bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Low Ionic Strength Reaction of Human Blood: Relationship between the Binding of Serum Immunoglobulin and Complement to Red Blood Cells and Surface Charge of the Cells
Authors: Hsu T. C. S. , Steinberg J. , Le Doux R. , Sawitsky A. 
Published: March 2008

Summary. Using the sucrose haemolysis reaction of Hartmann & Jenkins (1966) as a basic model, the low ionic strength reaction (LISR) of human blood was studied to determine: (1) serum Ig uptake by RBC with saline elution and 125I‐IgG uptake, and (2) complement fixation (CF) to RBC with lysis of PNH cells and C3/C4 antiglobulin haemagglutination (AH) of normal cells.The saline eluates were found to contain IgG and IgM with traces of IgA; their pH optima for the uptake by RBC were 6.0±0.5, 5.5±0.5 and c 5.0 respectively. The ratio of bound IgG to IgM was linearly related to the uptake pH. Both C4 AH and lysis were found to be optimum at pH 6.0±7.5, whereas the maximum C3 AH was at pH 6.0±0.5. The LISR performed at a constant pH (6.1±0.1) showed that an increasing concentration of neuraminidase (VCN) used in pretreatment of RBC was associated with a decrease in both IgG uptake and CF activity. A maximum VCN effect reduced the Ig uptake to c 20% of normal and abolished almost all the CF activity. An impaired LISR to various degrees was also observed with RBC pretreated with ficin, papain, bromelin, trypsin or protamine, and RBC from two individuals of En(a–) type. Preincubation of serum at LIS with and without RBC resulted in respectively a ‘complete’ and partial consumption of C in the fluid phase. The latter was not enhanced or inhibited by the addition of VCN‐treated RBC for preincubation.A hypothesis is proposed suggesting that in the LISR the Ig uptake by RBC is an electrostatic interaction of the oppositely charged RBC and Ig and the CF to RBC results from C activation by the cell‐bound IgG and IgM. In addition, a pH‐dependent inactivation of the cell‐bound C3 in the LISR is demonstrated.
Neonatal Erythrocyte Glutathione Peroxidase Deficiency as a Consequence of Selenium Imbalance during Pregnancy
Authors: Perona G. , Guidi G. C. , Piga A. , Cellerino R. , Milani G. , Colautti P. , Moschini G. , Stievano B. M. 
Published: March 2008

Summary. The red blood cell (RBC) glutathione peroxidase (GSH‐Px) activity and routine haematological parameters were measured in 38 healthy north Italian full‐term pregnant women and in their newborn infants. In 31 pairs the serum selenium concentration was also measured. Data were compared with those of 20 normal adult controls (10 males and 10 females).Newborn infants exhibited significantly lower RBC GSH‐Px activity and serum selenium concentrations than adult controls. Pregnant women had serum selenium values intermediate between those of adult female controls and their newborn infants.In both the pregnant women and newborns the RBC GSH‐Px activity correlated with the level of selenium in serum which suggests that the neonatal RBC GSH‐Px deficiency may be partially due to insufficient availability of selenium during pregnancy. Factors other than selenium concentration, e.g. hormonal and genetic, might also affect the RBC GSH‐Px activity as suggested by sex differences and mother/child concordances in enzyme activity found in our cases.
Hairy‐Cell Leukaemia: a B‐Lymphocytic Disorder
Authors: Jansen J. , Schuit H. R. E. , Van Zwet Th. L. , Meijer C. J. L. M. , Hijmans W. 
Published: March 2008

Fifteen cases of histologically proven hairy‐cell leukaemia (HCL) were studied with immunofluorescence, rosette, and phagocytosis techniques. Unfixed hairy cells (HC) bound all kinds of labelled antiserum; but after fixation with formaldehyde a much more selective binding was observed. In two cases no surface‐bound Ig was detected; four cases showed γ and in nine cases two or three heavy chains were found, α and δ being the most frequent. Few cases were clearly positive for μ. The picture was invariably monoclonal with respect to light chains. Cytoplasmic Ig was present in only 3/15 cases; it was always IgM.HC did not form E‐rosettes or react with a fluorescent anti‐T cell antiserum. No EAIgMC‐rosettes were formed. All cases showed Fc receptors, which were detected with EAIgG‐rosettes (13/13) or with antigen–antibody complexes (6/6). The density of Fc receptors varied widely. Incubation with latex particles resulted in cell‐associated particles in 16–63% of the HC; with Staphylococcus epidermidis, the percentage was 2–36. After enzyme treatment (lysostaphin), however, no ingested bacteria were found, which suggests that HC are essentially non‐phagocytic. At least 13 cases were therefore classified as B‐cell malignancies.
Relevance of Colony Forming Assays to Bone Marrow Transplantation with Particular Reference to Grafting for Aplastic Anaemia
Authors: Gordon M. Y. ,   for The Royal Marsden Hospital, Bone Marrow Transplantation Team
Published: March 2008

