Abstract：
:The activation, function, and development of peripheral T lymphocytes are dependent on the ability to signal properly through the surface T-cell antigen receptor (TCR)-CD3 complex. Transmission of such signals requires the activation of specific cytoplasmic protein tyrosine kinases (PTK) associated with the TCR. Recently, mutations in one such PTK, called ZAP-70, have been shown to be responsible for a rare, autosomal recessive form of severe combined immunodeficiency syndrome (SCID) in humans. This distinctive SCID syndrome is characterized by the selective absence of peripheral CD8+ T cells and by the presence of circulating CD4+ T cells that do not respond to TCR-mediated stimuli in vitro. T-cell immunodeficiency syndromes that arise as a consequence of inherited mutations in either the CD3epsilon or CD3gamma subunit proteins have also been described in rare patients. Absence of these TCR components results in severely decreased expression of the surface TCR-CD3 complex and defective signal transduction through the TCR. In this report, the clinical, laboratory, and molecular findings of these immunodeficiency disorders are described, insights are provided by these inherited defects into the pathways of TCR signal transduction, and T-cell development is discussed.
journal_name
Semin Hematoljournal_title
Seminars in hematologyauthors
Elder MEsubject
Has Abstractpub_date
1998-10-01 00:00:00pages
310-20issue
4eissn
0037-1963issn
1532-8686journal_volume
35pub_type
杂志文章,评审abstract：:It is now well accepted that a subgroup of patients with myelodysplastic syndromes (MDS) can recover from pancytopenia following immunosuppressive treatment (IST). For many years immunosuppression with antilymphocyte antibodies has been a standard treatment approach for patients with severe aplastic anemia (SAA). The ...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2012.07.004
更新日期：2012-10-01 00:00:00
abstract：:Despite the development of more effective therapies for multiple myeloma (MM) over the past decade, nearly all patients will eventually experience disease relapse and require further therapy. Designing the next generation of therapies for relapsed and refractory disease will depend on understanding the complex molecul...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1053/j.seminhematol.2012.05.005
更新日期：2012-07-01 00:00:00
abstract：:Though peripheral T-cell lymphoma (PTCL) is an area of significant unmet therapeutic need, a number of new treatment options are available for patients, especially those with relapsed or refractory disease. A plethora of drugs are now in development for PTCL, but drugs that truly target novel disease biology are notic...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2010.01.014
更新日期：2010-04-01 00:00:00
abstract：:Antibody-mediated rejection (AMR) is a major risk factor for graft loss following kidney transplantation. Traditional anti-humoral therapies provide suboptimal therapy and they do not deplete plasma cells, which are the source of antibody production. Proteasome inhibitors (PI) have been shown to deplete both transform...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2012.04.008
更新日期：2012-07-01 00:00:00
abstract：:Infectious diseases are the leading causes of death in patients with multiple myeloma (MM). Major changes have occurred in the spectrum of infections paralleling the changes in the treatment of MM. Bacteria (particularly gram-negative organisms) are the most frequent etiologic agents, and invasive fungal infections ca...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2009.03.006
更新日期：2009-07-01 00:00:00
abstract：:The use of combination chemotherapy has been successful in treating advanced or widespread Hodgkin's disease (HD). For patients with relapsed disease, the long-term prognosis is much poorer, despite the good rate of reinduction into a second or further remission using conventional treatments. The majority of such pati...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1999-07-01 00:00:00
abstract：:High-dose chemoradiotherapy with allogeneic bone marrow or peripheral blood stem cell transplantation (SCT) is a potentially curative treatment for advanced or poor-prognosis hematological malignancies. This procedure was initially considered as a means to deliver supralethal doses of chemotherapy and radiation for th...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/shem.2002.30909
更新日期：2002-01-01 00:00:00
abstract：:Smoldering myeloma (SMM) is a precursor state of multiple myeloma. It is defined by an M-protein concentration ≥3 g/dL and/or ≥10% clonal bone marrow plasma cells, in the absence of end-organ damage. Based on clinical observations, the natural history of SMM varies greatly, from stable, monoclonal gammopathy of undete...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2010.11.009
