Abstract:
:A 59-year-old man with beta-thalassaemia major is unusually well. He has no beta-chains in his haemoglobin but is heterozygous for the genes responsible for alphaA and for alphaG Philadelphia. In addition he is also heterozygous for the genes responsible for gammaF and a new gamma-chain, gamma75(E19) Ile-Thr, named gammaF Sardinia. It was not possible to isolate the mutant gamma-chain, but from the overall ratios of Ggamma-:Agamma-Chain and gamma75 Ile : gamma75 Thr, it could be inferred that the mutation had presumably occured in the Ggamma-chain. Some of the propositus' siblings have high levels of Hb A2, and it is suggested that they possibly carry a gene for Lepore chain with the deltabeta-crossover after residue delta126.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Grifoni V,Kamuzora H,Lehmann H,Charlesworth Ddoi
10.1159/000208204subject
Has Abstractpub_date
1975-01-01 00:00:00pages
347-55issue
6eissn
0001-5792issn
1421-9662journal_volume
53pub_type
杂志文章abstract::A new case of IgE myeloma is described. A 77-year-old woman presented with bone pain and fatigue. Serum protein analysis revealed a paraprotein of the IgE kappa type; bone marrow aspirate and immunofluorescence confirmed the diagnosis; ultrastructural examination showed immature plasma cells. Treatment with prednisone...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204851
更新日期:1991-01-01 00:00:00
abstract::The frequency of chronic lymphocytic leukemia (CLL) varies a great deal from one population to another. We have undertaken to compare the aspects of CLL in Maghreb and Europe through two series of comparable importance, studied during the same period and under similar conditions in Algiers and Amiens. This comparison ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206896
更新日期:1983-01-01 00:00:00
abstract:BACKGROUND:Not all patients with diffuse large B-cell lymphoma (DLBCL) are candidates for aggressive regimens. (90)Y ibritumomab tiuxetan ((90)Y-IT), an anti-CD20 radionuclide-conjugated antibody, has demonstrated clinical efficacy in DLBCL with a favorable toxicity profile. METHODS:This phase II trial investigated th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000368291
更新日期:2015-01-01 00:00:00
abstract::The distribution of thymus-dependent (T cells) and bone marrow-derived lymphocytes (B cells) was studied in 74 patients with Hodgkin's disease and 33 normal controls. A T cell deficit was found in untreated patients as well as in long-term survivors in remission. Therapy slightly enhanced the T cell depletion in Hodgk...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207911
更新日期:1976-01-01 00:00:00
abstract::Recombinant alpha-interferons are used as therapeutic agents in an increasing number of benign and malignant disorders. Long-term administration of recombinant alpha-interferon as a maintenance agent is associated with a small number of adverse side-effects which are responsible for patient intolerance of this drug. T...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204881
更新日期:1991-01-01 00:00:00
abstract::Essential thrombocythemia (ET), one of the chronic myeloproliferative disorders, is a clonal disorder of multipotent stem cells. Although most patients with ET have a prolonged benign course, a minority of patients may develop a blastic crisis similar to chronic myelogenous leukemia (CML). A case of ET terminating in ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204260
更新日期:1994-01-01 00:00:00
abstract::Hemochromatosis has generally been considered to be a genetic disease in which progressive iron accumulation over many years can lead to cirrhosis of the liver, hepatocellular carcinoma, diabetes, cardiomyopathy, and arthropathy. Iron depletion by phlebotomy has been the recommended therapy although a randomized trial...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000243797
更新日期:2009-01-01 00:00:00
abstract::A new deletion of the beta-globin gene cluster was characterized in a Turkish family. A 6-year-old male and his father were heterozygotes for this deletion. They presented with mild hypochromic microcytic anemia associated with elevated Hb F (15%) and normal Hb A2 levels (2.0%). This newly described Turkish type (delt...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203790
更新日期:1996-01-01 00:00:00
abstract::A case of systemic mastocytosis with unusual clinical manifestations, appearing as an isolated splenohepatomegaly, is described. The proliferative character is evident from the cytological characteristics of immaturity and the presence of a moderate percentage of mast cells in the peripheral blood. These cells make up...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207846
更新日期:1977-01-01 00:00:00
abstract::A 66-year-old Japanese woman was referred to us because of severe anemia and fever and presented at our hospital. She was eventually diagnosed as having acute myeloblastic leukemia (AML; M0) with non-Hodgkin lymphoma (NHL). We investigated the therapeutic efficacy of L-asparaginase (L-Asp), vincristine and prednisolon...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000243725
更新日期:2009-01-01 00:00:00
abstract::Neoplastic cells of 18 patients with multiple myeloma were studied using a panel of 6 monoclonal antibodies to B cells and monospecific antisera against the light chain types of immunoglobulin. OKT10 bound to the myeloma cells of all the patients, although only a small percentage of the cells reacted in 3 instances. M...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206423
更新日期:1984-01-01 00:00:00
abstract:BACKGROUND:Immunotherapy using recombinant human interleukin-2 (rIL-2) produces objective responses in a proportion of advanced cancer patients. While early investigators employed intravenous (i.v.) treatment regimens, recent clinical trials applied therapy schedules via subcutaneous (s.c.) injection, mostly in combina...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204503
更新日期:1993-01-01 00:00:00
abstract::Two patients with paraneoplastic cerebellar degeneration accompanying Hodgkin's disease were treated with plasma exchange in combination with chemotherapy or radiation therapy, respectively. Significant improvement in neurologic symptoms was obtained in 1 of them. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204012
更新日期:1995-01-01 00:00:00
abstract::Factor X inhibitors are rare. The few cases documented in the literature have occurred after viral prodromes, in association with cancer, or after exposure to antibiotics. Acquired factor X deficiencies are also rare and their etiology is largely unknown. We report a new case of a factor X inhibitor and review prior c...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000342115