The value of bone marrow colony‐forming assays in monitoring transplanted patients has been assessed by comparing results from two pairs of bone marrow recipients. One pair received marrow from their identical twins for acute lymphoblastic leukaemia; the other pair were grafted with allogeneic marrow from their siblings for aplastic anaemia. One of each pair showed successful engraftment while in the others the grafts failed.The colony‐forming assay was then used to investigate marrow function in five grafted aplastic patients. Of these, four rejected their first grafts and required further immunosuppression before engraftment could be accomplished. The remaining patient was immunosuppressed at the outset with antithymocyte globulin (ATG) and her first graft was successful. Sera from all five patients inhibited colony formation by normal human marrow and it is suggested that this activity was related to graft rejection as well as to the pathogenesis of the condition.
Haem Synthesis in Sideroblastic Anaemia
Authors: Konopka L. , Hoffbrand A. V. 
Published: March 2008

Measurement of delta‐aminolaevulinic acid (ALA) synthetase has been performed in bone marrow of 24 normal subjects and 20 patients with sideroblastic anaemia (five congenital, two pyridoxine‐responsive, 12 primary acquired and one secondary form due to isoniazid therapy). ALA‐synthetase activity without added pyridoxal‐phosphate in vitro was decreased in four of the congenital cases, and in all 12 primary acquired cases. In two of these 16 cases with low ALA‐synthetase activity and in a case with sideroblastic change secondary to isoniazid therapy, the enzyme activity was corrected to normal by pyridoxal‐phosphate in vitro.ALA‐synthetase activity was normal in both cases of pyridoxine‐responsive anaemia while they were receiving pyridoxine therapy but in one of these cases, studied at repeated intervals after withdrawal of pyridoxine therapy, the bone marrow ALA‐synthetase activity fell to very low levels unless pyridoxal‐phosphate was added in vitro. In this case the Km of the enzyme for pyridoxal‐phosphate was substantially greater than that of a control sample. Haem synthetase activity was reduced in the bone marrow of two of six cases of primary acquired sideroblastic anaemia and in one congenital case tested but was normal in the pyridoxine‐responsive patient studied on pyridoxine therapy. Nevertheless, this patient showed reduced incorporation of 14C‐labelled delta‐aminolaevulinic acid into bone marrow haem.It is concluded that both congenital and primary acquired sideroblastic anaemia are a heterogenous group of disorders, with different defects in haem synthesis, reduced activity of delta‐aminolaevulinic acid synthetase being common but not invariable in both types. Pyridoxine‐responsive (congenital) sideroblastic anaemia may be due to the presence of an abnormal ALA‐synthetase apoenzyme which requires excessive amounts of the coenzyme, pyridoxal‐phosphate, to achieve normal activity; other abnormalities of haem synthesis may also be present.
Reduced Leucocyte Alkaline Phosphatase Activity and Decreased NBT Reduction Test in Induced Iron Deficiency Anaemia in Rabbits
Authors: Celada Antonio , Herreros Vincente , Pugin Paul , Rudolf Hans 
Published: March 2008

Summary. Iron deficiency anaemia was induced in rabbits by repeated bleeding. The leucocyte alkaline phosphatase (LAP) of 26 ± 28 units was significantly reduced compared with control values of 233 ± 36 units (P < 0.001). Leucocyte NBT reduction was also diminished, both in Hanks solution (P < 0.01) and in autologous serum (P < 0.001). After administration of iron, these values returned to normal.The results suggest that reduced LAP may reflect a deficiency of iron dependent constituents which are necessary for the integrity of normal granulocyte metabolism.
Work Capacity, Heart Rate and Blood Lactate Responses to Iron Treatment
Authors: Ohira Y. , Edgerton V. R. , Gardner G. W. , Senewiratne B. , Barnard R. J. , Simpson D. R. 
Published: March 2008

