abstract：:Myelofibrosis with myeloid metaplasia, also known as idiopathic myelofibrosis (IF) or agnogenic myeloid metaplasia, is one of the characteristic manifestations of polycythemia vera (PV) in the spent phase, and has a particularly adverse prognosis. IF may also present de novo. To date, treatment strategies for both spe...

journal_title：Seminars in hematology

authors： Fruchtman SM

更新日期：2003-01-01 00:00:00

abstract：:Chronic myeloid leukemia (CML) is a hematopoietic disorder characterized by malignant expansion of bone marrow stem cells. Currently, the only unequivocally curative treatment for CML is allogeneic stem cell transplant. Unfortunately, a large proportion of CML patients are ineligible for such treatment and alternative...

journal_title：Seminars in hematology

authors： Barnes DJ,Melo JV

更新日期：2003-01-01 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

authors： Hoelzer D,Gökbuget N,Ottmann OG

更新日期：2002-10-01 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

authors： Ponka P

更新日期：2002-10-01 00:00:00

abstract：:The farnesyltransferase inhibitors (FTIs) have been shown in early clinical trials to elicit antitumor actions in a broad range of solid and hematologic malignancies. The mechanism of FTI action involves blockade of farnesyltransferase, an enzyme implicated in multiple cell-signaling pathways involved in proliferation...

journal_title：Seminars in hematology

authors： Cortes JE,Kurzrock R,Kantarjian HM

更新日期：2002-07-01 00:00:00

abstract：:Since warfarin remains the predominate drug administered for long-term anticoagulation, optimizing therapy for maximum antithrombotic effect with minimal bleeding risk continues to challenge clinicians. Genetic differences exist that affect an individual's response to warfarin. The clinician can use genetic informatio...

journal_title：Seminars in hematology

authors： Chai SJ,Macik BG

更新日期：2002-07-01 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

authors： Dale DC,Bolyard AA,Aprikyan A

更新日期：2002-04-01 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

authors： Giralt S,Anagnostopoulos A,Shahjahan M,Champlin R

更新日期：2002-01-01 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

authors： Wonke B

更新日期：2001-10-01 00:00:00

abstract：:Recombinant activated factor VII (rFVIIa; NovoSeven, Novo Nordisk, Denmark) induces hemostasis in life- and limb-threatening bleeds and in major surgery of hemophilia A and B patients, regardless of inhibitor titer. A total of more than 6,500 patients have been treated, and NovoSeven has been administered in more than...

journal_title：Seminars in hematology

authors： Hedner U

更新日期：2001-10-01 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

authors： Estey E

更新日期：2001-10-01 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

authors： Wollowitz S

更新日期：2001-10-01 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

authors： Hambleton J

更新日期：2001-10-01 00:00:00

abstract：:Chronic myeloid leukemia (CML) is probably the best understood human malignancy at the molecular level, but among the hardest to explain to patients concerning appropriate treatment options. At present, we do not know the long-term outcome of promising new therapies such as the tyrosine kinase inhibitor imatinib mesyl...

journal_title：Seminars in hematology

authors： Appelbaum FR

更新日期：2001-07-01 00:00:00

abstract：:This article summarizes clinical results of the Intergroupe Francophone du Myélome (IFM) trials: high-dose therapy (HDT) supported with autologous stem cells improves survival, melphalan 200 mg/m(2) is the best preparative regimen, unpurged peripheral blood stem cells (PBSC) are the recommended source of stem cells to...

journal_title：Seminars in hematology

authors： Attal M,Harousseau JL

更新日期：2001-07-01 00:00:00

abstract：:Stem cell transplantation is a recognized treatment for many hematologic malignancies. Human leukocyte antigen (HLA) testing is a key assessment in the evaluation of potential recipients and selection of the appropriate stem cell donor. Over the past several years, technological advances have moved HLA testing from a ...

