Abstract：
:To characterize T-cell large granular leukemia in Asia, 22 Chinese patients from a single institute were reported, together with an analysis of 88 Asian and 272 Western patients identified from the literature. In our cohort, anemia due to pure red cell aplasia (PRCA) occurred in 15/22 (68%) of cases, being the most common indication for treatment. Neutropenia was only found in 8/22 (36%) cases, and recurrent infections, the most important clinical problem in Western patients, were not observed. None of our cases presented with rheumatoid arthritis. These clinical features were consistently observed when compared with the 88 other Asian patients. Combined data from our cohort and other Asian cases showed that Asian patients, compared with Western patients, had more frequent anemia (66/110, 60% versus 113/240, 47%; p=0.044), attributable to a much higher incidence of PRCA (52/110, 47% versus 6/143, 4%; p<0.001). However, Western patients presented more frequently than Asian patients with neutropenia (146/235, 62% versus 33/110, 30%; p<0.001) and splenomegaly (99/246, 40% versus 16/110, 15%; p< 0.001). Notably, Western patients were about eight to ten times more likely than Asian patients to have rheumatoid arthritis (73/272, 27% versus 4/106, 4%; p<0.001) and recurrent infections (81/272, 30% versus 3/107, 3%; p<0.001). These clinicopathologic differences have important implications on disease pathogenesis and treatment.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Kwong YL,Au WY,Leung AY,Tse EWdoi
10.1007/s00277-009-0895-3subject
Has Abstractpub_date
2010-04-01 00:00:00pages
331-9issue
4eissn
0939-5555issn
1432-0584journal_volume
89pub_type
杂志文章abstract：:Haemoglobinopathies are the world's most frequently found monogenic disorders. In the cases with high oxygen affinity, the decrease in the liberation of the oxygen determines a secondary erythrocytosis. In this work, we present 17 unrelated families of Caucasian race and of Spanish origin, with ten variants of haemogl...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-008-0581-x
更新日期：2009-03-01 00:00:00
abstract：:The present study attempted to build a single nucleotide polymorphism (SNP)-based risk model for predicting overall survival (OS) and event-free survival (EFS) in patients with core binding factor acute myeloid leukemia (CBF-AML). Adopting genome-wide SNP array using Affymetrix SNP array 6.0, we analyzed 868,157 SNPs ...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-018-3260-6
更新日期：2018-06-01 00:00:00
abstract：:The objective of this study was to investigate whether metabolic tumor volume (MTV) by positron emission tomography (PET) can be a potential prognostic tool when compared with Ann Arbor stage, in stages II and III nodal diffuse large B cell lymphoma (DLBCL). We evaluated 169 patients with nodal stages II and III DLBCL...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-011-1357-2
更新日期：2012-05-01 00:00:00
abstract：:Fanconi anemia (FA) is a DNA repair disorder resulting from mutations in genes encoding for FA DNA repair complex components and is characterized by variable congenital abnormalities, bone marrow failure (BMF), and high incidences of malignancies. FA mosaicism arises from reversion or other compensatory mutations in h...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-020-03954-2
更新日期：2020-05-01 00:00:00
abstract：:Ruxolitinib is a promising option for treating steroid-refractory acute graft-versus-host disease (SR-aGVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). In this study, we describe ruxolitinib treatment for SR-aGVHD in HSCT patients with Epstein-Barr virus-associated hemophagocytic lymphohisti...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-019-03864-y
更新日期：2020-02-01 00:00:00
abstract：:In follicular lymphoma the t(14;18) might be useful as a tumor marker in predicting the quality of the response to treatment. We investigated whether analyzing numbers of t(14;18)-positive cells in peripheral blood correlated with remission status in individual patients receiving a variety of treatments. Numbers of ci...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-003-0762-6
更新日期：2003-12-01 00:00:00
abstract：:We evaluated the clinical features, treatment modalities, treatment responses, and prognosis of our patients with immune thrombocytopenia (ITP). Furthermore, we estimated the frequency of ITP in the Thrace region of Turkey. Two hundred sixteen patients diagnosed with ITP between 2000 and 2012 at our center were retros...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-014-2220-z
更新日期：2015-03-01 00:00:00
abstract：:Recent developments in the field of targeted therapy have led to the discovery of a new drug, plerixafor, that is a specific inhibitor of the CXCR4 receptor. Plerixafor acts in concert with granulocyte colony-stimulating factor (G-CSF) to increase the number of stem cells circulating in the peripheral blood (PB). Ther...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-010-1098-7
更新日期：2011-05-01 00:00:00
abstract：:Single-nucleotide polymorphisms (SNPs) of cytotoxic T lymphocyte antigen-4 (CTLA-4) are important risk factors associated with autoimmune diseases and malignancies. This study explored the association of CTLA-4SNPs with the development of myeloma and evaluated the outcome of patients receiving bortezomib-based regimen...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-017-3203-7
