Abstract:
:Allogeneic hematopoietic stem cell transplantation (HSCT) is the only potential cure for most bone marrow (BM) failure syndromes and hemoglobinopathies. Over the past decade, umbilical cord blood (UCB) has been used more frequently as a stem cell source in patients who lack a suitable BM donor. Although graft failure remains a significant problem, UCB transplantation (UCBT) using the optimal conditioning regimen can be a salvage treatment for patients without a suitable BM donor and warrants evaluation in further prospective studies.
journal_name
Semin Hematoljournal_title
Seminars in hematologyauthors
MacMillan ML,Walters MC,Gluckman Edoi
10.1053/j.seminhematol.2009.10.004subject
Has Abstractpub_date
2010-01-01 00:00:00pages
37-45issue
1eissn
0037-1963issn
1532-8686pii
S0037-1963(09)00135-8journal_volume
47pub_type
杂志文章,评审abstract::Although often overlooked, the life of the patient with severe hemophilia is characterized by both intermittent and chronic pain. Bleeds into joints and muscles cause extensive pressure on sensory nerves and, following recurrent bleeds, joint destruction, and synovial reaction is accompanied by constant pains that are...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2005.11.024
更新日期:2006-01-01 00:00:00
abstract::There is consensus that human herpesvirus 8 (HHV-8) is a necessary, albeit not sufficient, causal agent of Kaposi's sarcoma (KS). In the past several years, there have been rapid advances in our understanding of HHV-8 infection. In diagnosis, antibody detection is more sensitive than nucleic acid-based techniques. Whi...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/shem.2003.50013
更新日期:2003-04-01 00:00:00
abstract::Recombinant factor VIIa (rFVIIa; NovoSeven(R), Novo Nordisk, Bagsvaerd, Denmark) has been used for many years in the successful management of bleeding episodes in patients with hemophilia and inhibitors. More recently, rFVIIa has also shown considerable success as a hemostatic agent in trauma and surgery patients with...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2003.11.019
更新日期:2004-01-01 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::Myelodysplastic syndrome (MDS) with deletion 5q (del(5q)) is a distinct clinical and pathological disease subset that is exquisitely sensitive to lenalidomide for the treatment of red blood cell transfusion-dependent anemia. Although lenalidomide has erythropoeitic promoting activity in MDS without del(5q) (non-del(5q...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2017.06.003
更新日期:2017-07-01 00:00:00
abstract::A multicenter clinical trial of the thrombin inhibitor argatroban (Novastan; Texas Biotechnology, Houston, TX; Smith-Kline Beecham Pharmaceuticals, Philadelphia, PA) was recently conducted in patients with heparin-induced thrombocytopenia (HIT) and HIT that had progressed to thrombosis (HITTS). In patients defined by ...
journal_title:Seminars in hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:
更新日期:1999-01-01 00:00:00
abstract::Imatinib (Gleevec) (formerly STI571) competitively targets the adenosine 5-triphosphate (ATP) binding site of the kinase domain of ABL and was recently approved for the treatment of chronic myeloid leukemia (CML). Point mutations occurring in the kinase domain of BCR-ABL have been identified as a cause of imatinib res...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/shem.2003.50046
更新日期:2003-04-01 00:00:00
abstract::Cyclic neutropenia is a rare hematologic disorder, characterized by repetitive episodes of fever, mouth ulcers, and infections attributable to recurrent severe neutropenia. Fluctuations in blood cells are due to oscillatory production of cells by the bone marrow. Recent genetic, molecular, and cellular studies have sh...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/shem.2002.31917
更新日期:2002-04-01 00:00:00
abstract::This report suggests modest changes in the criteria used for the diagnosis of ET and allows tentative recommendations concerning therapy. As outlined in Table I, we believe that absent stainable marrow iron does not necessarily indicate iron deficiency in these patients and that the serum ferritin and RBC mean corpusc...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1997-01-01 00:00:00
abstract::Thrombocytopenia is one of the most common reasons for inpatient hematology consultations. The main challenges in the management of hospitalized patients with thrombocytopenia are to identify the underlying cause and to recognize when urgent interventions are required. Examination of the blood film is essential in the...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2011.08.002
更新日期:2011-10-01 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::Our advanced knowledge of coagulation has led to the synthesis of novel procoagulant substances, such as recombinant activated factor VII (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark). Similarly, in-depth understanding of the interaction between anticoagulant proteins and their natural inhibitors has led to th...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2005.11.023
更新日期:2006-01-01 00:00:00
abstract::The rationale for modulation of multidrug resistance (MDR) by inhibitors of the multidrug transporter, P-glycoprotein (P-gp) includes the following: (1) P-gp is expressed by human cancers, either at diagnosis or after failure of chemotherapy; (2) P-gp expression at diagnosis has been associated with a poor prognosis i...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1997-10-01 00:00:00
