Abstract:
:Allogeneic hematopoietic stem-cell transplantation (HSCT) for chronic granulomatous disease (CGD) with a reduced-intensity conditioning regimen can be expected to lead to less therapy-related mortality and late-onset impairment, whereas it has also been reported to increase the risk of unsustained mixed donor chimerism and late rejection after transplantation. Herein, we report a 4-year-old boy with CGD who was successfully treated with unrelated bone marrow transplantation with a reduced-intensity conditioning regimen (RIC). Fludarabine-based RIC, 4 Gy of total body irradiation, 120 mg/kg of cyclophosphamide, and 125 mg/m(2) of fludarabine, was adopted for transplantation, followed with 8.9 x 10(8)/kg mononucleated donor cells infused without T-cell depletion. Although hematopoietic engraftment was rapidly obtained by day +17, he developed unstable donor chimerism. After tacrolimus withdrawal, the patient showed grade III acute graft-versus-host disease (GVHD), and subsequently reached full donor chimerism by day +61. Twelve months post-transplant, the patient has remained well with stable and durable engraftment, 100% donor chimerism, and normal superoxide production, without the requirement of donor lymphocyte infusions (DLI).
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Hasegawa D,Fukushima M,Hosokawa Y,Takeda H,Kawasaki K,Mizukami T,Nunoi H,Ochiai H,Morio T,Kosaka Ydoi
10.1007/s12185-007-0017-8subject
Has Abstractpub_date
2008-01-01 00:00:00pages
88-90issue
1eissn
0925-5710issn
1865-3774journal_volume
87pub_type
杂志文章abstract::Autophagy (also known as macroautophagy) is a lysosomal degradation pathway for the clearance of cellular materials, which manifests as an adaptive response to stress stimuli. Over the past decade, numerous studies have linked autophagy with cancer initiation, progression, and chemoresistance. Autophagy defects in nor...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-018-2414-6
更新日期:2018-05-01 00:00:00
abstract::We report our experience with allogeneic peripheral blood stem cell transplantation (allo-PBSCT) following a nonmyeloablative conditioning regimen consisting of cytarabine (8 g/m2) and cyclophosphamide (120 mg/kg) in the treatment of 2 patients aged 50 and 55 years with refractory chronic myelomonocytic leukemia and c...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:2000-12-01 00:00:00
abstract::The Wilms tumor gene WT1 is expressed in leukemias and various kinds of solid tumors, including lung and breast cancer, and exerts an oncogenic function in these malignancies, suggesting that WT1 protein is a novel, overexpressed tumor antigen. The WT1 protein, in fact, is an attractive tumor rejection antigen in anim...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF02982574
更新日期:2002-08-01 00:00:00
abstract::DNA methylation provides a major epigenetic code (besides histone modification) of the lineage- and development-specific genes (such as regulators of differentiation in the hematopoietic lineages) that control expression of normal cells. However, DNA methylation is also involved in malignancies because aberrant methyl...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1532/ijh97.04094
更新日期:2004-08-01 00:00:00
abstract::Neutrophils and other phagocytes migrate to the site of infection, ingest pathogens, and destroy them after releasing granule contents and active oxygen. These activities of the cells are closely associated with a rapid reorganization of the cytoskeleton, in which actin polymerizes, cross-links, anchors to the membran...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF02981993
更新日期:2001-08-01 00:00:00
abstract::We evaluated the efficacy and safety of switching to nilotinib in CML-CP patients who had achieved MMR with continuous detectable BCR-ABL1 transcript levels after long-term imatinib treatment. Patients who had achieved MMR, but not deep molecular response (DMR), after > 18 months from the initiation of imatinib receiv...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s12185-018-2401-y
更新日期:2018-05-01 00:00:00
abstract::Interferon regulatory factor-8 (IRF8) is a transcription factor expressed in hematopoietic cells, particularly in mononuclear phagocytes [monocytes/macrophages and dendritic cells (DCs)] and their progenitors. Various studies have demonstrated that IRF8 is essential for the development of monocytes, DCs, eosinophils, ...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-015-1761-9
更新日期:2015-04-01 00:00:00
