Abstract:
BACKGROUND:Routine electrophoretic analysis fails to detect a monoclonal component (MC) in a considerable portion of AL amyloidosis patients. We investigated whether the combination of immunofixation (IF) on agarose gel electrophoresis and bone marrow plasma cell (BMPC) light chain kappa/lambda ratio analysis could contribute to diagnosis in these cases. The possible use of the BMPC kappa/lambda ratio in monitoring the clone was also investigated. METHODS:We performed BMPC kappa/lambda ratio analysis and IF of serum and urine in 16 selected patients with no detectable MC at routine analysis, despite clinical features suggestive of primary amyloidosis. An anti-idiotypic monoclonal antibody specific for the amyloidogenic immunoglobulin and the BMPC kappa/lambda ratio were used to monitor the clone in a patient who underwent autologous peripheral blood stem cell transplantation. RESULTS:Abnormal kappa/lambda ratios were found in 14 (sensitivity 87.5%), and a MC in 12 (sensitivity 75%). Combination of the two analyses confirmed diagnosis in all cases. In one patient changes in the size of the clone, monitored on serial bone marrow aspirates by an anti-idiotypic antibody, paralleled variations of the kappa/lambda ratio. CONCLUSIONS:This study demonstrates that the combined use of IF and the BMPC kappa/lambda ratio is extremely powerful in AL amyloidosis. In addition, the BMPC kappa/lambda ratio should be considered for monitoring the amyloidogenic clone when serum or urine MC is not quantifiable.
journal_name
Haematologicajournal_title
Haematologicaauthors
Perfetti V,Garini P,Vignarelli MC,Marinone MG,Zorzoli I,Merlini Gsubject
Has Abstractpub_date
1995-09-01 00:00:00pages
409-15issue
5eissn
0390-6078issn
1592-8721journal_volume
80pub_type
杂志文章相关文献
HAEMATOLOGICA文献大全abstract:BACKGROUND AND OBJECTIVES:Hepatitis C virus (HCV) infection is an important cause of morbidity and mortality in patients affected by hereditary bleeding disorders and treated with non-virus inactivated clotting factor concentrates during the 1970s. INFORMATION SOURCES:In this review, we briefly report the present know...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:
更新日期:2002-05-01 00:00:00
abstract:BACKGROUND:We have previously shown that bortezomib induces a depletion of alloreactive T cells and allows the expansion of T cells with suppressive properties. In the current study, we analyzed the potential synergistic effect of bortezomib in conjunction with sirolimus in order to reduce-graft-versus-host disease wit...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2011.058677
更新日期:2012-09-01 00:00:00
abstract::It is currently unknown whether immunosuppressive therapy or hematopoietic stem cell transplantation is the most appropriate treatment strategy for children with refractory cytopenia and normal karyotype or trisomy 8. We report on 31 children with hypoplastic refractory cytopenia treated with immunosuppressive therapy...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.10683
更新日期:2007-03-01 00:00:00
abstract::Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive th...
journal_title:Haematologica
pub_type: 临床试验,杂志文章
doi:10.3324/haematol.2015.132530
更新日期:2015-12-01 00:00:00
abstract::Next-generation sequencing (NGS) has transitioned from research to clinical routine, yet the comparability of different technologies for mutation profiling remains an open question. We performed a European multicenter (n=6) evaluation of three amplicon-based NGS assays targeting 11 genes recurrently mutated in chronic...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2019.234716
更新日期:2020-04-09 00:00:00
abstract::Pseudomonas aeruginosa is one leading gram-negative organism associated with nosocomial infections. Bacteremia is life-threatening in the immunocompromised host. Increasing frequency of multi-drug-resistant (MDRPA) strains is concerning. We started a retrospective survey in the pediatric hematology oncology Italian ne...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:10.3324/haematol.2009.020867
更新日期:2010-09-01 00:00:00
abstract::Recently, the principles of density gradient cell separation have been transferred to the marrow fractionation, and the Ficoll technique by using a COBE 2991 blood cell processor has been developed and widely employed as well. This method is particularly useful in view of a chemical antineoplastic purging intended for...
