Abstract:
:According to the 2008 WHO classification, the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN) includes atypical chronic myeloid leukaemia (aCML), chronic myelomonocytic leukaemia (CMML), MDS/MPN-unclassifiable (MDS/MPN-U), juvenile myelomonocytic leukaemia (JMML) and a "provisional" entity, refractory anaemia with ring sideroblasts and thrombocytosis (RARS-T). The remarkable progress in our understanding of the somatic pathogenesis of MDS/MPN has made it clear that there is considerable overlap among these diseases at the molecular level, as well as layers of unexpected complexity. Deregulation of signalling plays an important role in many cases, and is clearly linked to more highly proliferative disease. Other mutations affect a range of other essential, interrelated cellular mechanisms, including epigenetic regulation, RNA splicing, transcription, and DNA damage response. The various combinations of mutations indicate a multi-step pathogenesis, which likely contributes to the marked clinical heterogeneity of these disorders. The delineation of complex clonal architectures may serve as the cornerstone for the identification of novel therapeutic targets and lead to better patient outcomes. This review summarizes some of the current knowledge of molecular pathogenetic lesions in the MDS/MPN subtypes that are seen in adults: atypical CML, CMML and MDS/MPN-U.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Zoi K,Cross NCdoi
10.1007/s12185-014-1670-3subject
Has Abstractpub_date
2015-03-01 00:00:00pages
229-42issue
3eissn
0925-5710issn
1865-3774journal_volume
101pub_type
杂志文章,评审abstract::Autoimmune hemolytic anemia (AIHA) is a rare comorbidity in colorectal cancer (CRC) and has an unknown etiology. Previously, we described an AIHA case secondary to CRC with ectopic band 3 expression. Herein, we investigated ectopic band 3 expression and erythrocyte membrane-bound IgG in a CRC cohort. Between September...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-020-02831-y
更新日期:2020-05-01 00:00:00
abstract::Graft-versus-host disease (GvHD) causes severe mucositis, impairs feeding and favors infection. The objective of this study was to identify the impact of GvHD in the oral cavity. We reviewed all consecutive patients who developed oral GvHD after HSCT. The study period was over 14 years. 53 patients were identified. M/...
journal_title:International journal of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s12185-018-2520-5
更新日期:2018-12-01 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
abstract::The clinical importance of the isolation of nontuberculous mycobacteria (NTM) from respiratory specimens of stem cell transplant (SCT) recipients is not clear. We investigated the characteristics and clinical impact of NTM isolation in this population. Medical records of adult patients who underwent SCT at the blood a...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-015-1745-9
更新日期:2015-05-01 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::The distinction between RAEB, RAEB-T and AML M6a is difficult when erythroblasts in the bone marrow (BM) exceed 50%. We analyzed 19 children (2 RAEB, 13 RAEB-T and 4 AML M6a) enrolled in a prospective pathological central review in Japan and divided them into two groups according to the myeloblasts percentage among no...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s12185-008-0183-3
更新日期:2008-12-01 00:00:00
abstract::Abnormal platelet-derived growth factor receptor (PDGFR)-mediated signaling may cause hematologic neoplasm. The PDGFR beta (PDGFRB) gene, located at chromosome band 5q31-33, forms a fusion gene as a result of chromosome translocation. Although patients with PDGFRB rearrangement mostly present with myeloproliferative n...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-016-2167-z
更新日期:2017-05-01 00:00:00
abstract::A 2-year-old Japanese boy who presented with multiple cervical, axillary, and inguinal lymphadenopathy was diagnosed by immunocytochemical analysis as having myeloid/natural killer (NK) cell precursor acute leukemia. Leukemic blasts in the bone marrow were positive for CD56 (NK marker), CD7 (T-cell marker), CD33 (myel...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02982029
更新日期:2002-02-01 00:00:00
