Abstract:
:A baby girl born at 31 weeks gestation showed severe haemolytic anaemia and hyperbilirubinaemia which led to exchange transfusion within the first 12 hours of life. There was no blood group incompatibility between mother and child but there was a marked stomatocytosis of the baby's red blood cells. Family history revealed a congenital stomatocytosis in the mother. Biochemical characterization of the defect was performed. Phospholipid analysis of the erythrocyte membrane of mother and child showed an increase in phosphatidylserine with a compensatory decrease in phosphatidylcholine and phosphatidylethanolamine. SDS-electrophoresis showed multiple modifications of the protein pattern with a decrease in band 6, an increased content of band 4.1b, a slight decrease in band 7 and a clear change in the shape of the protein band 3 pattern. The results suggest that the basis of the observed abnormalities is a common defect in protein posttranslational modification, rather than multiple genetic defects in the synthesis of several proteins. Haematologic, biochemical and clinical course of the disease in this preterm infant are discussed.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Huppi PS,Ott P,Amato M,Schneider Hdoi
10.1111/j.1600-0609.1991.tb00554.xsubject
Has Abstractpub_date
1991-07-01 00:00:00pages
1-9issue
1eissn
0902-4441issn
1600-0609journal_volume
47pub_type
杂志文章abstract:INTRODUCTION:Increased levels of Wilms' tumor (WT1) mRNA have been used to establish risk categories in patients with acute myeloid leukemia (AML). Raised values of WT1 have been associated with progression in myelodysplastic syndrome (MDS). METHODS:We retrospectively analyzed the available bone marrow (BM) samples fr...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13275
更新日期:2019-09-01 00:00:00
abstract::10 patients with CLL and 2 with CML were treated with gradually increasing doses of 1 alpha(OH)D3, up to 4 micrograms daily during 6 wk. 3 patients with preleukemia and 1 with myelofibrosis were treated with 2 micrograms daily of 1 alpha(OH)D3 for a prolonged period up to 17 wk. The treatment with 1 alpha (OH)D3 did n...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb00160.x
更新日期:1987-07-01 00:00:00
abstract::An unusual case having IgM monoclonal gammopathy with clinical and pathologic features of multiple myeloma (MM) in association with neutrophilia and nephrotic syndrome is reported. The patient showed lytic bone lesions, decreased IgG and IgA levels, Bence-Jones proteinuria, nephrotic proteinuria with edema, and histol...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00869.x
更新日期:2007-07-01 00:00:00
abstract::No consecutive analysis of BAALC and WT1 expressions associated with core-binding factor AML (CBF-AML) from diagnosis to hematopoietic stem cell transplantation (HSCT) has yet been reported. We investigated BAALC and WT1 expressions using a method of real-time quantitative polymerase chain reaction (RQ-PCR) at diagnos...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12142
更新日期:2013-08-01 00:00:00
abstract:OBJECTIVES:Anthracyclines and cytarabine are cornerstones for intensive chemotherapy in acute myeloid leukemia (AML). The goals of this study were to comprehensively assess deviations from theoretical doses and the impact of body-surface area (BSA) on patients' characteristics, physicians' strategy, dose adjustment, an...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12850
更新日期:2017-05-01 00:00:00
abstract::The present trial was designed to test the effects of G-CSF on the duration of the second phase of induction chemotherapy in children with newly diagnosed acute lymphoblastic leukemia (ALL). A total of 32 patients were assigned randomly to a group that received (14 patients; group A) or a group that did not receive (1...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1111/j.1600-0609.1995.tb01816.x
更新日期:1995-08-01 00:00:00
abstract:OBJECTIVES:The benefits of combined chelation therapy with daily deferiprone (DFP) and subcutaneous desferrioxamine (DFO) have been widely reported in literature. We retrospectively evaluated the efficacy of different schedules of combined chelation therapy and the incidence of adverse events. METHODS:We evaluated 36 ...
journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.2010.01447.x
更新日期:2010-07-01 00:00:00
abstract::The value of Y-body study for assessment of haemopoietic engraftment was analyzed in 50 consecutive patients submitted to allogeneic bone marrow transplantation (BMT) (sex-matched in 28 cases, sex-mismatched in 22). The study was performed weekly on bone marrow and peripheral blood smears in all cases, and alveolar ma...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00851.x
更新日期:1988-05-01 00:00:00
abstract:INTRODUCTION:Diamond-Blackfan anemia (DBA) is characterized by hypoplastic anemia, congenital anomalies, and a predisposition for malignancies. Most of our understanding of this disorder stems from molecular studies combined with extensive data input from international patient registries. OBJECTIVES:To create an overv...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12995
更新日期:2018-02-01 00:00:00
abstract::Treatment with all-trans retinoic acid (ATRA) improves the prognosis of patients with acute promyelocytic leukemia (APL), but ATRA syndrome may occur as a possible fatal side effect, especially in cases refractory to medication or involving pulmonary hemorrhage. We describe two patients with APL who suffered from intr...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.0902-4441.2006.t01-1-EJH2852.x
更新日期:2006-11-01 00:00:00
abstract::We generated a 100-kb map of the region 5' of the PML (promyelocytic leukemia) gene on human chromosome 15q22 and identified a new gene provisionally named STORP for stomatin-related protein. The STORP gene is positioned 2 kb upstream of the PML gene in a head-to-head configuration, and contains 7 exons spanning a gen...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2000.90054.x
更新日期:2000-02-01 00:00:00
abstract::Most cytostatic drugs exert their effect on cells in active cell cycle. To improve the effect of cytostatic drugs we have tried, prior to treatment in vitro, to recruit tumor cells from G0 with growth factors. Leukemic cells from the bone marrows of 26 patients with AML and CML in blast crisis were incubated with G-CS...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1996.tb00285.x
更新日期:1996-01-01 00:00:00
abstract::Our aim was to assess liver iron content, in thalassaemic patients, by using three different MR protocols and compare their data. Ninety-four thalassaemic patients (44 M and 50 F, mean age 25.82 +/- 8.3 yrs), were enrolled in the study. In each patient, three measurements of the liver iron content were performed, with...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2009.01223.x
更新日期:2009-05-01 00:00:00
abstract::ECHELON-1 study is a randomized open-labeled controlled trial investigating whether addition of brentuximab vedotin to chemotherapy offers benefit over the standard chemotherapy regimen in advanced Hodgkin lymphoma. After a median follow-up of 24.6 months, it has met its primary endpoint the reduction of modified prog...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.13269
更新日期:2019-09-01 00:00:00
abstract:OBJECTIVES:Homoharringtonine (HHT) was efficient in therapying patients with acute myeloid leukemia (AML) in China, but little is known about the mechanism of its action. As the abnormal activation of JAK2 associated pathway is important to AML, we try to explore the effect of HHT on JAK2-STAT pathway in AML cells, thu...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2008.01116.x
更新日期:2008-10-01 00:00:00
abstract::Flow cytometry (FCM) has gained wide use in the determination of clonality in B-cell lymphoproliferative diseases and many methodological variations exist. We have compared the suitability of a) dual fluorochrome (FITC/PE)-labelled monoclonal antibodies, b) single fluorochrome (FITC)-labelled monoclonal antibodies and...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1992.tb01793.x
更新日期:1992-01-01 00:00:00
abstract:OBJECTIVE:Warfarin represents the most commonly prescribed oral anticoagulants, which functions as an antagonist of vitamin K, an essential factor of blood coagulation cascade. Warfarin has a narrow therapeutic index. An insufficient dose can cause failure of antithrombotic effect, and an overdose increases a risk of b...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12348
更新日期:2014-10-01 00:00:00
abstract::6 patients with massive splenomegaly but no peripheral adenopathy were diagnosed by splenectomy as having lymphoma; and were found to have widespread disease. Despite the seriousness of their illness at presentation, all have survived for a median time of 101.5 months and reached complete remission. ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb01156.x
