Abstract:
:Plasma cell leukemia (PCL) is a very aggressive and rare form of malignant monoclonal gammopathy characterized by the presence of plasmocytes in peripheral blood. It is classified as primary PCL occuring 'de novo', or as secondary PCL in patients with relapsed/refractory multiple myeloma. Primary PCL is a distinct clinicopathological entity from myeloma with different cytogenetic abnormalities and molecular findings, which are usually found only in advanced multiple myeloma. The clinical course is aggressive with short remissions and reduced overall survival. The diagnostic criteria are based on the percentage (>20%) and absolute number (2 × 10(9) /L) of plasma cells in peripheral blood. After establishing diagnosis, induction therapy should begin promptly which is aimed to rapid disease control and to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens, followed by high-dose therapy with autologous stem cell transplantation, are recommended. Allogeneic transplantation can be considered in younger patients. This article reviews recent knowledge of this hematological malignancy that is associated with a very poor prognosis.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Jelinek T,Kryukov F,Rihova L,Hajek Rdoi
10.1111/ejh.12533subject
Has Abstractpub_date
2015-07-01 00:00:00pages
16-26issue
1eissn
0902-4441issn
1600-0609journal_volume
95pub_type
杂志文章,评审abstract::Acute myelogenous leukemia with t(8;21)(q22;q22) developed in a 42-yr-old woman having thrombocytopenia with absent radii syndrome (TARS). Standard induction and postremission therapies were safely administered. With each successive chemotherapy, the onset of platelet recovery was not delayed, but peak platelet counts...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1034/j.1600-0609.2003.00054.x
更新日期:2003-04-01 00:00:00
abstract::In this study, Escherichia coli LPS dose-dependently (100-500 microg/ml) and time-dependently (10-60 min) inhibited platelet aggregation in human and rabbit platelets stimulated by agonists. LPS also dose-dependently inhibited the intracellular Ca2+ mobilization in human platelets stimulated by collagen. In addition, ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1999.tb01909.x
更新日期:1999-05-01 00:00:00
abstract:INTRODUCTION:Outcome of patients with standard-risk (SR) multiple myeloma (MM) has improved; however, subsets of patients do worse than expected. We sought to identify the factors associated with inferior outcome. METHODS:We evaluated 51 patients with SR MM that received upfront autologous hematopoietic stem cell tran...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12826
更新日期:2017-03-01 00:00:00
abstract:UNLABELLED:The role of growth hormone (GH) for maintaining normal erythropoiesis among non-GH-deficient elderly subjects is not known. OBJECTIVES:To determine relationships between the concentrations of serum insulin-like growth factor-1 (IGF-1) and it's carrier protein insulin-like growth factor binding protein 3 (IG...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00374.x
更新日期:2005-02-01 00:00:00
abstract:BACKGROUND:Limited progress had been made in prognostic stratification of patients with Hodgkin lymphoma (HL) until recent studies suggested that the number of CD68-expressing macrophages is prognostic in classical HL. However, its significance in Asian patients with HL has not been explored yet perhaps because of its ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01731.x
更新日期:2012-04-01 00:00:00
abstract::Polyclonal B cell activation occurred in 3 patients following treatment with intravenous immunoglobulin (i.v. Ig) for idiopathic thrombocytopenic purpura (ITP). The possibility that this may represent an anti-idiotype response and the hypothesis that prolonged remission of ITP may be induced by this mechanism are disc...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb00759.x
更新日期:1987-09-01 00:00:00
abstract:INTRODUCTION:Prenatal diagnosis of severe alpha- and beta-thalasssemia diseases is usually performed by DNA analysis. OBJECTIVE:To establish a simple method, we have evaluated the reliability of prenatal diagnosis by fetal blood analysis using automated capillary electrophoresis system. METHODS:Forty-seven fetal bloo...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2009.01245.x
更新日期:2009-07-01 00:00:00
abstract::Anaemia of inflammation (AI) is a frequent complication in patients suffering from chronic inflammatory disorders including infections, autoimmune and malignant disease. Cytokine imbalance with a shift towards T-helper (Th)1-type immune response seems to be important in the pathogenesis of this type of anaemia. Interf...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1046/j.0902-4441.2003.00197.x