abstract::A patient with t(9;22)-positive chronic myelogenous leukemia (CML) developed a resistance to therapy with imatinib mesylate (Glivec) which coincided with the appearance of t(5;6;12) in the same cells with t(9;22) [46,XX,t(5;6;12)(q14?;q21?;q23?),t(9;22)(q34;q11)]. She remains in a continuous chronic phase of CML. This...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-009-0275-8
更新日期:2009-05-01 00:00:00
abstract:BACKGROUND:The chronic myeloproliferative diseases (CMPDs) include chronic myelogenous leukemia (CML), primary (essential) thrombocythemia (PT), agnogenic myeloid metaplasia (AMM), and polycythemia vera (PV). Certain hematological malignancies have a different prevalence in our country than in countries with Caucasian ...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/BF02982113
更新日期:2002-06-01 00:00:00
abstract::Plasma cell leukemia (PCL) is a highly aggressive plasma cell disease characterized by a poor prognosis and a low response rate to conventional therapy. Herein, we describe a 69-year-old patient with primary PCL, developing testicular disease while in complete hematological remission, following by muscle involvement a...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-010-0745-z
更新日期:2011-02-01 00:00:00
abstract::We report the results of unrelated cord blood transplantation (CBT) in a 12-year-old boy with Philadelphia chromosome- positive chronic myelogenous leukemia in the chronic phase and a high body weight (68.5 kg at transplantation). Only 1 of the 2 units used engrafted. This unit was not human leukocyte antigen (HLA) cl...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.A10419
更新日期:2005-08-01 00:00:00
abstract::Recent studies have shown that tumors of relapsed acute myeloid leukemia (AML) present additional genetic mutations compared to the primary tumors. The base excision repair (BER) pathway corrects oxidatively damaged mutagenic bases and plays an important role in maintaining genetic stability. The purpose of the presen...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-018-2464-9
更新日期:2018-09-01 00:00:00
abstract::We describe a patient with Wegener granulomatosis (WG) who underwent long-term cyclophosphamide treatment and thereafter developed acute myelogenous leukemia (AML). After the AML was induced into remission, the patient received an allogeneic stem cell transplant (allo-SCT) from his sibling after undergoing a reduced-i...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/IJH97.05148
更新日期:2006-04-01 00:00:00
abstract::Cold agglutinin disease (CAD) is a rare, complement-mediated autoimmune hemolytic anemia. Patients with CAD in the United States and Europe have an increased incidence of thromboembolism (TE), but comparable information for patients in other regions is lacking. Thus, we examined TE risk for patients with CAD in Japan....
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-020-02899-6
更新日期:2020-09-01 00:00:00
abstract::Regulatory T-cells (Tregs) are major mediators of mammalian self-tolerance via cytotoxic T-lymphocyte antigen 4 (CTLA4) signaling pathways. An immune dysregulation syndrome associated with heterozygous germline mutations in CTLA4 was recently reported. Clinical features include recurrent infections, systemic lymphaden...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-020-02834-9
更新日期:2020-06-01 00:00:00
abstract::We report a case of successful umbilical cord blood transplantation (CBT) for Epstein-Barr virus (EBV)-associated lymphoproliferative disease (LPD) in a 6-year-old girl. The patient had hemophagocytic syndrome with excessive circulating levels of EBV DNA that was refractory to immunochemotherapy. Multiple hepatospleni...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1532/ijh97.04081
更新日期:2004-12-01 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::In an attempt to assess the effects and toxicity of brief induction chemotherapy plus involved-field irradiation for localized intermediate- and high-grade non-Hodgkin's lymphoma, we conducted a single-arm prospective trial between May 1987 and July 1991. Patients received four cycles of a five-drug chemotherapy regim...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1993-01-01 00:00:00
abstract::Narrowband ultraviolet B phototherapy (NB-UVB) is a therapeutic alternative for haematopoietic stem cell transplantation-related skin graft-versus-host disease (GVHD). The beneficial effects of this intervention may be induced by direct irradiation of inflammatory cells in the skin; however, the putative involvement o...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-014-1530-1