更新日期:1987-02-01 00:00:00
abstract::The effect of human recombinant interferon alpha 2 (IFN alpha 2) on hairy cells obtained from 16 patients was evaluated. All patients promptly responded to induction of remission with 2 X 10(6) U/m2 interferon alpha 2 b, three times a week, sc. In order to achieve a more detailed insight into the mode of action of int...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1987.tb01449.x
更新日期:1987-11-01 00:00:00
abstract::The oncogenes c-myc and c-ras are known to elicit a cooperative tumorigenicity. In this study we investigated their role in the pathogenesis of Hodgkin's disease. The expression of these oncogenes was determined in Hodgkin's disease patients by avidin-biotin peroxidase complex immunohistochemical staining and was comp...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00247.x
更新日期:1995-09-01 00:00:00
abstract:OBJECTIVES:As new, effective therapies emerge for acute lymphoblastic leukaemia (ALL), the results of clinical trials need to relate to standard of care. METHODS:We used the population-based Swedish ALL Registry to evaluate characteristics, treatment and long-term outcome in 933 patients with diagnosis between 1997 an...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13247
更新日期:2019-08-01 00:00:00
abstract::Cobalamin levels are frequently low in patients with dementia, but it is unclear if they represent definable deficiency and what the mechanisms are. Therefore, patients being evaluated for dementia who had low cobalamin levels but no obvious evidence of deficiency were studied hematologically, neurologically and with ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1995.tb00679.x
更新日期:1995-04-01 00:00:00
abstract:OBJECTIVE:Fluconazole or posaconazole is a standard of care in antifungal prophylaxis for patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT). However, many patients need to interrupt standard prophylaxis due to intolerability, drug-drug interactions, or toxicity. Micafungin has come to promin...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13372
更新日期:2020-04-01 00:00:00
abstract::Haemophilia remains a complex disorder to diagnose and manage, requiring close cooperation between multidisciplinary healthcare professionals. There are still many unmet challenges in haemophilia care. The first Team Haemophilia Education (THE) meeting, held on 7-8 May 2015 in Amsterdam, The Netherlands, aimed to prom...
journal_title:European journal of haematology
pub_type:
doi:10.1111/ejh.12760
更新日期:2016-07-01 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
abstract:OBJECTIVES:An aberrant pattern of expression of L-selectin and intercellular adhesion molecule 1 (ICAM1) may characterise CD34+ blast cells in myelodysplastic syndromes (MDS) and secondary acute myeloid leukaemia (sAML). METHODS:In a three-colour flow cytometric assay, we evaluated the expression of L-selectin and ICA...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00986.x
更新日期:2008-02-01 00:00:00
abstract:OBJECTIVE:Hematological treatment decisions in older adults with hematological malignancies are complex. Our objective is to study the impact of a comprehensive geriatric assessment on hematological treatment decision in older patients and the factors associated with change in treatment plan. METHODS:We conducted a cr...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13570
更新日期:2020-12-19 00:00:00
abstract:UNLABELLED:Protein C (PC) deficiency is an autosomal dominant inherited disorder associated with spontaneous and recurrent thrombotic events. Factor V Leiden (FVL) increases the risk of thrombosis in PC-deficient type I families. We have investigated the relationship between PC deficiency genotype and clinical phenotyp...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2011.01742.x
更新日期:2012-04-01 00:00:00
abstract:BACKGROUND:The single nucleotide polymorphism SNP309 with a change from T to G in the promoter region of the MDM2 gene is shown to increase the MDM2 protein levels and attenuate the p53 levels and associates with disease progression in several tumors. OBJECTIVE:In this study, the role of the polymorphism was investiga...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2010.01470.x
更新日期:2010-09-01 00:00:00
abstract:OBJECTIVES AND METHODS:We conducted a retrospective analysis to evaluate the outcomes of 28 heavily pretreated (median 3 (2-6) treatment lines, sixteen (57%) allotransplanted) relapsed/refractory acute myeloid leukemia patients who had failed salvage venetoclax-based therapies. RESULTS:The median age was 59 years (20-...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13527
更新日期:2021-01-01 00:00:00