Summary. Changes in haemoglobin (Hb), work performance, heart rate and postexercise blood lactate were studied in iron deficient, anaemic subjects for 16 d following total dosage infusion of iron dextran, i.v. (30–50 ml). Six adult men and 14 women were subjects with initial Hb levels of 6.6±0.6 g/dl (mean±SEM) for the iron treatment group (n=10) and 8.0±0.7 for the placebo group (saline infusion, n=10). Serum iron levels were 0.51±0.15 and 0.67±0.12 mg/l for the two groups, respectively. Haemoglobin and maximal work time increased significantly within 4 d after iron treatment and continued to increase up to 16 d. No changes were found in the placebo subjects. Heart rates at a given exercise intensity were lower in the iron treatment group than in control subjects who had the same Hb levels but had not been treated with iron. Post‐exercise venous blood lactate was similar on succeeding days after iron treatement even though the subjects reached higher work loads.These results demonstrate that treatment of iron deficient, anaemic subjects with iron dextran results in improved work capacity within 4d and a lower heart rate at a given work load after treatment which cannot be accounted for totally by the elevation of Hb concentration.
Congenital Erythrocytosis: a New Form Associated with an Erythropoietin‐dependent Mechanism
Authors: Whitcomb W. H. , Peschle C. , Moore M. , Nitschke R. , Adamson J. W. 
Published: March 2008

Summary.Three patients with probable congenital erythrocytosis were studied to determine the role of erythropoietin (ESF) in their disease. In addition, haemoglobin function was measured and ESF excretion determined in response to reduction in the haemoglobin concentration. In two cases ESF excretion was clearly elevated above normal, and in the third excretion was normal even at an elevated PCV. In all, phlebotomy of 15–20% of the blood volume led to more than doubling in ESF excretion. This occurred in the presence of normal molecular haemoglobin function. Studies of the renal vasculature were normal. These results suggest a new form of congenital erythrocytosis associated with increased ESF production which is reciprocally related to the oxygen carrying capacity of the blood, but in the absence of any demonstrable abnormality in oxygen transport. These findings suggest an inherited defect, likely residing in the renal sensor responsible for the production of ESF. This defect is possibly due to impaired recognition of available oxygen or else a decrease in oxygen supply at the subcellular level.
Myeloid and Erythroid Colony Growth in Non‐anaemic Patients with Fanconi's Anaemia
Authors: Daneshbod‐Skibba Ghodsi , Martin Jean , Shahidi Nasrollah T. 
Published: March 2008

Summary.Myeloid and erythroid colony growth of the bone marrow and peripheral blood cells from one anaemic and four non‐anaemic patients with Fanconi's anaemia were studied. Markedly decreased myeloid and erythroid colony growth was found in all five patients regardless of their haematological status. The decreased colony formation in non‐anaemic patients with Fanconi's anaemia is thought to be due to an intrinsic cellular defect.
Superoxide Dismutase, Glutathione Peroxidase, Catalase and Lipid Peroxidation of Normal and Sickled Erythrocytes
Authors: Das Salil K. , Nair Rajagopalan C. 
Published: March 2008

Summary.Sickled erythrocytes showed reduced glutathione peroxidase and catalase activities in comparison to normal erythrocytes. In addition, increased levels of superoxide dismutase and ‘peroxidation potential’as well as fluorescent lipid pigments and malonaldehyde suggestive of membrane lipid peroxidation were found in sickled erythrocytes. Finally, sickled erythrocytes showed membrane ‘bound’Heinz bodies as well as reduced membrane lipids and unsaturated fatty acids. From these observations, it is suggested that membrane lipid peroxidation occurs in sickled cells.
Shear Rate Dependent Inhibition of Platelet Adhesion and Aggregation on Collagenous Surfaces by Antibodies to Human Factor VIII/von Willebrand Factor
Authors: Baumgartner H. R. , Tschopp T. B. , Meyer Dominique 
Published: March 2008

Summary.The effect of heterologous and homologous antibodies to factor VIII/von Willebrand factor (F.VIII/VWF) and purified VWF on the interaction of human platelets with subendothelium and with a surface consisting of collagen fibrils was investigated using annular flow chambers. Wall shear rates of 200–5200 s−1 were produced by varying the flow rate of the citrated blood which was circulated through these chambers by a pump. Surface coverage with platelets and with platelet aggregates was measured morphometrically. The antibodies had no effect on platelet adhesion at low shear rates. However, an increasing inhibition of adhesion was observed with increasing shear rates. The antibodies to F.VIII/VWF and to purified VWF virtually abolished adhesion at shear rates which are present in the microvasculature (1300–5200 s−1). By contrast, antibodies to factor VIII:C which were isolated from two patients with haemophilia A had no effect on adhesion. In addition antibodies to F.VIII/VWF and to purified VWF inhibited adhesion‐induced aggregation. We conclude that F.VIII/VWF plays an essential role as cofactor for platelet adhesion to subendothelium and collagen fibrils at high blood shear rates, i.e. when the time available for the establishment of a stable bond between platelets and collagenous surfaces is short.
Red Cell Pyrimidine 5′‐Nucleotidase and Glutathione in Myeloproliferative and Lymphoproliferative Disorders
Authors: Lieberman J. E. , Gordon‐Smith E. C. 
Published: March 2008