更新日期：2018-03-01 00:00:00
abstract：:Sickle cell disease (SCD) is a relatively common inherited hemolytic anemia among individuals of African descent. Genetic factors might clarify clinical diversity of the disease and variations in treatment response. Some researchers investigated heme oxygenase-1 (HMOX1) or chemokine receptor 5 (CCR5Δ32) genotypes amon...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-019-03697-9
更新日期：2019-08-01 00:00:00
abstract：:To characterize the clinical features, associated disorders, and treatment of necrobiotic xanthogranuloma (NXG), a rare non-Langerhans cell histiocytosis, we conducted a retrospective review of pathologically confirmed NXG at Mayo Clinic Arizona from 1987 to June 2017. Data on clinical findings, laboratory findings, a...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-018-3301-1
更新日期：2018-08-01 00:00:00
abstract：:Aplastic anemia is a rare complication of thymoma and is properly documented in only few cases. Here, we report the case of a previously healthy, 65-year-old patient who was found simultaneously to have a spindle-cell thymoma and severe hypoplastic anemia with a mild infiltration of the bone marrow by CD4+ and CD8+ T ...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s002770050437
更新日期：1998-10-01 00:00:00
abstract：:Although B chronic lymphocytic leukemia (B-CLL) cells express the alpha chain of the interleukin-2 (IL-2) receptor CD25, little is known about the effect of IL-2 on apoptosis in B-CLL cells. We have shown previously that stimulation of B-CLL cells with a CpG-oligonucleotide induces IL-2 high affinity receptors. In our...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-010-0994-1
更新日期：2010-11-01 00:00:00
abstract：:Patients with malignancy often present with a variety of coagulation abnormalities which may ultimately lead to recurrent arterial and venous thromboses. Recently the presence of antiphospholipid antibodies in cancer patients has been proposed as one of the potential mechanisms promoting hypercoagulability. Here we re...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-002-0565-1
更新日期：2002-12-01 00:00:00
abstract：:Acute leukemia (AL) patients may experience more than one episode of bloodstream infection (BSI) caused by the same pathogen during the entire chemotherapy program. In order to identify factors influencing BSI recurrence (R-BSI) during subsequent phases of treatment, we analyzed all BSIs occurring to consecutively tre...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-013-1965-0
更新日期：2014-05-01 00:00:00
abstract：:Acute graft-versus-host disease (aGVHD) has become the important complication post-allogeneic hematopoietic stem cell transplantation. Abnormally activated T cells might play an important role in the pathogenesis of aGVHD. But its exact mechanism remains poorly understood. T cell immune response cDNA 7 (TIRC7) has bee...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-015-2300-8
更新日期：2015-06-01 00:00:00
abstract：:Most cases of erythrocytosis occur secondary to chronic tissue hypoxia or as a clonal disease such as polycythemia vera with somatic mutations in the Janus kinase 2 (JAK2) gene. Rarely, erythrocytosis is caused by hereditary gene mutations. This study investigated hereditary gene mutations in 38 unrelated Korean patie...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-014-2006-3
更新日期：2014-06-01 00:00:00
abstract：:Nondeletional hereditary persistence of fetal hemoglobin (nd-HPFH), a rare hereditary condition resulting in elevated levels of fetal hemoglobin (Hb F) in adults, is associated with promoter mutations in the human fetal globin (HBG1 and HBG2) genes. In this paper, we report a novel type of nd-HPFH due to a HBG2 gene p...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-008-0643-0
更新日期：2009-06-01 00:00:00
abstract：:Many studies have confirmed that overexpressed WT1 exists in leukemic cells, especially in AML. However, the immunophenotypic features of this sort of leukemic cells remain to be unclarified. We retrospectively analyzed the immunophenotype of 283 newly diagnosed AML patients with intermediated and poor cytogenetic ris...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-019-03808-6
更新日期：2020-02-01 00:00:00
abstract：:Several revisions of International Prognostic Index (IPI) have been proposed for patients with diffuse large B-cell lymphoma (DLBCL) after the introduction of rituximab. Expanding evidence suggests that baseline absolute lymphocyte count (ALC) is also an independent factor for outcome prediction. We investigated the o...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-013-1807-0
更新日期：2013-11-01 00:00:00
abstract：:The aim of this epidemiologic population survey was to assess the penetrance of the most frequent hemochromatosis (HFE) gene variants in ethnic Danish men. A cohort of 6,020 men aged 30-53 years was screened for HFE C282Y, H63D, and S65C variants by restriction fragment length polymorphism analysis. Subsequently, iron...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-008-0679-1
更新日期：2009-08-01 00:00:00
abstract：:Indolent follicular lymphomas are diseases which are generally incurable with conventional therapy. Although patients can survive for prolonged periods, the median duration of first remissions is about 2.5 years, and subsequent remissions progressively shorten with time. High-dose therapy with hematopoietic stem cell ...