abstract::We report here the successful treatment of cold agglutinin-associated refractory hemolysis with bortezomib in a patient with Waldenström's macroglobulinemia (WM). A 78-year-old man was referred to our hospital with cold hemagglutinemia of unknown cause. Laboratory examination revealed a hemoglobin concentration of 6.9...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1775-3
更新日期:2015-08-01 00:00:00
abstract::We present the case of a 62-year-old Japanese woman with relapsed adult T-cell leukemia/lymphoma (ATLL) who was treated with humanized anti-CCR4 monoclonal antibody (KW-0761). Although this antibody was highly effective against refractory ATLL, 6 months after the final KW-0761 infusion, the patient complained of hypox...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-013-1278-z
更新日期:2013-03-01 00:00:00
abstract::von Willebrand factor (VWF) performs its hemostatic functions through binding to various proteins. The A1 domain of VWF contains binding sites of not only physiologically important ligands, but also exogenous modulators that induce VWF-platelet aggregation. Sulfatides, 3-sulfated galactosyl ceramides, that are express...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-008-0065-8
更新日期:2008-05-01 00:00:00
abstract::Since the presence of erythroblasts (Ebl) in the peripheral blood of patients suspected to have aplastic anemia (AA) has been thought to suggest an error in diagnosis, such patients may not receive appropriate therapy promptly, with potentially fatal results. However, we recently experienced patients who had the typic...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1994-08-01 00:00:00
abstract::Allogeneic stem cell transplantation is an established treatment modality for a variety of hematologic malignancies. Unfortunately it carries a high risk of complications and toxicities related to the intensive preparative regimen which is traditionally used for pre-transplant myeloablation and the graft versus host d...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/BF03165241
更新日期:2002-08-01 00:00:00
abstract::We evaluated the efficacy of systemic high-dose methotrexate (HD-MTX) for CNS prophylaxis in a prospectively recruited cohort of DLBCL patients at high risk of CNS relapse. High-risk CNS relapse was defined as the involvement of ≥ 2 extranodal sites with elevated lactate dehydrogenase (LDH); CNS international prognost...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-019-02653-7
更新日期:2019-07-01 00:00:00
abstract::Homeostasis of the hematopoietic system has its roots in the maintenance of hematopoietic stem cells (HSCs) in the bone marrow (BM). HSCs change both phenotypically and functionally with physiological age. The alterations noted in aged HSCs are thought to be a consequence of both cell-intrinsic and extrinsic changes. ...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-014-1641-8
更新日期:2014-10-01 00:00:00
abstract::Transfusion is believed to be the main cause of iron overload in Japan. A nationwide survey on post-transfusional iron overload subsequently led to the establishment of guidelines for iron chelation therapy in this country. To date, however, detailed clinical information on the entire iron overload population in Japan...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-016-2141-9
更新日期:2017-03-01 00:00:00
abstract::A 17-year-old man with severe hemophilia A (factor VIII <1%) developed intermittent left upper quadrant pain. He had a high titer of factor VIII inhibitor (1024 Bethesda units/mL) and was diagnosed with intramural hematoma of the jejunum. He was managed conservatively with activated prothrombin complex concentrate (AP...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.06053
更新日期:2006-08-01 00:00:00
abstract::Lymphomas are a heterogeneous group of disease entities with well-defined clinical, morphological, immunophenotypic, and cytogenetic characteristics. Moreover, regional and racial differences have been reported in their incidence and subtype compositions. Here, we reviewed the epidemiology of lymphomas and summarized ...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-018-2403-9
更新日期:2018-04-01 00:00:00
abstract::Severe congenital neutropenia (SCN) is characterized by a maturation arrest of myeloid progenitor cells at the stage of promyelocytes in bone marrow and low levels of mature neutrophils in peripheral blood. To date, little is known regarding the underlying pathomechanism of SCN. A defective response of neutrophil prec...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1999-12-01 00:00:00
abstract::Polycomb-group genes (PcG), identified by Drosophila genetics, are believed to maintain positional information by constituting a cellular memory system. Recently this system has been proved to be supported by epigenetic transcription regulation. PcG products comprise two distinct complexes, PcG complex 1 and 2. First ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-007-0006-y
