نتایج جستجو برای: autoimmune lymphoproliferative synd

تعداد نتایج: 67222  

Journal: :Blood 2014
Elena Boggio Nausicaa Clemente Anna Mondino Giuseppe Cappellano Elisabetta Orilieri Casimiro L Gigliotti Erika Toth Ugo Ramenghi Umberto Dianzani Annalisa Chiocchetti

In autoimmune/lymphoproliferative syndrome (ALPS), defective Fas death receptor function causes lymphadenomegaly/splenomegaly, the expansion of T-cell receptor αβ(+) CD4/CD8 double-negative T cells, and frequent development of hematologic autoimmunity. Dianzani autoimmune lymphoproliferative disease (DALD) has a similar phenotype but lacks the expansion of double-negative T cells. This work sho...

2006
Samin Alavi Mohammad Taghi Arzanian Zahra Chavoshzadeh Maryam Esteghamati

Autoimmune lymphoproliferative syndrome is a disorder of lymphoid system regulation characterized by chronic splenomegaly, lymphadenopathy and autoimmune phenomena especially immune-mediated cytopenias. The hallmark of the disease is the presence in peripheral blood and lymphoid tissue of increased numbers of a normally rare T lymphocyte subset, usually referred to as “double-negative” T cells....

Journal: :international journal of pediatrics 0
amitabh singh senior research associate, department of pediatrics, aiims, new delhi-110029, india. anirban mandal senior resident, department of pediatrics, aiims, new delhi-110029, india. rachna seth additional professor, department of pediatrics, aiims, new delhi-110029, india.

autoimmune lymphoproliferative syndrome (alps) is a rare, inherited disorder of immune dysregulation secondary to defective lymphocyte apoptosis. this leads to uninhibited proliferation of lymphoid tissue manifesting with lymphadenopathy, hepatosplenomegaly, autoimmune cytopenia, and an increased risk of lymphoid malignancy. we report a 2 year old boy with fever, generalized lymphadenopathy, he...

Journal: :Journal of Allergy and Clinical Immunology 2021

Journal: :Blood 2011
Julie E Niemela Lianghao Lu Thomas A Fleisher Joie Davis Iusta Caminha Marc Natter Laurel A Beer Kennichi C Dowdell Stefania Pittaluga Mark Raffeld V Koneti Rao João B Oliveira

Somatic gain-of-function mutations in members of the RAS subfamily of small guanosine triphosphatases are found in > 30% of all human cancers. We recently described a syndrome of chronic nonmalignant lymphadenopathy, splenomegaly, and autoimmunity associated with a mutation in NRAS affecting hematopoietic cells, and initially we classified the disease as a variant of the autoimmune lymphoprolif...

Journal: :Current opinion in rheumatology 2003
M Ramos-Casals O Trejo M García-Carrasco J Font

PURPOSE OF REVIEW Chronic hepatitis C virus infection often has autoimmune clinical and analytic features. This review analyzes recent data on the close association of chronic hepatitis C virus infection with autoimmune and lymphoproliferative processes. RECENT FINDINGS Hepatitis C virus infection has been associated with both organ-specific (thyroiditis, diabetes) and systemic autoimmune dis...

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