Abstract:
:Traditionally, acute myeloid leukemia (AML) has been diagnosed and classified based on the morphologic and cytochemical criteria of the French-American-British (FAB) classification system. However, more recent studies have demonstrated that the cytogenetic and molecular genetic abnormalities consistently associated with distinct forms of AML confer the most important prognostic information. Each broad category of de novo AML that arises in infants and children, young adults, and the elderly, or, AML arising secondary to antecedent myelodysplasia (MDS) or prior exposure to chemotherapy, radiotherapy, or environmental agents is characterized by distinct cytogenetic and molecular genetic abnormalities. Over the past 15 years, a number of these recurrent cytogenetic abnormalities have been cloned and characterized, yielding valuable insights into the mechanisms of leukemogenesis and providing powerful molecular tools for precise diagnosis and monitoring of minimal residual disease. As cytogenetic and molecular genetic characterization of AML cells is now considered crucial for both diagnostic and therapeutic decision-making, it is essential to have a working knowledge of the molecular basis of AML. This chapter reviews the molecular genetic features of different forms of AML and the new automated molecular technologies for residual disease assessment; the emerging molecular paradigms in this disease that are being exploited for therapeutic decision-making are presented.
journal_name
Semin Hematoljournal_title
Seminars in hematologyauthors
Willman CLsubject
Has Abstractpub_date
1999-10-01 00:00:00pages
390-400issue
4eissn
0037-1963issn
1532-8686journal_volume
36pub_type
杂志文章,评审abstract::The risk of postoperative venous thromboembolism (VTE) is dependent on the type of surgical procedure and specific characteristics of the patient. Patients with intrinsic factors placing them at higher risk of VTE remain at high risk, even if the procedural risk is low. The identification of factors such as age, obesi...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(00)90094-5
更新日期:2000-07-01 00:00:00
abstract::Diffuse large B-cell lymphoma (DLBCL) is the most frequent subtype of non-Hodgkin lymphoma in western countries. High prognostic heterogeneity is observed among patients with DLBCL with long-term survival ranging from 30% to more than 90% according to clinic-biological prognostics such as the International Prognostic ...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2015.01.001
更新日期:2015-04-01 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::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::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::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::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::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::The B-cell receptor (BCR) is of critical importance for normal B cells and for the majority of B-cell malignancies, especially chronic lymphocytic leukemia (CLL). The two major subsets of CLL are biologically distinct, being derived from B cells at different stages of differentiation and carrying unmutated (U-CLL) or ...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2014.05.003
更新日期:2014-07-01 00:00:00
abstract::Mechanisms of resistance to the tyrosine kinase inhibitor (TKI) imatinib had been modeled in vitro even prior to the first reports of clinical resistance in patients with chronic myeloid leukemia (CML). The discovery that BCR-ABL is reactivated at the time of resistance and the unveiling of point mutations within the ...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1053/j.seminhematol.2010.06.005
更新日期:2010-10-01 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::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::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::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 new high-purity recombinant factor VIII preparation has been developed for the treatment of hemophilia A. Structurally, this factor VIII preparation, B-domain deleted recombinant factor VIII (BDDrFVIII), differs from other recombinant and plasma-derived factor VIII preparations in that most of the B-domain has been ...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(01)90103-9
更新日期:2001-04-01 00:00:00
abstract::Inhibition of antibody-coated platelet destruction in patients with immune thrombocytopenic purpura (ITP) is a well-known mechanism of treatment effect. A number of interventions that would ameliorate the thrombocytopenic effect of ITP patient plasma when infused into normal recipients were demonstrated in 1965. Subse...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(00)90104-5
更新日期:2000-07-01 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::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 cyclophosphamide, vincristine, prednisone, bleomycin, doxorubicin, procarbazine (COP-BLAM) programs of combination chemotherapy were administered to patients with advanced diffuse large cell lymphoma. The original COP-BLAM programs were designed to deliver intense multidrug therapy maximizing tumor kill. COP-BLAM ...
journal_title:Seminars in hematology
pub_type: 杂志文章
doi:
更新日期:1988-04-01 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::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::Because optimal management of iron chelation therapy in patients with sickle cell disease and transfusional iron overload requires accurate determination of the magnitude of iron excess, a variety of techniques for evaluating iron overload are under development, including measurement of serum ferritin iron levels, x-r...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(01)90059-9
更新日期:2001-01-01 00:00:00
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...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(00)90018-0
更新日期:2000-10-01 00:00:00
abstract::The prolonged and severe myelosuppression associated with high-dose chemotherapy and autologous bone marrow transplantation is a major causative factor in patient morbidity and mortality resulting from infection and other causes. The recent molecular cloning, in vitro expression, and formulation of recombinant human c...
journal_title:Seminars in hematology
pub_type: 杂志文章
doi:
更新日期:1989-04-01 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::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::Von Willebrand disease (VWD) is the most commonly inherited bleeding disorder, caused by inheritance of a quantitative or qualitative abnormality of von Willebrand factor (VWF). While the majority of patients with VWD are successfully treated with adjunctive therapies or with the synthetic vasopressin analog desmopres...
journal_title:Seminars in hematology
pub_type: 杂志文章,评审
doi:10.1016/s0037-1963(01)90131-3
更新日期:2001-10-01 00:00:00
abstract::From a computational perspective, Bayesian methods can be viewed as a natural extension of familiar confidence intervals and significance tests, which sheds light on their meaning. This viewpoint shows that no special software is required to compute Bayesian results, leaving the distinctions between conventional and B...
journal_title:Seminars in hematology
pub_type: 杂志文章
doi:10.1053/j.seminhematol.2008.04.004
更新日期:2008-07-01 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::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