更新日期:2008-01-01 00:00:00
abstract::Detection of autoantibodies to erythrocytes is of fundamental importance in the diagnosis of autoimmune hemolytic anemia (AIHA). The routinely used direct antiglobulin test (DAT) has the disadvantage of low sensitivity. In this study, we investigated the optimal test conditions of measurement of red blood cell (RBC)-b...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02981936
更新日期:2001-02-01 00:00:00
abstract::The prognostic significance of Bcl-2, Bcl-6, p53, topoisomerase II, and β-tubulin expression was evaluated in diffuse large B-cell lymphoma (DLBCL) patients treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab. Eight-year progression-free survival (PFS, P = 0.006) and overall survival (...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1911-0
更新日期:2016-02-01 00:00:00
abstract::We retrospectively analyzed 126 acute myelogenous leukemia (AML) patients aged > or =60 years who had all been referred to the same hematological department between 1989 and 1999. In 76 de novo AML cases, 53 patients (median age, 72 years) were treated with combination chemotherapy (CT) for remission induction. Comple...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02981978
更新日期:2002-01-01 00:00:00
abstract::Spontaneous improvement (SI) occurs more frequently in children with chronic immune thrombocytopenia (cITP) than in adults. It is generally accepted that, with the exception of splenectomy, conventional medical approaches for cITP do not change the natural course of the disease. Previous studies on pediatric cITP have...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-012-1211-x
更新日期:2012-12-01 00:00:00
abstract::We describe the case of a patient with peripheral gamma/delta T-cell lymphoma (T-ML) with hepatosplenomegaly, generalized lymphadenopathy, and bone marrow involvement. A 44-year-old man had lymphoma, which became clinically apparent 2 months after the onset of myositis and insulin-dependent diabetes mellitus. A cervic...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1993-06-01 00:00:00
abstract::Multiple myeloma is a disease involving the clonal evolution of plasma cells that produce monoclonal immunoglobulin; however, other products, such as ammonia and amylase, reportedly are secreted by neoplastic plasma cells. We describe a patient with immunoglobulin A (IgA) myeloma who showed a high serum level of carci...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.06169
更新日期:2007-02-01 00:00:00
abstract::Recombinant activated factor VII (rFVIIa) (NovoSeven) is a novel hemostatic agent originally developed to treat bleeding episodes in hemophilic patients with inhibitors against coagulation factors VIII and IX. In recent years, rFVIIa has also been employed for the management of uncontrolled bleeding in a number of con...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1532/IJH97.E0517
更新日期:2006-02-01 00:00:00
abstract::Osteonecrosis (ON) is a potentially disabling complication encountered in children who receive chemotherapy for acute lymphoblastic leukemia (ALL). Considering the possible effect of ethnic difference on the clinical features of symptomatic ON in pediatric ALL, we retrospectively evaluated 245 children with ALL who we...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1790-4
更新日期:2015-07-01 00:00:00
abstract::Multicenter, prospective study was conducted to evaluate the efficacy of biweekly romiplostim in maintaining platelet ≥ 30 × 10(9)/L for at least 4 weeks. Treatment was started with a weekly injection (1 mcg/kg), and the dose was escalated until a titrated dose was achieved that maintained a platelet 50-200 × 10(9)/L ...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s12185-015-1889-7
更新日期:2016-01-01 00:00:00
abstract::We describe two episodes of CMV retinitis in a pediatric patient who underwent a CD34+ selected graft from his haploidentical father. Both recipient and donor were cytomegalovirus (CMV) seropositive. Both episodes occurred late post-grafting during a phase of complete immunological recovery with sufficient numbers of ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-008-0126-z
更新日期:2008-09-01 00:00:00
abstract::The study was aimed to explore the efficacy and safety of allo-HSCT with high-dose cyclophosphamide-induced immune tolerance for SAA. In the present study, 20 cases (12 male, 8 female; average age = 17.8 years) received reduced-intensity conditioning allo-HSCT from August 2012 to August 2014 in the Beijing Military Re...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-016-2106-z
更新日期:2016-12-01 00:00:00