journal_title:Haematologica
pub_type: 临床试验,杂志文章
doi:
更新日期:1991-03-01 00:00:00
abstract:BACKGROUND:31D8 monoclonal antibody (mAb) has been shown to bind heterogeneously to human neutrophils, identifying subsets of cells which differ in their functional response to chemotactic stimuli. In this study we used 31D8 mAb to determine whether differences in neutrophil subpopulations might explain the long-lastin...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1995-03-01 00:00:00
abstract:BACKGROUND:Macrophages of the reticuloendothelial system play a key role in recycling iron from hemoglobin of senescent or damaged erythrocytes. Heme oxygenase 1 degrades the heme moiety and releases inorganic iron that is stored in ferritin or exported to the plasma via the iron export protein ferroportin. In the plas...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2009.020123
更新日期:2010-08-01 00:00:00
abstract::Patients with refractory or relapsed and refractory multiple myeloma who no longer receive benefit from novel agents have limited treatment options and short expected survival. del(17p) and t(4;14) are correlated with shortened survival. The phase 3 MM-003 trial demonstrated significant progression-free and overall su...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.3324/haematol.2014.117077
更新日期:2015-10-01 00:00:00
abstract::A case of thrombotic thrombocytopenic purpura (TTP) in a 40-year-old bisexual man unaware of being HIV positive is reported. The hematologic syndrome represented the first clinical manifestation of this viral infection. The clinical picture, characterized by severe hemolytic microangiopathic anemia, thrombocytopenia, ...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1994-05-01 00:00:00
abstract::We evaluate the long-term results of a prospective clinical study enrolling more than 100 adult patients with Burkitt lymphoma/leukemia. Depending on extent of disease, treatment consisted of six to eight rituximab infusions and four to six courses of intensive chemotherapy (attenuated in patients aged >55 years) with...
journal_title:Haematologica
pub_type: 临床试验,杂志文章,多中心研究
doi:10.3324/haematol.2013.086827
更新日期:2013-11-01 00:00:00
abstract::Chronic graft-versus-host disease is a serious complication in long-term survivors of allogeneic hematopoietic stem cell transplantation, with several organ systems affected. Chronic graft-versus-host disease is an important cause of morbidity and mortality in allogeneic hematopoietic stem cell transplantation. This a...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.13311
更新日期:2008-11-01 00:00:00
abstract::This review deals with the chronic lymphoproliferative disease of large granular lymphocytes endowed with T and natural killer cell characteristics. The disease is sufficiently characterized to allow its distinction from other lymphoproliferative disorders of the T cell type. The heterogeneous clinical course and labo...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:
更新日期:1989-01-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Continuous erythropoietin receptor activator (C.E.R.A.) is an innovative agent with unique erythropoietin receptor activity and a prolonged half-life, which has the potential for administration at extended dosing intervals. The objectives of this dose-finding study were to evaluate the hemoglo...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.3324/haematol.10730
更新日期:2007-04-01 00:00:00
abstract:BACKGROUND:The mdr-1 gene, which codes for a 170-kd transmembrane glycoprotein (P170), is frequently overexpressed in multidrug resistant (MDR) tumor cell lines and in spontaneous tumors, including leukemia and lymphoma. However, it is also constitutively expressed as a normal gene in normal tissues. METHODS:We used h...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:1993-01-01 00:00:00
abstract::As the defective genes for more and more genetic disorders become unravelled, it is clear that patients with apparently identical genotypes can have many different clinical conditions even in simple monogenic disorders. Beta thalassemia occurs when there is a deficiency in the synthesis of beta globin chains. The clin...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:
更新日期:2005-05-01 00:00:00
abstract:BACKGROUND:The myeloproliferative neoplasms, essential thrombocytosis, polycythemia vera and primary myelofibrosis, share the same acquired genetic lesion, but the concept of JAK2 V617F serving as the sole lesion responsible for these neoplasms is under question, and there has been interest in identifying additional mu...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2011.045591
更新日期:2011-10-01 00:00:00
abstract::Hematopoietic differentiation is driven by transcription factors, which orchestrate a finely tuned transcriptional network. At bipotential branching points lineage decisions are made, where key transcription factors initiate cell type-specific gene expression programs. These programs are stabilized by the epigenetic a...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2017.174516
更新日期:2018-01-01 00:00:00
abstract::The last decade has witnessed great advances in our understanding of the genetic and biological basis of childhood acute lymphoblastic leukemia (ALL), the development of experimental models to probe mechanisms and evaluate new therapies, and the development of more efficacious treatment stratification. Genomic analyse...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2020.247031
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Thrombosis is the major risk factor for death in patients with paroxysmal nocturnal hemoglobinuria. Previous case reports indicate that venous thrombosis in patients with paroxysmal nocturnal hemoglobinuria is amenable to thrombolysis. DESIGN AND METHODS:We reviewed the outcome of thrombolytic therapy for p...