更新日期:2004-02-01 00:00:00
abstract::Splenic volume was measured by visual assessment of planar images of the spleen, and also by single photon emission computerised tomography (SPECT) using 99mtechnetium tin colloid, in a group of 33 patients with primary thrombocythaemia (PT) or reactive thrombocytosis. Volumes greater than 337 cm3 correlated strongly ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00188.x
更新日期:1988-04-01 00:00:00
abstract:OBJECTIVES:Internal tandem duplications (ITDs) of the fms-like tyrosine kinase 3 ( FLT3) gene occur in 13-35% of patients with acute myeloid leukemia (AML). FLT3-ITD is associated with poor clinical outcome and is an indication for allogeneic stem cell transplantation (allo-SCT). METHODS:To investigate FLT3-ITD length...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2012.01785.x
更新日期:2012-07-01 00:00:00
abstract:INTRODUCTION:Health-related quality of life (HRQoL) is impaired in patients with hemophilia; however, the impact in mild/moderate hemophilia B and affected women is not well characterized. OBJECTIVE:To evaluate factors that affect HRQoL in adults with hemophilia B and caregivers of affected children. METHODS:US adult...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13055
更新日期:2018-06-01 00:00:00
abstract:OBJECTIVES:The purpose of this study was to evaluate serum leptin levels in idiopathic thrombocytopenic purpura (ITP), in order to determine the influence of leptin on the pathogenesis of ITP. SUBJECTS AND METHODS:Forty-six untreated patients with chronic ITP were compared with 40 healthy people of similar age, sex an...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2004.00231.x
更新日期:2004-05-01 00:00:00
abstract::We analyzed the expression of the inhibitor of apoptosis survivin by immunocytochemistry in bone marrow cells from patients with chronic myelomonocytic leukemia (CMML) to evaluate possible abnormalities in comparison with other myelodysplastic (MDS) and myeloproliferative syndromes, and to investigate a possible corre...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.0902-4441.2006.t01-1-EJH2588.x
更新日期:2006-06-01 00:00:00
abstract:OBJECTIVES:Interactions between hemopoietic cells and the stromal microenvironment or immunoreactive cells are mediated by specific cell surface receptors. The expression of those molecules may alter the adhesive qualities (mobility and homing) as well as immune response behavior of leukemic blasts. L-Selectin (CD62L) ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2003.00140.x
更新日期:2003-11-01 00:00:00
abstract::Spontaneous remission in patients with acute myeloid leukemia (AML) is a rarely reported phenomenon of usually short duration. The etiology remains unclear, but an association with preceding blood transfusions or bacterial infections has been reported. Triggered immune responses are suggested to play a potential role ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.2004.00248.x
更新日期:2004-07-01 00:00:00
abstract:OBJECTIVE:Cell cycle arrest by FOXO transcription factors involves in transcriptional activation of p27(kip1), although the exact mechanism remains unclear. And it has been evidenced that reduced level of p27(kip1) which is frequently occurred in human cancers has been associated with poor prognosis. In this study, our...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01077.x
更新日期:2008-08-01 00:00:00
abstract:OBJECTIVE:We report an extension study of patients with essential thrombocythaemia (ET) in the Hungarian Myeloproliferative Neoplasm (HUMYPRON) Registry, which demonstrated that over 6 years anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombotic events (TEs) vs...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13459
更新日期:2020-10-01 00:00:00
abstract:BACKGROUND:Primary Hepatic (PHL) and Primary Splenic (PSL) non-Hodgkin's Lymphoma are rare entities. Small series of PHL and PSL have been reported, suggesting a non-fortuitous association with Hepatitis C Virus (HCV) infection. The prognosis is believed to be dismal, with early recurrence and short survival. PATIENTS...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01081.x
更新日期:2008-07-01 00:00:00
abstract::Several plasma cells morphological changes have been described in monoclonal gammopathies, including intracytoplasmic crystals. We report one case of indolent kappa-chain multiple myeloma with renal insufficiency, featuring plasma cells with Auer-rod-like intracytoplasmic inclusions. The relationship between such aber...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2001.t01-1-00485.x
