Shwachman-Diamond syndrome.

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 delineation of specific erythrocyte glycolytic enzyme defects during the past three decades has clarified hitherto unexplained hereditary hemolytic syndromes. The glycolytic enzymopathies have proven to be important, not as a public health problem, but because the investigation of these experimental models of natu...

journal_title：Seminars in hematology

authors： Tanaka KR,Zerez CR

更新日期：1990-04-01 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

authors： Nucci M,Anaissie E

更新日期：2009-07-01 00:00:00

abstract：:Natural killer cells are an essential component of the innate immune system and play a crucial role in immunity against malignancies, without, at difference with T cells, requiring antigen priming or inducing graft-versus-host-disease. Hence, Natural Killer cells can provide a valuable source of allogeneic "off-the-sh...

journal_title：Seminars in hematology

authors： Caruso S,De Angelis B,Carlomagno S,Del Bufalo F,Sivori S,Locatelli F,Quintarelli C

更新日期：2020-10-01 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

authors： O'Connor OA

更新日期：2010-04-01 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

authors： Connors JM,Klimo P

更新日期：1987-04-01 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

authors： Green R,Dwyre DM

更新日期：2015-10-01 00:00:00

abstract：:Continuous daily treatment with all-trans-retinoic acid (ATRA) in patients with acute promyelocytic leukemia has been associated with a marked decline in the plasma drug concentration at the time of relapse. Recent pharmacologic studies have attempted to determine the reasons for the progressive reduction of plasma co...

journal_title：Seminars in hematology

authors： Adamson PC

更新日期：1994-10-01 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

authors： Skoda R,Prchal JT

更新日期：2005-10-01 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

authors： Kongtim P,Ciurea SO

更新日期：2019-07-01 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

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

更新日期：1999-01-01 00:00:00

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

authors： Parikh AR,Olnes MJ,Barrett AJ

更新日期：2012-10-01 00:00:00

abstract：:In previous studies, treatment with epoetin alfa facilitated preoperative donation of autologous blood (AB). However, some patients may not be able to donate sufficient AB to meet their surgical blood requirements when the time to surgery is short. In this multicenter, double-blind, placebo-controlled study, the abili...

journal_title：Seminars in hematology

authors： Tryba M

更新日期：1996-04-01 00:00:00

abstract：:Endogenous thrombopoietin (TPO) stimulates platelet production in nonhuman primates by inducing dose-dependent megakaryocyte development from early marrow hematopoietic progenitors and subsequent proliferation and endoreduplication. In nonhuman primates, recombinant human TPO, nonpegylated or pegylated rHu megakaryocy...

journal_title：Seminars in hematology

authors： Harker LA

更新日期：1998-07-01 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

authors： Monroe DM

更新日期：2008-04-01 00:00:00

abstract：:Mechanisms that support migration of leukocytes have been studied extensively in vitro. The adhesion of neutrophils under conditions of flow at venous shear rates primarily involves members of the selectin family. E-selectin is upregulated by cytokine stimulation of endothelial cells (ECs); P-selectin is upregulated b...

journal_title：Seminars in hematology

authors： Smith CW

更新日期：1993-10-01 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：:Thrombopoietin (TPO) regulates thrombopoiesis through activation of TPO receptors on the megakaryocyte cell surface, resulting in increased platelet production. The TPO receptor agonists are novel treatments for patients with chronic ITP aimed at increasing platelet production through interactions with the TPO recepto...

journal_title：Seminars in hematology

authors： Siegal D,Crowther M,Cuker A

更新日期：2013-01-01 00:00:00

abstract：:Mouse models of transplantation have been indispensable to the development of bone marrow transplantation (BMT). Their role in the generation of basic science knowledge is invaluable and is subject to discussion below. However, this article focuses on the direct role and relevance of mouse models towards the clinical ...

journal_title：Seminars in hematology

authors： Arber C,Brenner MK,Reddy P

更新日期：2013-04-01 00:00:00

abstract：:Untreated anemia is common in cancer patients. Previous studies have demonstrated that both the existence of cancer and treatment with chemotherapy can suppress the normal endogenous erythropoietic response to anemia, making some cancer patients transfusion cadidates. In placebo-controlled phase III studies, administr...

journal_title：Seminars in hematology

authors： Glaspy J

更新日期：1997-07-01 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

authors： Schlegelberger B,Heller PG

更新日期：2017-04-01 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：:In the era of modern communication, the physician and patient relationship has evolved to include an entirely new dimension-social media. This new dimension offers several opportunities for patient education, research and its dissemination, and professional development for health care providers; it can also serve as a...

journal_title：Seminars in hematology

authors： Abuhadra N,Majhail NS,Nazha A

更新日期：2017-10-01 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

authors： Gangat N,Wolanskyj AP

更新日期：2013-07-01 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

authors： Sousou T,Friedberg J

更新日期：2010-04-01 00:00:00

abstract：:The standard treatment of multiple myeloma is systemic chemotherapy. Despite 30 years of drug development in myeloma, there are no new drug regimens significantly superior to melphalan and prednisone. In addition, phase II studies of new drugs in myeloma have been disappointing, with low response rates and no prolonga...

journal_title：Seminars in hematology

authors： Kraut EH,Ju R,Muller M

更新日期：1998-07-01 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

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

authors： Burwick N,Shimamura A,Liu JM

更新日期：2011-04-01 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：:Bendamustine is a bifunctional mechlorethamine derivative that shares similarities to other alkylators; however, the presence of a benzimidazole ring may confer "nucleoside-like" properties and may allow the stabilization of the molecule leading to longer lasting DNA damage. Though bendamustine has demonstrated promis...

journal_title：Seminars in hematology

authors： Leoni LM

更新日期：2011-04-01 00:00:00