Abstract：
:Comprehensive study of the major chromosomal/molecular abnormalities in children and adults with acute lymphoblastic leukemia (ALL) has demonstrated prognostic utility for many of these anomalies, to the extent that cytogenetic and molecular genetic evaluations are now required for optimal clinical management of newly diagnosed cases. For example, the t(12;21)/TEL-AML1 (ETV6-CBFA2) or hyperdiploid karyotypes each identifies subgroups of children who can be cured with well-tolerated chemotherapy based primarily on drugs with few long-term toxicities, such as L-asparaginase and antimetabolites. By contrast, the t(1;19)/E2A-PBX1 identifies a subtype of ALL that responds much better to more intensive regimens that rely on genotoxic drugs. At the extreme end of the risk spectrum, the t(4;11)/MLL-AF4 and t(9;22)/BCR-ABL almost always confer a dire prognosis in both children and adults with ALL, who warrant high-dose chemotherapy and hematopoietic stem cell rescue to sustain or even induce first remission. Such chromosomal/molecular markers are being incorporated into risk classification schemes, as they convey prognostic information that cannot be gleaned from conventional risk factors such as immunophenotype, presenting age, and the initial circulating leukemic blast cell count. The most exciting prospect is the discovery of drugs that inhibit specific oncogenes, as illustrated by the BCR-ABL tyrosine kinase inhibitor STI-571.
journal_name
Semin Hematoljournal_title
Seminars in hematologyauthors
Ferrando AA,Look ATdoi
10.1016/s0037-1963(00)90018-0subject
Has Abstractpub_date
2000-10-01 00:00:00pages
381-95issue
4eissn
0037-1963issn
1532-8686pii
S0037196300000305journal_volume
37pub_type
杂志文章,评审abstract：:The past decade has seen improvements in overall survival (OS) for patients with the two most common lymphoma histologies: diffuse large B-cell lymphoma (DLBCL) and follicular lymphoma (FL). In FL, at least four independent datasets have confirmed these survival improvements. The monoclonal antibody rituximab has sign...
journal_title：Seminars in hematology
pub_type：
doi：10.1053/j.seminhematol.2008.02.005
更新日期：2008-04-01 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：: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：: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：:The introduction of chimeric antigen receptor (CAR) T-cell therapy in acute lymphoblastic leukemia (ALL) has dramatically altered the landscape of treatment options available to children and adults with ALL. With complete remission induction rates exceeding 70% in most trials and FDA approval of one CD19 CAR T-cell co...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2020.07.002
更新日期：2020-07-01 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：: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：:Hemangiomas and lymphangiomas are two main types of angiomatous disease that occur most commonly in infancy and childhood. Most hemangiomas resolve spontaneously, but some endanger vital structures such as the lung, as in pulmonary hemangiomatosis, a rare and universally fatal disease. Occasionally, hemangiomatous les...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1990-07-01 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：:Cytogenetic data suggest that acute myeloid leukemia (AML) develops through a process of branching evolution, especially during relapse and progression. Recent genomic data from AML cases using digital sequencing, temporal comparisons, xenograft cloning, and single-cell analysis indicate that most, if not all, AML cas...
journal_title：Seminars in hematology
pub_type： 杂志文章
doi：10.1053/j.seminhematol.2014.08.004
更新日期：2014-10-01 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：:Advances in recombinant DNA manufacturing technology have now made possible the production of a highly purified and active recombinant factor IX (rFIX) product. Recombinant factor IX was developed by (1) stable insertion of the genes for both factor IX and PACE-SOL (a truncated, soluble serine protease needed to enhan...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1998-04-01 00:00:00
abstract：:Most of the major advances in thrombosis research have occurred in the last 50 years, reflecting progress in biomedical sciences and clinical trials methodology. Improved understanding of the mechanisms of thrombogenesis has led to the discovery of a plethora of new antithrombotic agents that target many of the key st...
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：: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：: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：:Recombinant activated factor VII (rFVIIa) is being increasingly used to treat bleeding associated with a variety of non-hemophilic coagulopathic indications, and its mechanism of action in these areas is under active investigation. Numerous studies have shown that FVIIa binds with low affinity to activated platelets; ...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2008.03.013
更新日期：2008-04-01 00:00:00
abstract：:During myeloid differentiation, the pluripotent hematopoietic stem cell passes through several well-defined morphologic stages within the bone marrow. These changes include progressive nuclear segmentation and the acquisition of stage-specific granules. Primary granules appear at the myeloblast stage, and are found in...
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：:Primary immune thrombocytopenic purpura (ITP) remains a diagnosis of exclusion both from nonimmune causes of thrombocytopenia and immune thrombocytopenia that develops in the context of other disorders (secondary immune thrombocytopenia). The pathobiology, natural history, and response to therapy of the diverse causes...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2008.12.005
更新日期：2009-01-01 00:00:00
abstract：:Activation products of the terminal complement cascade potently affect granulocyte function, inducing, for example, their migration toward (chemotaxis), and adherence to (opsonization), microbes, and stimulating their production of microbicidal oxygen radicals such as superoxide anion, and the like. We present studies...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1979-04-01 00:00:00
abstract：:Severe neutropenia renders patients susceptible to life-threatening bacterial and fungal infections. Despite improvements in supportive care and antimicrobial therapy, morbidity and mortality remains significant. Since the 1960s, granulocyte transfusions have been used to either treat or prevent serious infections in ...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2019.11.002
更新日期：2019-10-01 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：:Idiopathic thrombocytopenic purpura (ITP) is an illness of primary acquired thrombocytopenia occurring in the absence of marrow failure. Splenectomy was first used as a treatment for ITP in 1913. However, with the realization that opsonin (critical for the optimal killing of invasive micro-organisms by white blood cel...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1016/s0037-1963(00)90114-8
更新日期：2000-01-01 00:00:00
abstract：:Over the past 2 decades, treatment of Hodgkin's disease has evolved considerably through innovations in the management of various stages. The impact of various treatments on the 5-, 10-, and 15-year results is being balanced against delayed morbidity, such as organ damage and second malignancies, produced by the inten...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：
更新日期：1988-04-01 00:00:00
abstract：:Hydroxyurea (HU) is the first widely used treatment to have an impact on the severity of disease in adult patients with sickle cell anemia, but limited data are available for younger patients or those with variant genotypes. We reviewed 324 months of experience with HU in 16 patients from 5.3 to 18.4 years of age trea...
journal_title：Seminars in hematology
pub_type： 临床试验,杂志文章
doi：
更新日期：1997-07-01 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：: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. Recen...
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：:Even though the overall outcome after allogeneic transplant has improved significantly in the last decades, late infectious diseases are still the most important causes of late morbidity and mortality. Here, impaired immune reconstitution and therapy of chronic graft-versus-host disease (GVHD) represent the major risk...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/j.seminhematol.2011.10.009
更新日期：2012-01-01 00:00:00
abstract：:Iron is a vitally important element in mammalian metabolism because of its unsurpassed versatility as a biologic catalyst. However, when not appropriately shielded or when present in excess, iron plays a key role in the formation of extremely toxic oxygen radicals, which ultimately cause peroxidative damage to vital c...
journal_title：Seminars in hematology
pub_type： 杂志文章,评审
doi：10.1053/shem.2002.35638
更新日期：2002-10-01 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：: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