更新日期:2001-08-01 00:00:00
abstract:OBJECTIVE:The primary objective was to assess the effect of central nervous system involvement in acute myeloid leukemia (CNS-AML) on outcomes after allogeneic hematopoietic stem cell transplant (allo-HCT). The secondary objective was to assess the utility of pretransplant cerebrospinal fluid (CSF) assessment in AML. ...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/ejh.13314
更新日期:2019-11-01 00:00:00
abstract::This study describes a retrospective analysis on the transplant outcome of 56 consecutive patients with myelodysplastic syndrome (MDS) according to their response to hypomethylating agents (HMA). While 2-yr disease-free survival (DFS) of patients who transformed to acute myeloid leukemia (n = 12) was 25%, that of the ...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/ejh.12038
更新日期:2013-02-01 00:00:00
abstract:OBJECTIVE:Autologous stem cell transplantation (ASCT) has improved progression-free survival (PFS) and overall survival in eligible patients with newly diagnosed multiple myeloma (NDMM); however, relapse occurs. Maintenance therapy with lenalidomide (Len-Mt) extends survival and delays relapse and the subsequent initia...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13497
更新日期:2020-11-01 00:00:00
abstract::In a phase II study 28 patients with advanced multiple myeloma were treated with a five drug regimen consisting of vincristine, BCNU, adriamycin, melphalan and dexamethasone. 11 out of 13 patients without prior chemotherapy showed significant remissions (greater than 25% tumor cells mass reduction), 7 of them had more...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00831.x
更新日期:1988-03-01 00:00:00
abstract::An intensive protocol utilizing mitoxantrone, high-dose cytarabine, vincristine, etoposide and methylprednisolone as induction therapy for chronic myeloid leukemia in blast transformation is described. Fourteen patients were treated, with a remission/second chronic phase achieved in 64%. None of the 3 patients older t...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1996.tb00488.x
更新日期:1996-07-01 00:00:00
abstract:INTRODUCTION:Primary and post-ET/PV myelofibrosis are myeloproliferative neoplasms harboring in most cases driving mutations in JAK2, CALR or MPL, and a variable number of additional mutations in other genes. Molecular analysis represents a powerful tool to guide prognosis and clinical management. Only about 10% of pat...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1111/ejh.13318
更新日期:2019-12-01 00:00:00
abstract::Hemoglobinopathies are the most common autosomal recessive disorders and are mostly inherited in a recessive manner. However, certain mutations can affect the globin chain stability, leading to dominant forms of thalassemia. The aim of this work was the molecular and structural characterization of two heterozygous in-...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13029
更新日期:2018-06-01 00:00:00
abstract::In addition to BCR, various rare fusion partners for the ABL1 gene have been reported in leukemia. We have identified the fusion gene SNX2-ABL1 in a pediatric case of acute lymphoblastic leukemia (ALL), which has only once previously been reported in an adult patient. Cytogenetic analysis detected this fusion gene ari...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12234
更新日期:2014-03-01 00:00:00
abstract::A 62-yr-old man with two healthy daughters was diagnosed with osteomyelofibrosis. To our surprise, a female XX-karyotype was observed in bone marrow and confirmed in PHA-stimulated T-lymphocytes from peripheral blood. Further molecular genetic investigation revealed a submicroscopic translocation between the short arm...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12555
更新日期:2015-09-01 00:00:00
abstract::It is well-documented that iron chelation by desferrioxamine protects/improves the cardiac function in blood transfusion-dependent children suffering from beta-thalassaemia. In patients who do not become dependent upon blood transfusion until adulthood (ANT-patients), iron chelation by desferrioxamine may affect the c...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb00981.x
更新日期:1997-10-01 00:00:00
abstract:BACKGROUND:Non-Hodgkin lymphoma patients have a 25% increased risk of secondary primary neoplasms (SPNs). Regarding the controversy about the increased risk of SPN in patients exposed to radioimmunotherapy (RIT), we have analyzed this issue in a cohort of follicular lymphoma (FL) patients treated with/without RIT. PAT...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12775
更新日期:2016-12-01 00:00:00