Purtilo K Sakamoto J L Sullivan A C Synnerholm D Hanto R Simmons M Anvret R Collins G Klein

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...

Journal: :Iranian journal of allergy, asthma, and immunology 2004
Toshio Miyawaki

Epstein-Barr virus (EBV) is a ubiquitous human gamma-herpesvirus that infects about 95% of the adult population. The majority of primary infections occurs in early childhood and is generally subclinical; it can cause infectious mononucleosis (IM), which is usually a self-limiting lymphoproliferative disorder. However, infection of EBV occasionally results in severe, often lethal diseases, which...

Journal: :Cancer research 1981
D T Purtilo K Sakamoto A K Saemundsen J L Sullivan A C Synnerholm M Anvret J Pritchard C Sloper C Sieff J Pincott L Pachman K Rich F Cruzi J A Cornet R Collins N Barnes J Knight B Sandstedt G Klein

Multiple methods, pedigree analysis, clinical evaluation, and Epstein-Barr virus (EBV)-specific serology, EBV DNA hybridization of tissues to probe for viral genome, staining of touch imprints for EBV nuclear-associated antigen, establishment of spontaneous infected B-cell lines from peripheral blood or tissues, examination of peripheral blood smears, and hematopathology studies, were used to s...

Journal: :The Journal of Experimental Medicine 2000
Silvia Parolini Cristina Bottino Michela Falco Raffaella Augugliaro Silvia Giliani Roberta Franceschini Hans D. Ochs Hermann Wolf Jean-Yves Bonnefoy Roberto Biassoni Lorenzo Moretta Luigi D. Notarangelo Alessandro Moretta

2B4 is a surface molecule involved in activation of the natural killer (NK) cell-mediated cytotoxicity. It binds a protein termed Src homology 2 domain-containing protein (SH2D1A) or signaling lymphocyte activation molecule (SLAM)-associated protein (SAP), which in turn has been proposed to function as a regulator of the 2B4-associated signal transduction pathway. In this study, we analyzed pat...

2010
S. Nicole Chadha David Amrol

X-linked lymphoproliferative disease, also known as Duncan's syndrome, is a rare genetic disorder that causes exaggerated immune responses to Epstein-Barr virus (EBV) infection and often leads to death. Patient presentation varies but can include signs and symptoms typical of EBV, pancytopenia, and fulminant hepatitis.

Journal: :Atlas of Genetics and Cytogenetics in Oncology and Haematology 2012

Journal: :Allergology international : official journal of the Japanese Society of Allergology 2018
Hirokazu Kanegane Akihiro Hoshino Tsubasa Okano Takahiro Yasumi Taizo Wada Hidetoshi Takada Satoshi Okada Motoi Yamashita Tzu-Wen Yeh Ryuta Nishikomori Masatoshi Takagi Kohsuke Imai Hans D Ochs Tomohiro Morio

Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited diseases of the immune system. The definite diagnosis of PID is ascertained by genetic analysis; however, this takes time and is costly. Flow cytometry provides a rapid and highly sensitive tool for diagnosis of PIDs. Flow cytometry can evaluate specific cell populations and subpopulations, cell surface, intracellular and ...

Journal: :iranian journal of blood and cancer 0
h abolghasemi tehranسازمان اصلی تایید شده: دانشگاه علوم پزشکی بقیه الله (baqiyatallah university of medical sciences) e shahverdi tehranسازمان اصلی تایید شده: دانشگاه علوم پزشکی بقیه الله (baqiyatallah university of medical sciences) f dolatimehr tehranسازمان اصلی تایید شده: دانشگاه علوم پزشکی بقیه الله (baqiyatallah university of medical sciences) r mehdi oghli tehranسازمان اصلی تایید شده: دانشگاه علوم پزشکی بقیه الله (baqiyatallah university of medical sciences)

autoimmune lymphoproliferative syndrome (alps) is a rare inherited disorder of apoptosis. it usually presents with chronic lymphadenopathy, splenomegaly, and symptomatic cytopenia in a child. herein, we report a 14-year-old boy with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis who was treated before alps was diagnosed for the patient. this case should alert pediatricians to consi...

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