bluuebunny/crossref_metadata_2025_split huggingface dataset:
Thalassaemia Types among Kurdish Jews in Israel
Authors: Horowitz A. , Cohen T. , Goldschmidt E. , Levene C. 
Published: March 2008

The existence of thalassaemia among Jews of the Kurdish community is, by now, well established. Among the earlier reports were those of Schieber (1945), Matoth, Shamir and Freundlich (1955) and Dreyfuss (1955).Several estimates of the thalassaemia gene frequency in this community have been made but none of these took account of the more recent classification of thalassaemia types (Ingram and Stretton, 1959; Huehns and Shooter, 1965). The main distinction is between α‐ and β‐thalassaemias. Although the clinical signs and many haematological deviations are similar in α‐ and β‐trait carriers, the haemoglobin A2 fraction is increased in β‐thalassaemia, but normal or decreased in α‐thalassaemia trait. Moreover, part of the α‐trait carriers have been reported to display inclusion bodies in their red cells after staining with Brilliant Cresyl Blue (Fessas, 1963).Matoth and Pinhas (1958) gave a gene frequency estimate of 0.066 for Kurdish Jews, which was based on the incidence of thalassaemia major in the community. This estimate applies only to the β‐thalassaemia gene(s) and disregards the possible existence of α‐thalassaemia. Cohen, Goldschmidt, Ada, Matoth, Theodor and Szabo (1959) screened families of Kurdish Jews for iron‐resistant hypochromic and microcytic anaemia. They arrived at an estimate of 0.25 for the frequency of thalassaemia heterozygotes, including probably the majority of α‐ as well as β‐trait carriers without distinction between them. Ramot, Abrahamov, Freier and Gafni (1964) observed a slightly lower heterozygote frequency of 0.20. Their screening method was based on elevated levels of Hb A2 and of foetal haemoglobin and probably detected only a small fraction of α‐thalassaemia trait carriers.Haemoglobin H disease was reported in several Israeli families from various Oriental countries (Ramot, Sheba, Fisher, Ager and Lehmann, 1959; Klibansky, Djaldetti, Joshua and De Vries, 1960; Zlotnik, Ramot and Hamosh, 1964). Among these, only the kinship described by Zlotnik et al. (1964) has an affinity to the Kurdish community, since some of its members originated from Urfa in Eastern Turkey.The aim of the present survey was to detect the different thalassaemia types existing in the community of Kurdish Jews, to study any possible interaction between them and to estimate their relative, though not their absolute, frequencies.
Anabolic Agents and Relative Polycythaemia
Authors: Verwilghen R. , Louwagie A. , Waes J. , Vandenbroucke J. 
Published: March 2008

after the administration of testosterone an increase in the haemoglobin concentration may occur, and the difference in haemoglobin concentration between males and females has been ascribed to this hormonal influence. This hypothesis has been confirmed experimentally by Kennedy (1962). An increase in the haemoglobin concentration has also been brought about by the administration of ‘anabolic’ drugs (Booij and Kuypers, 1962; Everse and Van Keep, 1962). Both testosterone and anabolic drugs have induced remissions in congenital and acquired aplastic anaemia (Kennedy and Gilbertson, 1957; Shahidi and Diamond, 1959, 1961; Kennedy, 1962). These authors also reported polycythaemia in some cases following treatment with androgens or anabolic drugs. Gardner and Pringle (1961) have described polycythaemia in a woman suffering from a tumour of the adrenal cortex. Booij and Kuypers (1962) reported two cases of polycythaemia after prolonged administration of Nandrolone‐phenylpropionate (Durabolin) at high dosage, and they also demonstrated that this drug had an erythropoietic action in rats. But, like most other workers they based their observations only on measurements on peripheral blood and they made no blood volume determinations. Thus they were unable to distinguish between an increase in haemoglobin concentration due to a decrease of the plasma volume and an absolute increase of the whole red cell mass. Kennedy (1957), however, observed a return to normal values of the red cell mass during treatment of breast cancer with testosterone and even an increase exceeding the normal values in some of the patients. Unfortunately no plasma volume determinations were made.The results in three patients with clinical signs of polycythaemia after prolonged administration of anabolic drugs are reported. Examination of the peripheral blood showed an increase in haemoglobin concentration and packed cell volume. However, determination of the plasma and red cell volume showed that these changes were due to a decrease of the plasma volume.
Sites of Red Cell Destruction in the Haemolytic Anaemia of Adoptively Immunized NZB Mice
Authors: Barnes R. D. S. , Tuffrey Maureen 
Published: March 2008

