Abstract:
:A mild anemia (hemoglobin 9 g/dl) was found in a patient from Seville (Spain) with marked morphological abnormalities in the peripheral blood smear. The red cell osmotic fragility showed a mild resistance curve with a mean cell fragility (MCF) of 0.375% NaCl (normal = 0.450). Chemical Chemical and thermal instability test and search for inclusion bodies gave positive results. Hemoglobin electrophoresis at pH 8.9 revealed absence of Hb A, a major component of fast mobility (94%), and increased Hb F and Hb A2 levels (1.5% and 4.6%, respectively). The fast fraction, isolated and purified by means of cellulose acetate electrophoresis, precipitated in acid acetone and treated with urea 8 M and mercaptoethanol, revealed an anomalous beta chain. Trypsin-digested globin peptides were separated by high-voltage electrophoresis at pH 6.4 and ascendant chromatography. With differential staining, an extra peptide was detected in an unusual site, more anodic than alpha Tp4 but in lower position. Peptide map of the fast beta chain, stained with ninhydrin, and also for Tyr, confirmed the position of the new peptide and the absence of the usual beta Tp13. The new peptide, separated by high-voltage electrophoresis at pH 3.5, revealed absence of Val and the presence of an additional Glu residue, which should appear only in position beta 126. The diagnosis of Hb Hofu (alpha 2 beta 2 126 Val----Glu; H4) was reached, thus interpreting its increase and the absence of Hb A, as an association with beta o-thalassemia, producing a mild hemolytic anemia. Evidence was obtained that Hb Hofu is a mild unstable hemoglobin variant.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Arends T,Garlín G,Guevara JM,Amesty C,Pérez-Bández O,Lorkin PA,Lehmann H,Castillo Odoi
10.1159/000206274subject
Has Abstractpub_date
1985-01-01 00:00:00pages
51-4issue
1eissn
0001-5792issn
1421-9662journal_volume
73pub_type
杂志文章abstract::Slow intravenous infusion of Na laurate (NaL) into guinea pigs caused a rapid appearance of platelet aggregates in the arterial blood and a precipitous fall in platelet counts. During the infusion of Na arachidonate (NaA) thrombocytopenia developed slowly, and few and smaller platelet aggregates appeared in the arteri...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207864
更新日期:1977-01-01 00:00:00
abstract::Leukemic cells of a patient with acute T-lymphoblastic leukemia (T-ALL) exhibiting the uncommon clinical feature of hypogammaglobulinemia were examined in terms of surface markers and immunologic functions. Employing various monoclonal reagents reacting with surface antigens present on T cells and additional conventio...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206496
更新日期:1984-01-01 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::Monosomy 7 myelodysplasia is a rare hematological entity and is associated with morphological abnormalities in bone marrow and peripheral smear, and poor prognosis in children. We describe 2 children with infantile monosomy 7 myelodysplasia which evolved to leukemia. One of them died after 1 month, and the other is st...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204135
更新日期:1994-01-01 00:00:00
abstract::The in vitro immune functions of peripheral blood lymphocytes have been studied in 12 children with beta-thalassaemia major and hypersplenism. The study was performed prior to splenectomy and on the 2nd, 6th, 15th and 30th day after splenectomy. It was found that before splenectomy, patients had low numbers of blood l...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205886
更新日期:1987-01-01 00:00:00
abstract::We have studied the amount and intracellular distribution of erythroblastic alkaline and acid phosphatase, nonspecific esterase, and N-acetyl-beta-glucosaminidase in 50 patients with acquired dyserythropoiesis. 19 morphologically normal bone marrow smears served as controls. Alkaline phosphatase was found in all contr...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206406
更新日期:1984-01-01 00:00:00
abstract::Peripheral blood lymphocytes from patients with chronic lymphocytic leukemia (CLL), lymphoplasmacytoid lymphoma, centrocytic lymphoma and hairy cell leukemia were studied by scanning electron microscopy (SEM). In general, SEM revealed rather homogenous cell populations. Most lymphocytes displayed a moderately villous ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207888
更新日期:1977-03-01 00:00:00
abstract::Fourteen patients with hematologic neoplasia (11 acute myeloid leukemias, 2 non-Hodgkin's lymphomas and 1 blast crisis of chronic myeloid leukemia) who underwent high-dose cytosine arabinoside (HIDARAC) therapy with or without sequential asparaginase (ASNase) were investigated in order to evaluate liver toxicity and a...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000206094
更新日期:1986-01-01 00:00:00
abstract::An unusual complication of chronic lymphocytic leukemia (CLL) is reported. The patient, a 79-year-old man, had a long standing history of CLL, that had been complicated by the development of a Guillain-Barré-like syndrome and a peripheral biclonal gammopathy. The biclonal immunoglobulins identified in the serum were I...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204593
更新日期:1992-01-01 00:00:00
abstract::The transcription factors GATA-1 and GATA-2 play key roles in gene regulation during erythropoiesis. Gene ablation studies in mouse revealed that GATA-2 is crucial for the maintenance and proliferation of immature hematopoietic progenitors, whereas GATA-1 is essential for the survival of erythroid progenitors as well ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000065660
更新日期:2002-01-01 00:00:00
abstract::The activity of gamma-glutamyl transpeptidase was investigated in normal and leukemia leukocytes. Enzyme activity was positively correlated with the proportion of mature neutrophils and monocytes. In isolated leukocytes from patients with acute myeloid leukemia low values were obtained compared to controls. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207384
更新日期:1980-01-01 00:00:00