journal_title：Seminars in hematology

authors： Bray RA

更新日期：2001-04-01 00:00:00

abstract：:The value of prophylaxis against venous thromboembolism (VTE) is increasingly accepted in most surgical specialties, although the potential reduction in fatal pulmonary embolism has recently been questioned. The burden of VTE in hospital patients nevertheless remains high, partly attributable to underuse of thrombopro...

journal_title：Seminars in hematology

authors： Wille-Jørgensen P

更新日期：2001-04-01 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

authors： Sandberg H,Almstedt A,Brandt J,Castro VM,Gray E,Holmquist L,Lewin M,Oswaldsson U,Mikaelsson M,Jankowski MA,Bond M,Scoble HA

更新日期：2001-04-01 00:00:00

abstract：:Acute promyelocytic leukemia (APL) is characterized by reciprocal chromosomal translocations that always Involve the retinoic acid receptor-alpha (RARalpha) gene on chromosome 17. RARalpha variably fuses to the PML, PLZF, NPM, NuMA, and STAT 5b genes (X genes), leading to the generation of X-RARalpha and RARalpha-X fu...

journal_title：Seminars in hematology

authors： Rego EM,Pandolfi PP

更新日期：2001-01-01 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

authors： Brittenham GM,Sheth S,Allen CJ,Farrell DE

更新日期：2001-01-01 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

authors： Soignet S

更新日期：2000-10-01 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

authors： Ferrando AA,Look AT

更新日期：2000-10-01 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

authors： Reed CJ

更新日期：2000-10-01 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

authors： Bussel JB

更新日期：2000-07-01 00:00:00

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

authors： Samama MM

更新日期：2000-07-01 00:00:00

abstract：:Two membrane proteins express the antigens that comprise the Kell blood group system. A single antigen, Kx, is carried on XK, a 440-amino acid protein that spans the membrane 10 times, and more than 20 antigens reside on Kell, a 93-kd, type II glycoprotein. XK and Kell are linked, close to the membrane surface, by a s...

journal_title：Seminars in hematology

authors： Lee S,Russo D,Redman CM

更新日期：2000-04-01 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

authors： Bell WR Jr

更新日期：2000-01-01 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

authors： Margolis DA,Casper JT

更新日期：2000-01-01 00:00:00

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 wi...

journal_title：Seminars in hematology

authors： Willman CL

更新日期：1999-10-01 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

authors： Appelbaum FR

更新日期：1999-10-01 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

authors： Hirsh J,Weitz JI

更新日期：1999-10-01 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

authors： Metcalf D

更新日期：1999-10-01 00:00:00

abstract：:Tumor cells from patients with B-cell malignancies exhibit a number of immunophenotypic characteristics that may be responsible for their survival. It has been shown, for example, that down-modulation of CD154 (CD40 ligand), which allows B cells to respond to T cells, may account for some of the immune defects of thes...

journal_title：Seminars in hematology

authors： Nadler LM

更新日期：1999-10-01 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

authors： Laurence AD,Goldstone AH

更新日期：1999-07-01 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

authors： Campo E,Raffeld M,Jaffe ES

更新日期：1999-04-01 00:00:00

abstract：:Serum vitamin B12 levels are often low in human immunodeficiency virus (HIV)-infected patients. However, only a few patients appear to have actual vitamin B12 deficiency. A low red cell folate level accompanying the low vitamin B12 level makes the presence of vitamin B12 deficiency more likely. Our experience suggests...

journal_title：Seminars in hematology

authors： Remacha AF,Cadafalch J

更新日期：1999-01-01 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

authors： Walenga JM,Jeske WP,Wood JJ,Ahmad S,Lewis BE,Bakhos M

更新日期：1999-01-01 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

authors： Tefferi A

更新日期：1999-01-01 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

authors： Elder ME

更新日期：1998-10-01 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

authors： Warkentin TE

更新日期：1998-10-01 00:00:00