abstract::Macrocytic anemia, defined as a mean cell volume (MCV) ≥100 fL in adults, has a narrow differential diagnosis that requires evaluation of the peripheral blood smear as well as additional laboratory testing taken in conjunction with clinical information that includes patient history and physical examination findings. T...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2015.06.001
更新日期:2015-10-01 00:00:00
abstract::The stem cell origination of the clonal process in chronic myeloproliferative disorders (CMPDs) is well established. In chronic myelogenous leukemia (CML), the primary genetic process has been characterized. However, current information regarding the mechanisms of phenotypic diversity among the CMPD and the downstream...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1999-01-01 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::Psoralens plus ultraviolet A (UVA) light inactivate viruses and bacteria as well as leukocytes. A system employing the synthetic psoralen compound amotosalen hydrochloride (S-59), in combination with UVA light, is being developed to decontaminate platelet concentrates and plasma in a blood-bank setting. S-59 is a hete...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(01)90118-0
更新日期:2001-10-01 00:00:00
abstract::In this review, we discuss disease-causing alterations of RUNT-related transcription factor 1 (RUNX1), a master regulator of hematopoietic differentiation. Familial platelet disorder with predisposition to myeloid leukemia (FPDMM) typically presents with (1) mild to moderate thrombocytopenia with normal-sized platelet...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2017.04.006
更新日期:2017-04-01 00:00:00
abstract::The present review will briefly summarize the interplay between coagulation and inflammation, highlighting possible effects of direct inhibition of factor Xa and thrombin beyond anticoagulation. Additionally, the rationale for the use of the new direct oral anticoagulants (DOACs) for indications such as cancer-associa...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2014.03.002
更新日期:2014-04-01 00:00:00
abstract::Virtually all human cells are endowed with the capacity to commit suicide using an evolutionarily conserved mechanism that involves activation of caspase-family cell death proteases. Caspase activation culminates in a cell death process known as "apoptosis." The activation of these intracellular proteases is carefully...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(00)90055-6
更新日期:2000-10-01 00:00:00
abstract::Mantle-cell lymphoma (MCL) is a lymphoproliferative disorder derived from a subset of naive pregerminal center cells characterized by a nodular or diffuse proliferation of atypical lymphoid cells with a monoclonal B-cell phenotype and coexpression of CD5. Two cytologic variants have been identified, typical and blasti...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1999-04-01 00:00:00
abstract::Anemia of chronic disease (ACD) or inflammation may be secondary to infections, autoimmune disorders, chronic renal failure, or malignancies. It is characterized by an immune activation with an increase in inflammatory cytokines and resultant increase in hepcidin levels. In addition, inappropriate erythropoietin level...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2013.06.006
更新日期:2013-07-01 00:00:00
abstract::TET2 is one of the most frequently mutated genes in myeloid neoplasms. TET2 loss-of-function perturbs myeloid differentiation and causes clonal expansion. Despite extensive knowledge regarding biochemical mechanisms underlying distorted myeloid differentiation, targeted therapies are lagging. Here we review known bioc...
journal_title:Seminars in hematology
pub_type: 杂志文章
doi:10.1053/j.seminhematol.2020.12.002
更新日期:2021-01-01 00:00:00
abstract::Chronic lymphocytic leukemia (CLL) is a clonal proliferation of mature-appearing, but maturationally immature B cells; T cells are rarely involved. Most data suggest that CLL cells are frozen at an early step in maturation; this can be overcome by several techniques such as in vitro incubation with tumor promotors. Al...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1987-10-01 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::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::Large scale sequencing projects over the past 2 decades have led to the identification of many common genomic alterations in hematolymphoid neoplasms, some of which with diagnostic, therapeutic, and prognostic implications [1-3]. Although these alterations can be tested individually with high sensitivity and specifici...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2018.05.006
更新日期:2019-01-01 00:00:00
abstract::In both normal and abnormal vascular biology, platelet-leukocyte-RBC-EC adhesive and functional interactions appear to play a significant role. The clinical hallmarks of the TTP/HUS syndromes, including prominent platelet aggregation and thrombi without prominent soluble coagulation system activation along with microa...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1997-04-01 00:00:00
abstract::Patients with acute myeloid leukemia (AML) who achieve complete remission after induction therapy require post remission therapy (PRT) in order to remain disease free. The role of autologous hematopoietic cell transplantation (autoHCT) in the PRT setting is controversial and is largely based on older trials that were ...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2019.01.001
更新日期:2019-04-01 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