abstract:OBJECTIVE:To review the diagnostic significance, safety and possible risk factors of splenectomy in fever of unknown origin (FUO) with splenomegaly. METHODS:The records of 54 patients with FUO and splenomegaly who underwent splenectomy in our hospital in the past 20 years were reviewed retrospectively. Pathologic find...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000118632
更新日期:2008-01-01 00:00:00
abstract::The first thrombocytopenia cases related to the human immunodeficiency virus (HIV) were described even before its isolation in 1983. Subsequently, multiple mechanisms have been proposed to elucidate the etiology of thrombocytopenia. In addition to other types of cytopenia affecting patients with HIV, thrombocytopenia ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000313782
更新日期:2010-01-01 00:00:00
abstract::It has been proposed that iron overload may adversely affect liver disease outcome. The recent identification of 2 mutations in the HFE gene related to hereditary haemochromatosis (Cys282Tyr and His63Asp) provided an opportunity to test whether they are associated with hepatic iron accumulation and the activity and se...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040991
更新日期:2000-01-01 00:00:00
abstract::We review the problem of lymphoma classification in the light of the Revised European-American Lymphoma (REAL) scheme, recently proposed by the International Lymphoma Study Group (ILSG). The REAL classification is a list of clinicopathologic entities, all well known from the literature, upon which the ILSG members agr...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203875
更新日期:1996-01-01 00:00:00
abstract::Patients with HIV-associated lymphocyte-depleted Hodgkin lymphoma (HIV-HL) often present with advanced, extranodal disease and aggressive clinical features, limiting definitive therapeutic intervention. Here we report two patients with HIV-HL who presented with multi-organ dysfunction as an initial manifestation of th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000500222
更新日期:2020-01-01 00:00:00
abstract::95 of 1,019 (9.3%) sera with monoclonal immunoglobulins (MIg) were found to have cold-reacting lymphocytotoxins (LCT). There was no difference in the prevalence of LCT in multiple myeloma, macroglobulinemia, cancer, lymphoma or benign monoclonal gammopathy. Prevalence of LCT was similar in various classes and types of...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206328
更新日期:1985-01-01 00:00:00
abstract::A hemophilia B patient, seropositive for HIV antibodies since 1984, came to us in March 1989 with a severe necrotizing lesion of the nose. It was an erythematous lesion and looked like rhinophyma. Microbiological examination of the skin biopsy showed the presence of Cryptococcus neoformans. At the time of the study, t...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205038
更新日期:1990-01-01 00:00:00
abstract::A 64-year-old man with multiple sclerosis developed a circulating anticoagulant. Three immunoglobulin fractions (I = IgG; II =IgG+IgA; III = IgM) were separated from patient serum by 33-percent ammonium sulfate precipitation and DEAE-52 cellulose column chromatography. Fractions I (IgG) and II (IgG+IgA) had factor VII...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207993
更新日期:1976-01-01 00:00:00
abstract::12 thalassaemic patients from Northern Sardinia showing the beta + phenotype were examined by isoelectric focusing and high-performance liquid chromatography techniques for the determination of the variant A gamma T globin chain of the foetal haemoglobin. Two patients (16.7%) were homozygotes for the A gamma T gene va...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206057
更新日期:1986-01-01 00:00:00
abstract::Plasma samples from patients with disseminated intravascular coagulation (DIC) associated with acute promyelocytic leukemia (APL) exhibited higher levels of the D-fragment of fibrin and fibrinogen degradation products [FDP(D)], with relatively lower levels of cross-linked fibrin degradation products (XDP), than sample...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205392
更新日期:1989-01-01 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
abstract::Malaria parasites growing inside human erythrocytes differ from mammalian cells in their mode of acquisition of bioavailable iron and in their susceptibility to the antiproliferative action of iron chelators. We have assessed here three major properties associated with these phenomena: (a) the stage-dependent nature o...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203952
更新日期:1996-01-01 00:00:00
abstract::The goal of the study was to investigate changes in expression of selected growth factors tentatively involved in regeneration of haematopoietic tissues (bone marrow and spleen) following cyclophosphamide (CY) damage in the mouse. The bone marrow (BM) and spleen were examined separately, since the regenerating pattern...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000068490
更新日期:2003-01-01 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::In cutaneous T-cell lymphomas (CTCL; mycosis fungoides and Sézary syndrome), the standard therapies tend to be effective but not curative. Single drug therapy with either interferon-alpha or retinoids shows a response rate of about 45%. In this article, we report the results obtained in the treatment of CTCL with a co...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204582
更新日期:1993-01-01 00:00:00
abstract:BACKGROUND:It is a common belief among women that iron compounds have unpleasant gastrointestinal side effects. OBJECTIVE:To assess the gastrointestinal side effects of iron prophylaxis in pregnancy. METHODS:A randomized, double-blind study comprising 404 healthy pregnant women allocated to four groups taking ferrous...
journal_title:Acta haematologica
pub_type: 杂志文章,随机对照试验
doi:10.1159/000089466
更新日期:2006-01-01 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::A modified cytochemical method was developed for demonstrating magnesium-activated adenosine triphosphatase in haemic cells. The enzyme was shown in a variety of normal and leukaemic, unfixed cells. The high sensitivity of this enzyme to all fixatives tried in this study hampered further exploration and characterizati...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205983
更新日期:1987-01-01 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