更新日期：2011-01-01 00:00:00
abstract：:The availability of antibodies reactive with antigens expressed only by hematopoietic cells has provided clinical investigators with new tools for use in developing therapies for acute myeloid leukemia (AML). Studies performed to date have investigated the use of such antibodies in an unmodified state, combined with p...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1999-10-01 00:00:00
abstract：:Anemia is a common, multifactorial condition among older adults. The World Health Organization (WHO) definition of anemia (hemoglobin concentration <12 g/dL in women and <13 g/dL in men) is most often used in epidemiologic studies of older adults. More than 10% of community-dwelling adults age 65 years and older has W...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2008.06.006
更新日期：2008-10-01 00:00:00
abstract：:Indolent non-Hodgkin lymphoma (NHL) comprises a group of incurable, generally slow-growing lymphomas highly responsive to initial therapy, with a relapsing and progressive course. Rituximab, an anti-CD20 antibody, has had a large impact on the treatment of indolent NHL. Its effectiveness as a single agent and in conju...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2010.01.003
更新日期：2010-04-01 00:00:00
abstract：:Recent advances in haploidentical stem cell transplantation have enabled the use of human leukocyte antigen-half matched related donors for allogeneic stem cell transplantation and helped overcome one of the most important limitation in transplantation, which is donor availability, especially for the non-Caucasian pop...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2018.08.003
更新日期：2019-07-01 00:00:00
abstract：:Over the last two decades quality of life (QoL) and the social challenges of allogeneic hematopoietic stem cell transplant (allo-HSCT) survivors have been emerging as subjects of extensive research and are now considered as very important aspects in the pretransplant evaluation and management of allo-HSCT recipients. ...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2011.10.004
更新日期：2012-01-01 00:00:00
abstract：:Management of patients with beta-thalassemia is based on adequate, safe blood transfusions (free of transfusion-transmitted diseases) and prevention of iron overload. Iron overload causes multiple endocrinopathies, contributes to osteoporosis, and is the cause of cardiac disease. Cardiac disease, secondary to iron dam...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(01)90029-0
更新日期：2001-10-01 00:00:00
abstract：:Seventy-six patients with previously untreated advanced Hodgkin's disease have been treated with the MOPP/ABV (mechlorethamine, vincristine, procarbazine, prednisone/doxorubicin, bleomycin, vincristine) hybrid program. Ten patients also received involved field radiation to the mediastinum for residual nodal disease af...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：
更新日期：1987-04-01 00:00:00
abstract：:From work particularly In the last two decades of the century, the cell populations forming blood cells can now be purified and cultured clonally so that blood cell formation can be analyzed in vitro. A large number of specific regulators of this process have been identified and mass-produced in recombinant form. Thre...
journal_title：Seminars in hematology
pub_type： 历史文章,杂志文章,评审
doi：
更新日期：1999-10-01 00:00:00
abstract：:Bone marrow transplantation (BMT) from a human leukocyte antigen (HLA)-matched sibling is the treatment of choice in children and young adults with severe aplastic anemia (SAA). However, because only 30% of patients have a suitable donor, more aggressive nontransplant immunosuppressive regimens have been used, with re...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(00)90029-5
更新日期：2000-01-01 00:00:00
abstract：:Immune thrombocytopenia (ITP) is a bleeding disorder characterized by low platelet counts due to decreased platelet production as well as increased platelet destruction by autoimmune mechanisms. A shift toward Th1 and possibly Th17 cells together with impaired regulatory compartment, including T-regulatory (Tregs) and...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2013.03.011
更新日期：2013-01-01 00:00:00
abstract：:Acute myeloid leukemia (AML) is a heterogeneous disease characterized by multiple genetic and epigenetic alterations. The major causes of treatment failure remain disease relapse and treatment toxicity. However, major advances in biological determinants of disease relapse, development of targeted molecules, improvemen...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2015.03.001