Summary. Red cell reduced glutatione (GSH) and pyrimidine 5′‐nucleotidase (Pyr 5′‐NT) were measured in a variety of myeloproliferative and lymphoproliferative disorders. Raised levels of GSH were found in chronic lymphocytic leukaemia, Hodgkin's disease, non‐Hodgkin's lymphoma, Waldenstrom's macroglobulinaemia and myeloma. Decreased activity of Pyr 5′‐NT was found in acute myeloblastic leukaemia, acute lymphoblastic leukaemia, chronic granulocytic leukaemia, chronic lymphocytic leukaemia, Hodgkin's disease and non‐Hodgkin's lymphoma. There was no correlation between the raised GSH levels and decreased Pyr 5′‐NT levels.
The Anaemia of P. falciparum Malaria
Authors: Abdalla S. , Weatherall D.J. , Wickramasinghe S. N. , Hughes Madeleine 
Published: March 2008

Summary.The haematological changes in a group of young Gambian children with P. falciparum malaria have been analysed. In children with acute infection anaemia was most marked during the period after treatment. Although many of these patients developed a positive direct Coombs test during this period of the illness it is not clear whether the anaemia which occurs after treatment has an immune basis. A second group of children showed quite different haematological findings. They appear to have a more chronic form of P. falciparum malaria infection, were profoundly anaemic at presentation, showed gross dyserythropoietic changes in their bone marrows, and had a full reticulocyte response and rise in haemoglobin after treatment. A third group of children were encountered whose haematological abnormalities were intermediate to those of the acute and chronic groups. These findings indicate that the patho‐physiological mechanisms responsible for the anaemia ofP. falciparum malaria are different at different stages of the illness.
Iron Binding Proteins in Selected Human Peripheral Blood Cell Sets: Immunofluorescence
Authors: Nishiya Koji , Chiao Jen Wei , Sousa Maria De 
Published: March 2008

Summary.Selected populations of human peripheral blood T lymphocytes, B lymphocytes, monocytes and polymorphonuclear cells were examined for their intracellular content of lactoferrin transferrin and ferritin by an indirect inimunofluorescence technique. Lactoferrin was found in polymorphs but not in lymphoid cells. Two different lactoferrin staining patterns were observed which we designated ‘perinuclear’, characterized by a ring of positive material round the nucleus, and cytoplasmic, in which most positive material was distributed in the cytoplasm. The former staining pattern was found in high density polymorphs the latter was associated with low density polymorphs occasionally found contaminating the peripheral blood mononuclear cell suspension. After incubation in vitro, the peri‐nuclear pattern changed to cytoplasmic staining.Transferrin was found in T cells and polyniorphs. In contrast, only a few transferrin containing cells were detected in the B cell and monocyte fractions. Following overnight incubation, a halo of positive material was found surrounding T cells stained for transferrin, suggesting that T cells released transferrin during incubation. Ferritin was also found in T cells and adherent cells. Following latex particle ingestion, the intensity of ferritin staining was markedly increased. Only a small proportion of lymphoid cells and monocytes stained for transferrin or ferritin in the total peripheral blood mononuclear cell suspension. The results indicate, therefore, that in response to manipulation procedures used routinely for the selection of human peripheral blood lymphoid cells, detectable amounts of transferrin and ferritin are present in T but not B cells. The fact that T cells are equipped with proteins known to participate in binding and storage of iron may constitute, at least in part, the basis for the contribution of these cells to the regulation of other major biological systems.
The Genetic Basis of Hb Q‐H Disease
Authors: Higgs D. R. , Hunt D. M. , Drysdale H. C. , Clegg J. B. , Pressley L. , Weatherall D. J. 
Published: March 2008