journal_title：Annals of hematology
pub_type： 杂志文章,评审
doi：10.1007/s002770050503
更新日期：1999-05-01 00:00:00
abstract：:Thrombotic thrombocytopenia purpura (TTP) and atypical hemolytic uremic syndromes (aHUS) are distinct clinical disorders characterized by hemolytic anemia, thrombocytopenia, microthrombi, and end organ damage. TTP is characterized by a low ADAMTS13 activity level at diagnosis of <10 % ADAMTS13 activity, while aHUS is ...
journal_title：Annals of hematology
pub_type： 临床试验,杂志文章
doi：10.1007/s00277-015-2411-2
更新日期：2015-09-01 00:00:00
abstract：:In contrast to adult medicine, specific scoring systems predicting the treatment response for an individual pediatric patient (pt) with chronic myeloid leukemia (CML) have not yet been defined. We evaluated to what extend prognostic scores as described for adults (e.g., Sokal, Hasford, EUTOS score) resulted in compara...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-015-2367-2
更新日期：2015-08-01 00:00:00
abstract：:Aberrant promoter methylation of RASSF6 and RASSF10 occurs at a high frequency in acute lymphoblastic leukemia (ALL). Because of the complexity of the current minimal residual disease (MRD) detecting-methods, the DNA methylation status of the RASSF6 and RASSF10 genes could potentially become biomarkers for the assessm...
journal_title：Annals of hematology
pub_type： 临床试验,杂志文章
doi：10.1007/s00277-019-03775-y
更新日期：2019-12-01 00:00:00
abstract：:Azacitidine and decitabine, two hypomethylating agents, are known to be effective in the treatment of high-risk myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML) patients who cannot endure intensive cytotoxic chemotherapy or are not eligible for transplantation. However, the treatment response rate is l...
journal_title：Annals of hematology
pub_type： 杂志文章,评审
doi：10.1007/s00277-018-3464-9
更新日期：2018-11-01 00:00:00
abstract：:Marked polyclonal immunoglobulin (Ig)G4 hypergammaglobulinemia has exceptionally been reported. Here we report on two Algerian patients who presented a syndrome characterized by anemia, plasmacytic lymphadenopathy, renal manifestations, and a marked polyclonal IgG4 hypergammaglobulinemia leading to a hyperviscosity sy...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-006-0158-5
更新日期：2006-12-01 00:00:00
abstract：:Preliminary data suggest a faster immune recovery following non-myeloablative stem cell transplantation because of the persistence of recipient T cells, but the real impact on post-transplant infectious complications remains unknown. We retrospectively analysed the incidence of cytomegalovirus (CMV) infection in twent...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s00277-003-0706-1
更新日期：2003-10-01 00:00:00
abstract：:Standard conditioning for allogeneic bone marrow transplantation induces high transplant-related mortality (TRM) in patients with a poor performance status. Less intensive regimens have been tested to reduce the TRM; our purpose was to evaluate the feasibility and tolerability of a new combination: thiotepa and fludar...
journal_title：Annals of hematology
pub_type： 杂志文章
doi：10.1007/s002770100344
更新日期：2001-09-01 00:00:00
abstract：:Patients with chronic lymphocytic leukemia (CLL) who receive chemoimmunotherapy and do not achieve complete remission experience significantly shortened progression-free interval (PFS). Additionally, the majority of patients treated for relapsed disease demonstrate evidence of measurable disease. Eradication of minima...
journal_title：Annals of hematology
pub_type： 临床试验,杂志文章
doi：10.1007/s00277-016-2683-1
更新日期：2016-06-01 00:00:00