autoimmune haemolytic anaemia in the NZB strain of mice was first described by Bielchowsky, Helyer and Howie (1959) and a positive direct Coombs test (D.C.T.) in this condition was first reported by Holmes, Gorrie and Burnet (1961). The presence of another red‐cell autoantibody which cross‐reacts with human red cells (A.H.R.) has recently been observed in these mice (Holborow, Barnes and Tuffrey, 1965). It seems likely that the spleen also plays a part in this disease process. Splenomegaly has been noted and, in addition, splenectomy in young animals seems to reduce haemolysis in spontaneously diseased NZB mice (Helyer and Howie, 1963). Diminution of the effects of haemolytic disease following splenectomy in young male animals has been confirmed by Holmes and Burnet (1963). We have recently succeeded in inducing the formation of both D.C.T. and A.H.R. antibodies in young NZB mice by adoptively immunizing these animals with spleen cells from older animals possessing these antibodies. It appears that in such animals, the occurrence of the A.H.R. antibody enhances the increased red‐cell destruction associated with a positive D.C.T. (Barnes and Tuffrey, 1966).Using a 51Cr labelled red‐cell method in such an experimental model, we have now tried to relate the occurrence of these two antibodies to the haematological disturbance, paying particular attention to the role of the liver and spleen in the haemolytic process.
The Aplastic Anaemia–Paroxysmal Nocturnal Haemoglobinuria Syndrome
Authors: Lewis S. M. , Dacie J. V. 
Published: March 2008

SUMMARYSeven patients are described who suffered from the aplastic anaemia‐paroxysmal nocturnal haemoglobinuria syndrome. All had been first diagnosed as having aplastic anaemia. In one case the bone marrow became repopulated but the patient developed severe intravascular haemolysis and died of liver failure due to intrahepatic venous thrombosis. Two patients died of marrow aplasia; haemolysis was minimal and was only a laboratory phenomenon. Another patient also died of marrow aplasia but in his case haemolysis had contributed significantly to his illness. In three patients the bone marrow became repopulated completely or partially and the patients have recovered clinically despite the persistence of a small proportion of PNH red cells.The relationship between aplastic anaemia and PNH is discussed. It seems likely that the link between the diseases is the development of an abnormal clone of haemopoietic cells in a regenerating, previously aplastic, marrow.The effect of the superimposition of the PNH defect on the prognosis in aplastic anaemia is discussed. In five cases haemolysis had little or no influence on the course of the disease; but in two patients intravascular haemolysis was severe and was undoubtedly harmful.The influence of splenectomy on the course of the disease was studied. It was performed in four of the patients, but in only one did it seem to have a beneficial effect.
Regulation of Platelet Production: ‘Hypersplenism’ in the Experimental Animal
Authors: Gabriele Geswalda de , Penington D. G. 
Published: March 2008

SUMMARYThe distribution and survival of 51Cr‐labelled platelets in normal, splenectomized and ‘hypersplenic’ rats together with calculation of the total body platelets demonstrate that the influence of the spleen on the circulating platelet count is primarily due to pooling of platelets, the size of the pool being related to splenic size. In states of normal platelet production splenic enlargement would be associated with reduction in the circulating platelet count.
The Platelet Destruction Site in Thrombocytopenic Purpuras
Authors: Najean Y. , Ardaillou N. , Dresch C. , Bernard J. 
Published: March 2008

SUMMARYThe site of sequestration of 51Cr‐labelled platelets has been studied in 465 subjects, of whom 317 suffered from idiopathic thrombocytopenic purpura. The validity of the method was demonstrated in several ways: a given subject usually showed the same site of platelet sequestration when investigated more than once even after a long interval; there were characteristic and very different platelet sequestration curves in the thrombocytopenias due to bone marrow hypoplasia, hypersplenism or ITP; and there was a correlation between the preoperative in vivo results and the radioactivity found in the spleen after splenectomy.In ITP the destruction of labelled platelets was more often splenic in children and in patients whose thrombocytopenia responded to steroid therapy. There was a perfect correlation between the site of platelet destruction and the platelet rise immediately after splenectomy. There was a good correlation between the site of platelet destruction and the long‐term effectiveness of splenectomy.
The Esterase Activity in Megaloblasts, Leukaemic and Normal Haemopoietic Cells
Authors: Rozenszajn L. , Leibovich M. , Shoham D. , Epstein J. 
Published: March 2008

Summary. Esterase activity was investigated in blood cells of peripheral blood and bone marrow in healthy subjects and in various pathological conditions. As substrates were used: α‐naphthyl acetate, naphthol AS‐D acetate and naphthol AS‐D chloroacetate. Fast blue B and fast garnet G. B. C. were used as coupling agents.The distribution of AS‐D acetate esterase in different blood cells was described. Strong α‐naphthyl acetate esterase activity was found in monocytes, reticulum cells, megakaryocytes and in monoblasts in cases of monocytic leukaemia. Weak activity was shown in thrombocytes, and in lymphocytes.With naphthol AS‐D chloroacetate as substrate strong enzymatic activity was demonstrated in the cells of the neutrophilic myelocytic series. The activity was strong in the more mature cells. Blasts from acute myelocytic leukaemia showed enzymatic activity.The use of α‐naphthyl acetate and naphthol AS‐D chloroacetate as substrates proved to be of help in differentiating between the three types of acute leukaemia.In cases with megaloblastic erythropoiesis only strong α‐naphthyl acetate esterase activity was demonstrated in the megaloblasts. Successful treatment of pernicious anaemia with B12, resulted in disappearance of megaloblasts and cessation of the strong esterase activity. There seems to be a correlation between strong naphthyl acetate esterase activity and the presence of megaloblasts. An explanation of this phenomenon is suggested.
Absence of Intrinsic Factor from Human Portal Plasma during 57CoB12 Absorption in Man
Authors: Cooper B. A. , White J. J. 
Published: March 2008