更新日期:2014-04-01 00:00:00
abstract::Elderly/comorbid patients with chronic lymphocytic leukemia (CLL) require low-toxicity treatments. Internationally, the standard treatment for such patients is chlorambucil and an anti-CD20 therapy; however, chlorambucil is not approved in Japan. The aim of the present study was to evaluate the safety, efficacy and ph...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-017-2233-1
更新日期:2017-08-01 00:00:00
abstract::The heart is a rare primary site of lymphoma, and cardiac involvement is thought to bring a poorer prognosis. A framework of known clinical presentations, diagnostic features, disease complications, treatments, and outcomes to improve prognostication was constructed by a systematic review in 2011. However, some aspect...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-020-02881-2
更新日期:2020-07-01 00:00:00
abstract::African Burkitt lymphomas (BL) are highly aggressive lymphomas mainly affecting children and young adults in Africa. This lymphoma was marked by its high sensitivity to chemotherapy in comparison to Sporadic Burkitt lymphoma. In this study, we evaluated the treatment response and survival of patients with CMA protocol...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-010-0591-z
更新日期:2010-06-01 00:00:00
abstract::It has been reported that Nak(a)-negative platelets lack GP IV. To examine the GP IV genetic defects in Nak(a)-negative platelets, we studied four unrelated Nak(a)-negative subjects by amplifying their GP IV cDNA, which were synthesized from platelet mRNA using the reverse transcriptase/polymerase chain reaction (RT/P...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:
更新日期:1993-04-01 00:00:00
abstract::Gene therapies are classified into two major categories, namely, in vivo and ex vivo. Clinical trials of human gene therapy began with the ex vivo techniques. Based on the initial successes of gene-therapy clinical trials, these approaches have spread worldwide. The number of gene therapy trials approved worldwide inc...
journal_title:International journal of hematology
pub_type: 杂志文章,评审
doi:10.1007/s12185-016-2030-2
更新日期:2016-07-01 00:00:00
abstract::The present study (ClinicalTrials.gov Identifier: NCT02221492) was conducted to assess the efficacy and safety of plerixafor for the mobilization and collection of haematopoietic stem cells (HSCs) for autologous transplantation in Japanese non-Hodgkin lymphoma (NHL) patients. In this randomized phase 2 study, patients...
journal_title:International journal of hematology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s12185-018-2505-4
更新日期:2018-11-01 00:00:00
abstract::Pluripotent stem cells of hematopoiesis are included among CD34+ cells in the blood and bone marrow. After granulocyte-colony stimulating factor (G-CSF) mobilization, 1-2% of the mononuclear cells in the blood are CD34+ cells, which can be obtained by leukapheresis. We performed CD34+ progenitor cell transplantation i...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1016/s0925-5710(97)00088-1
更新日期:1998-01-01 00:00:00
abstract::To elucidate the relationship between treatment with granulocyte colony-stimulating factor (G-CSF) and the development of chromosomal abnormalities and clonal evolution in adult aplastic anemia (AA) patients, we performed a prospective multicenter study. Of the 104 registered patients, 91 were found by the central rev...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:
更新日期:2003-02-01 00:00:00
abstract::We propose an algorithm based on a slightly modified version of MD Anderson Cancer Center (MDACC) score (i.e., mutational status of IgVH, LDH, presence of high-risk FISH abnormalities), β2-microglobulin and separation of clinical monoclonal B-cell lymphocytosis (cMBL) from chronic lymphocytic leukemia (CLL) to predict...
journal_title:International journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s12185-014-1634-7
更新日期:2014-09-01 00:00:00
abstract::To assess response to cyclosporine A, and/or corticosteroids, and possible factors influencing the response in adult patients with acquired pure red cell aplasia (PRCA). Clinical data from 42 cases were retrospectively analyzed. These patients received cyclosporine A (CsA), and/or corticosteroids (CS), or other immuno...
journal_title:International journal of hematology
pub_type: 杂志文章
doi:10.1007/s12185-018-2446-y
更新日期:2018-08-01 00:00:00