更新日期：2015-07-01 00:00:00
abstract：:A number of human disorders, dubbed ribosomopathies, are linked to impaired ribosome biogenesis or function. These include but are not limited to Diamond Blackfan anemia (DBA), Shwachman Diamond syndrome (SDS), and the 5q- myelodysplastic syndrome (MDS). This review focuses on the latter two non-DBA disorders of ribos...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2011.01.002
更新日期：2011-04-01 00:00:00
abstract：:Current therapeutic strategies for autoimmune diseases primarily rely on immunosuppression, but global immune suppression results in an increased risk for severe infection and malignancy. In contrast, immuomodulation is another therapeutic approach employing intrinsic or environmental regulators that exert modulatory ...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1053/j.seminhematol.2016.04.009
更新日期：2016-04-01 00:00:00
abstract：:Anemia is frequent and significantly adds to the morbidity of cancer patients, and has been associated with decreased quality of life (QOL). Three open-label community-based studies of epoetin alfa in cancer-related anemia (two using three-times-weekly dosing and one using once-weekly dosing) in more than 7,000 patien...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(00)90062-3
更新日期：2000-10-01 00:00:00
abstract：:Myelodysplastic syndromes (MDS) are clonal disorders characterized by ineffective hematopoiesis and subsequent frequent development of acute myeloid leukemia (AML). In children and adolescents, MDS are uncommon disorders, accounting for less than 5% of hematopoietic malignancy, with great heterogeneity in presentation...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1053/j.seminhematol.2007.10.006
更新日期：2008-01-01 00:00:00
abstract：:Analysis of data from 806 patients with newly diagnosed adult acute myelocytic leukemia (AML) (not including acute promyelocytic leukemia [APL] patients) treated at M.D. Anderson Cancer Center from 1995 through 1999 indicated that among patients entering a particular week of induction therapy, mortality rates were 6%,...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1016/s0037-1963(01)90042-3
更新日期：2001-10-01 00:00:00
abstract：:Both thalidomide and intermittent high-dose dexamethasone are agents with established activity against multiple myeloma. We summarized our experience with thalidomide alone, and then in combination with dexamethasone, for groups of patients with myeloma resistant or relapsing despite standard treatments. Criteria of r...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2003.09.006
更新日期：2003-10-01 00:00:00
abstract：:By definition, myeloproliferative disorders (MPDs) are caused by an acquired somatic mutation of a hematopoietic progenitor/stem cell and have sporadic occurrence. However, well-documented families exist with first-degree relatives acquiring one or several MPDs. It is reasonable to assume that the germ-line mutation(s...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2005.08.002
更新日期：2005-10-01 00:00:00
abstract：:Thrombosis is a common and potentially serious complication of immune-mediated heparin-induced thrombocytopenia (HIT). Discontinuation of heparin is a simple and important maneuver in patients with suspected HIT. Unfortunately, thrombosis often occurs even in those patients in whom heparin was discontinued because of ...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1998-10-01 00:00:00
abstract：:5-Aza-2'-deoxycytidine (decitabine; Dacogen, MGI Pharma, Inc, Bloomington, MN) is a cytidine analog that inhibits DNA methyltransferases resulting in loss of DNA methylation with subsequent gene re-expression. This compound was first synthesized over 40 years ago and since that time much information has been learned r...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2005.05.006
更新日期：2005-07-01 00:00:00
abstract：:Imatinib mesylate (Gleevec, Novartis Pharmaceuticals Corp, East Hanover, NJ; Glivec, Novartis Pharma AG, Basel, Switzerland), a signal transduction inhibitor with preferential effects against the tyrosine kinase activity of the protein product of the ABL proto-oncogene, induced hematologic responses in >or=90% of pati...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/shem.2002.36927
更新日期：2002-10-01 00:00:00
abstract：:The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry enrolls all consecutive patients for whom plasma exchange treatment is requested for clinically diagnosed TTP-HUS within a defined geographic region. During 14.5 years, from January 1, 1989 until June 30, 2003, 301 patients h...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1053/j.seminhematol.2003.10.001
更新日期：2004-01-01 00:00:00