更新日期:2013-01-01 00:00:00
abstract::A 67-year-old woman with agnogenic myeloid metaplasia, who underwent splenectomy 1 year after the diagnosis, experienced spontaneous hematologic remission 6 years after splenectomy. Ten months before her anemia improved, peripheral leukocytes no longer showed a shift to the left and peripheral erythroblasts had disapp...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205163
更新日期:1990-01-01 00:00:00
abstract::Natural killer (NK) cell activity against K562 cell targets and the distribution of T cell subpopulations were investigated in the peripheral blood of 25 patients affected by beta thalassemia major, 18 clinically healthy heterozygotes, and 25 age-matched normal subjects. It was found that thalassemia major patients di...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206598
更新日期:1984-01-01 00:00:00
abstract::Although multiple myeloma (MM) remains an incurable disease, its treatment has improved over the past decade. This improvement has been at least in part due to the introduction of novel antimyeloma agents with new mechanisms of action, including those that target both myeloma cells and the tumor microenvironment, with...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000094682
更新日期:2006-01-01 00:00:00
abstract::Primary lymphoma of the female genital tract is very rare. We report the case of a 36-year-old woman who was referred to our hospital because of an indeterminate Pap smear test. The colposcopy showed a thickening of the posterior vaginal wall and various irregular ulcerated nodular lesions. Histological examination, i...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000337336
更新日期:2012-01-01 00:00:00
abstract::Analysis of donor chimerism has become a routine method for the documentation of engraftment after allogeneic hematopoietic stem cell transplantation (HSCT). In recent years several groups have also focused on the application of this technique for the detection of relapsing disease after allogeneic HSCT. This review a...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000077555
更新日期:2004-01-01 00:00:00
abstract::A 26-year-old female with Ki-1-positive large-cell anaplastic lymphoma is reported. The neoplastic cells were phenotypically and genotypically of T cell origin. Initially, neoplastic cells invaded the skin and lymph nodes, and then invaded the sternal and vertebral bones, ribs and the iliopsoas muscle. Central nervous...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204672
更新日期:1992-01-01 00:00:00
abstract::Human urinary erythropoietin has been purified to homogeneity. The seven-step procedure yielded a preparation with a potency of 225,000 U/mg protein. SDS-polyacrylamide gel electrophoretic analysis of the purified hormone revealed a single protein band with a molecular weight of about 35,000 that migrated with the bio...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205057
更新日期:1990-01-01 00:00:00
abstract::The fibrinolytic response to surgical trauma was studied longitudinally in 7 pigs by measuring the fibrinolytic activity in the blood and vein wall simultaneously. The results indicated a marked suppression in fibrinolytic activity in the blood which was closely correlated with the activity in the vein wall. This corr...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207397
更新日期:1980-01-01 00:00:00
abstract::Treating patients with hemophilia and inhibitors is often problematic. The presence of inhibitors negatively impacts the effectiveness of treatment to achieve hemostasis especially in patients with hemophilia B, owing mainly to allergic reactions to factor IX (FIX) concentrates and the low success rate of immune toler...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000508722
更新日期:2020-07-22 00:00:00
abstract::A 16-year-old Japanese woman with acute myelogenous leukemia (AML) and t(6;9) received peripheral blood stem cell transplantation (PBSCT). Although chromosomal studies just prior to and following PBSCT showed a normal karyotype, reverse transcription-polymerase chain reaction (RT-PCR) detected the mRNA derived from th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203996
更新日期:1995-01-01 00:00:00
abstract::The level of granulocyte-macrophage colony-stimulating factor (GM-CSF) in the cerebrospinal fluid from 14 infants and children with meningitis and 6 patients who suffered other diseases besides meningitis was measured by our sensitive enzyme linked immunosorbent assay for GM-CSF. The minimal detection level of GM-CSF ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204796
更新日期:1991-01-01 00:00:00
abstract::Three consecutive patients considered to have end-stage acquired aplastic anaemia were given 100-160 mg/kg antilymphocyte globulin (ALG) i.v. followed by an infusion of 2-3.8 x 10(8) nucleated marrow cells/kg i.v. from HL-A one haplotype-identical, MLC-positive family donors. All patients showed autologous marrow reco...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208005
更新日期:1976-01-01 00:00:00
abstract::Pyruvate kinase (PK) from four patients with moderate to severe congenital non-spherocytic haemolytic anaemia was characterized by methods recommended by the ICSH. The possibility that two of the patients are true homozygotes cannot be ruled out, while the other two apparently represent double heterozygotes. All but o...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204184
更新日期:1994-01-01 00:00:00
abstract::The presence of the sickle cell (Hb S) gene in Saudi Arabia was first reported by Lehmann et al. in 1963 [Nature 198, pp. 492-493]. Later, Hb S, alpha- and beta-thalassaemia, glucose-6-phosphate dehydrogenase deficiency and other enzymopathies were shown to occur at a variable prevalence in different regions of the co...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205861
更新日期:1987-01-01 00:00:00
abstract::It was confirmed that activation of the kallikrein-kinin enzyme system in cryoglobulinemia might be initiated by activation of factor XII to factor XIIa by cryoglobulin. It was also demonstrated that cryoglobulin or fibrin clots lost their ability to redissolve on warming to 37 degrees C, and consequently the lysis ti...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206563
更新日期:1984-01-01 00:00:00
abstract::Tumor cell (TC) contamination of stem cell products can contribute to relapse after high dose chemotherapy and stem cell rescue. A new purging technology using replication-deficient recombinant adenovirus (Adv) containing the p53 tumor suppressor gene (Adv-p53) has been suggested to reduce tumor contamination of autol...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040931
更新日期:1999-01-01 00:00:00