Summary.A Chinese family has been studied in which two siblings have haemoglobin Q‐H disease. Using a combination of haematological and haemoglobin analysis, globin chain synthesis, analysis of α/β globin messenger RNA ratios and restriction endonuclease mapping, it has been shown that each of these siblings has received one chromosome on which both α chain genes have been deleted and another on which there is only a single α chain locus which carries the αQ mutation. Their genotype is thus ——/—αQ. Despite the fact that the haemoglobin Q mutation in this family is carried on a chromosome with a single α chain locus, heterozygous carriers for the variant have only 25% or less haemoglobin Q. Our observations indicate that the molecular basis for haemoglobin Q‐α thalassaemia is similar to that for the common form of haemoglobin H disease in Orientals. Furthermore, they provide clear evidence that the level of an α chain variant in heterozygous carriers is not a reliable reflection of the number of α globin genes.
Another Example of Haemopoietic Chimaerism in Dizygotic Twins
Authors: Bird G. W. G. , Gibson M. , Wingham June , Mackintosh Pauline , Watkins Winifred , Greenwell Pamela 
Published: March 2008

Summary.Another example of haemopoietic chimaerism in dizygotic twins is described. Each twin had two distinct blood cell populations. The red cell populations differed in three blood group systems and in other genetic characters, and the white cell population in HLA types and in XX/XY karyotypes. The biological significance of these findings is discussed with special reference to the relative proportions of the two red and white cell populations, the ABH blood group gene‐specified glycosyltransferase levels, and the HLA types.
Deep Vein Thrombosis and Changes in Coagulation and Fibrinolysis after Gynaecological Operations in Chinese: the Effect of Oral Contraceptives and Malignant Disease
Authors: Tso S. C. , Wong V. , Chan Vivian , Chan T. K. , Ma H. K. , Todd D. 
Published: March 2008

Summary.Of 154 Chinese patients who underwent gynaecological operations, four showed a positive 125I fibrinogen leg scan for venous thrombosis, an overall incidence of 2.6%. In those who were on oral contraceptives and had major pelvic surgery for benign conditions, the incidence was 10.5%; in those who had Wertheim hysterectomy for carcinoma of cervix, it was 6.7%. This confirms the rarity of post‐operative thromboembolism in the Chinese. Fragment E showed a biphasic rise after major operation due to tissue injury and venous thrombosis. In patients with malignancy, the postoperative ‘fibrinolytic shutdown’, represented by decreased plasminogen activator together with increased α1, antitrypsin and C inhibitor levels, was more marked. In addition, α2 macroglobulin level was lower and fell significantly after operation. In patients on oral contraceptives, fragment E levels were higher after surgery and there was no decrease in plasminogen activator levels. Antithrombin III levels did not fall except in three of the four patients with venous thrombosis. A marked increase in fragment E level and a decrease in antithrombin III level might be useful diagnostic markers for postoperative venous thrombosis.
Ultrastructural Cytochemistry of Leukaemic Cells: Characterization of the Early Small Granules of Monoblasts
Authors: O'Brien Maureen , Catovsky D. , Costello Christine 
Published: March 2008

Summary An ultrastructural study of blast cells showing either monocytic or granulocytic differentiation was carried out with the acid phosphatase (AP) and myeloperoxidase (MPO) reactions. Eight cases of acute myeloid leukaemia (AML) and three of chronic granulocytic leukaemia in blast crisis were studied. A hitherto unrecognized small lysosomal granule characterized by AP activity and lack of MPO was present in the majority of cells of all six monoblastic leukaemias. These granules ranged from 0·05 to 0·2 μm in size and were distributed throughout the cytoplasm, frequently at the periphery of the cells. A small proportion of monoblasts showed AP reactivity in the Golgi cisternae. Both AP and MPO were positive in the granules of promonocytes; however, MPO positive granules were predominant in late promonocytes.Larger granules (0·2–0·6 μm) with MPO reactivity were characteristic of myeloblasts. In only two out of four cases did these granules show AP positivity, suggesting that, in contrast to monoblasts, AP activity is a late feature of myeloblastic differentiation. This study shows that ultrastructural cytochemistry may be helpful in the recognition and classification of acute leukaemias by demonstrating the early differentiation features of monocytic and granulocytic precursors.
The Increased Susceptibility of Young Red Cells to Invasion by the Malarial ParasitePlasmodium falciparum
Authors: Pasvol G. , Weatherall D. J. , Wilson R. J. M. 
Published: March 2008

SummaryThe relationship between red cell age and susceptibility to invasion by the malarial parasitePlasmodium falciparumhas been examined by several different methods including short‐term cultures of parasitized human blood. The results indicate that reticulocytes and young red cells are more susceptible to invasion by this parasite as compared with metabolically older cell populations. This is contrary to the current belief that red cells of all ages are invaded indiscriminately byP. falciparum.This observation has important theoretical, clinical and practical implications; its mechanism remains as yet unclear.