journal_title:Haematologica
pub_type: 杂志文章,评审
doi:10.3324/haematol.2011.049767
更新日期:2012-03-01 00:00:00
abstract:BACKGROUND:Phosphatidylserine exposure by red blood cells is acknowledged as a signal that initiates phagocytic removal of the cells from the circulation. Several disorders and conditions are known to induce phosphatidylserine exposure. Removal of phosphatidylserine-exposing red blood cells generally occurs by macropha...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2011.048694
更新日期:2012-04-01 00:00:00
abstract::A case-control study was conducted among Italian children treated with a stem cell transplant (SCT). Cases (n = 43) were allogeneic recipients with osteonecrosis, and the controls (n = 129) were matched to the corresponding cases on the basis of survival, SCT center and date of transplant. Univariate analysis showed t...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究
doi:
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND:Mutation C1149R in the von Willebrand factor (VWF) gene has been thought to cause autosomal dominant severe type 1 von Willebrand disease (VWD). DESIGN AND METHODS:Eight patients from three unrelated families with this mutation were included in the present study who had distinct VWF abnormalities, not descr...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2008.003301
更新日期:2009-05-01 00:00:00
abstract::Pediatric thromboembolism (≤18 years) is very rare (0.07-0.14/10,000/year) but may be more prevalent in children with severe thrombophilia (protein C, protein S or antithrombin deficiency). The aim of this study was to define the prevalence and clinical characteristics of pediatric thrombosis in subjects with inherite...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2018.210666
更新日期:2019-12-01 00:00:00
abstract::There is emerging consensus that a pro-inflammatory condition contributes to the vaso-occlusive complications of sickle cell disease (SCD). We evaluated the potential value of inflammatory mediators as early markers of severity of painful vaso-occlusive crises (VOC) in SCD. We assayed the plasma levels of cytokines, s...
journal_title:Haematologica
pub_type: 信件
doi:
更新日期:2004-07-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Treatment of acute myeloid leukemia (AML) in older patients remains unsatisfactory. The BGMT 95 trial for older patients set out to improve the outcome of these patients by adding a third drug (lomustine) to a 5+7 idarubicin and cytarabine schedule at induction and evaluating intermediate-dose...
journal_title:Haematologica
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.3324/haematol.11068
更新日期:2007-10-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Although acquired aplastic anemia is usually a chronic disorder, a small proportion of patients with aplastic anemia was reported to recover spontaneously without specific therapy such as allogeneic bone marrow transplantation or immunosuppressive treatment. We attempted to determine the rate ...
journal_title:Haematologica
pub_type: 杂志文章
doi:
更新日期:2001-09-01 00:00:00
abstract::New drugs are needed for the treatment of relapsed acute lymphoblastic leukemia and preclinical evaluation of the MEK inhibitor, selumetinib, has shown that this drug has excellent activity in those leukemias with RAS pathway mutations. The proapoptotic protein, BIM is pivotal in the induction of cell death by both se...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2017.185975
更新日期:2019-09-01 00:00:00
abstract::Patients with advanced myeloma experience a high symptom burden particularly near the end of life, making timely hospice use crucial. Little is known about the quality and determinants of end-of-life care for this population, including whether potential increases in hospice use are also accompanied by "late" enrollmen...
journal_title:Haematologica
pub_type: 杂志文章
doi:10.3324/haematol.2018.187609
更新日期:2018-08-01 00:00:00