Abstract:
:Familial hemophagocytic lymphohistiocytosis (FHL) is a rare and fatal disease of early childhood characterized by a non-malignant accumulation of activated T lymphocytes and histiocytes in the reticuloendothelial system. Moreover, immune system derangement, with prominent hypercytokinemia and low or absent cytotoxic T and natural killer (NK) cell activity, is a consistent feature of this autosomal recessive disorder. Recent work has demonstrated that the degree of spontaneous caspase activation in FHL lymphocytes is attenuated in vitro whereas Fas-mediated caspase activation and apoptosis induction remains unmitigated, and FHL can thus be distinguished from the related chronic disorder of immune regulation termed autoimmune lymphoproliferative syndrome or ALPS. However, subsequent studies have identified mutations in the gene encoding perforin, a cytotoxic granule constituent required for apoptotic killing of target cells, in a number of FHL patients. Hence, the underlying defect in FHL may be conceived of as a lack of apoptosis triggering within the immune system, rather than apoptosis resistance per se. These observations represent an important step in our understanding of the pathogenesis of FHL and also serve to emphasize the pivotal role of cellular (perforin-based) cytotoxicity in the regulation of immune homeostasis.
journal_name
Leuk Lymphomajournal_title
Leukemia & lymphomaauthors
Fadeel B,Orrenius S,Henter JIdoi
10.3109/10428190109097672keywords:
subject
Has Abstractpub_date
2001-06-01 00:00:00pages
13-20issue
1-2eissn
1042-8194issn
1029-2403journal_volume
42pub_type
杂志文章,评审abstract::The incidence and pattern of secondary neoplasms in patients with acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA)-containing regimens is not well described. We compared 160 patients with APL treated with ATRA plus idarubicin (n = 54) or ATRA plus arsenic trioxide (ATO) (n = 106) for the ...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428194.2014.953143
更新日期:2015-05-01 00:00:00
abstract::Bone marrow toxicity is a great challenge for physicians treating patients with non-Hodgkin's lymphoma (NHL) and prescribed chemotherapy. Granulocyte colony-stimulating factor (G-CSF) prevents myelotoxicity, but the optimal timing and scheduling of G-CSF administration has not been ascertained. We investigated leukocy...
journal_title:Leukemia & lymphoma
pub_type: 临床试验,杂志文章
doi:10.3109/10428199509054760
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abstract::Patients with CTCL are at increased risk for bacteremia which is a leading cause of morbidity and mortality. We assessed risk factors for and the impact of bacteremia on survival in a retrospective cohort of 188 CTCL patients at a single US academic institution treated between 1990 and 2018. With a median follow up of...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
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abstract::The combination of chromosomal translocations associated with bcl-2 rearrangement [t(14;18)] and c-myc rearrangement [t(8;14), t(8;22), or t(2;8)] has infrequently been detected in lymphoproliferative disorders. We have recently identified four cases of a B-cell malignancy exhibiting this dual translocation. In additi...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428199309148200
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
doi:10.1080/10428194.2018.1434886
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abstract::Intra-vascular lymphoma is usually reported as a rare and fatal disorder. We describe here the first case of an intra-vascular lymphoma revealed by a nephrotic syndrome for which a durable remission has been obtained by 8 cycles of bi-mensual CHOP and Rituximab therapy. In this report, 18 fluorodesoxyglucose tomoscint...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428190410001683822
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abstract::The expression of c-kit receptor (c-kit R; CD117) and CD34 was examined in acute myeloid leukemia (AML), acute lymphoid leukemia (ALL), chronic myeloid leukemia (CML) in blastic transformation (BT), and myelofibrosis (MF) in myeloid BT. In myeloid leukemia including AML, CML-myeloid BT and MF-myeloid BT, both c-kit R ...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
doi:10.3109/10428199509049769
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428194.2011.562573
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428199309147366
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
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abstract::Isolated central nervous system involvement in Richter's syndrome (RS) is extremely rare and only 6 such cases have been described, so far. We report a 60-year-old woman with B-cell chronic lymphocytic leukemia (B-CLL) heavily pretreated with cladribine based regimens and rituximab in whom RS in the brain was first ma...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428190310001638823
更新日期:2004-06-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
doi:10.3109/10428194.2010.530361
更新日期:2011-01-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,meta分析
doi:10.3109/10428194.2015.1065977
更新日期:2016-01-01 00:00:00
abstract::It is unknown whether rituximab increases the risk of second primary malignancies (SPMs) in patients with diffuse large cell B-cell lymphoma (DLBCL). We assessed SPMs in DLBCL patients diagnosed between 1996 and 2014 in comparison with the general Israeli population and dependent on rituximab treatment. Jewish patient...
journal_title:Leukemia & lymphoma
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abstract::Post-transplantation lymphoproliferative disease (PTLD) is an unique iatrogenic complication after bone marrow transplantation (BMT) and solid organ transplantation (SOTx). The pattern of EBV related lymphoma in Chinese is different from Caucasians. We surveyed the incidence, clinical and pathological spectrum of PTLD...
journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428190290033341
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428190701361844
更新日期:2007-07-01 00:00:00
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journal_title:Leukemia & lymphoma
pub_type: 临床试验,杂志文章,多中心研究
doi:10.3109/10428191003721342
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.3109/10428194.2013.789508
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
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doi:10.3109/10428194.2014.894189
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章
doi:10.1080/10428194.2019.1665666
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
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journal_title:Leukemia & lymphoma
pub_type: 杂志文章,评审
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journal_title:Leukemia & lymphoma
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