Summary The 57CoB12 activity in blood obtained from the portal vein during 57CoB12 absorption did not have the biological or the immunological features in vitro of intrinsic factor from normal human gastric juice.This indicates that 57CoB12 probably is not absorbed from the intestine with intrinsic factor.
The Metabolism of Iron‐Dextran Given as a Total‐Dose Infusion to Iron Deficient Jamaican Subjects*
Authors: Wood J. K. , Milner P. F. A. , Pathak U. N. 
Published: March 2008

Summary The movements of iron‐dextran, following a total dose infusion of up to 2550 mg. iron in six severely iron deficient patients, have been studied using a 59Fe tagged complex. The preparation is removed from the plasma in 8–10 days. Fifty per cent of the iron was incorporated into haemoglobin in 3–4 weeks and the iron stores replenished rapidly.Using organ scanning, the liver was confirmed as the site of primary plasma clearance, but accumulations of iron were also detected in the spleen and bone marrow. Only minute quantities of radioactivity appeared in the urine. No untoward effects on iron metabolism have been detected, but reduction of the serum iron and total iron binding capacity to low levels 6 weeks post infusion is of interest. An attempt has been made to interpret the findings to elucidate the ferrokinetics of this method of treatment.
Post‐Transfusion Survival of Red Cells Stored in Liquid Nitrogen
Authors: Morrison F. S. , Mollison P. L. , Robson D. C. 
Published: March 2008

Summary In 13 cases in which red cells were stored in a PVP‐ACD mixture at 4°C. for not more than 24 hours before being frozen in liquid nitrogen and were then stored in liquid nitrogen vapour (−150°C.) for periods up to 486 days, post‐transfusion 51 Cr survival at 24 hours varied from 57 to 83.5 per cent with a mean of 69.8 per cent. There was no evidence that the period of storage at −150°C. had any influence on survival. On the other hand, minor variations in the method of labelling the cells with 51 Cr did appear to affect the results; there was some evidence that repeated washing of the cells in PVP‐plasma had an unfavourable effect.In a further 14 cases in which red cells were stored in PVP‐plasma at 4°C. for up to 48 hours before freezing and for 1–32 days after thawing, evidence was obtained first, that storage for more than 24 hours before freezing was deleterious and, second, that storage for up to 12 days probably had no adverse effects and, thirdly, that storage for 15 days or more after thawing was very deleterious.In two experiments in which cells were labelled both with DF32P and with 51Cr it was found that Cr did not elute at an abnormal rate from red cells which had been frozen and thawed after being mixed with PVP. Accordingly, standard corrections for Cr elution may be applied and it may be concluded that the true 24‐hour survival of red cells frozen and thawed in liquid nitrogen averages about 73.5 per cent.
Occlusion of Plasma Proteins by Human Fibrin: Studies using Trace‐Labelled Proteins
Authors: Regoeczi E. 
Published: March 2008

Summary The occlusion of 13 different proteins by human fibrin has been investigated using radioactive techniques. Each of the proteins examined exhibited different tendencies to be retained by clots. For a satisfactory demonstration of this, studies of the effects of varying protein and fibrinogen concentration were required and are reported here.With normal plasma, the contamination of the syneresed and washed fibrins with albumin and transferrin was negligible whereas significant quantities of β‐lipoproteins, haptoglobin‐haemoglobin complex, free haemoglobin and IgG‐globulins were trapped. Substantial contaminations were noted with macroglobulins (IgA, IgM and α2‐macroglobulin), and above all with plasminogen, of which more than 50 per cent was adsorbed to the fibrin. Bence Jones proteins were also occluded by fibrin, Type K more so than Type L, whereas iodinated thrombin showed no affinity for fibrin.Some effects of clotting conditions on occlusion are also reported, and it is shown that in dilute plasmas occlusion is reduced and in covalently bound fibrin it is enhanced.
A Study of the Interdependence of Red Cell and Bone Marrow Stem Cell Populations
Authors: Orr J. S. , Kirk J. , Gray Kathleen G. , Anderson J. R. 
Published: March 2008

Haemolytic anaemia was produced in rabbits by the administration of a regular course of incompatible red cell iso‐antibody. A greatly increased rate of red cell production was observed but haemoglobin and reticulocyte counts exhibited vigorous and continuing fluctuations with, in the adult rabbits, a regular periodicity. The effects of steroids and splenectomy on this antibody‐mediated red cell destruction were found to be virtually unobservable, and the implications of this with regard to the haemolytic anaemias of man resulting from red cell destruction by iso‐ or auto‐antibodies are discussed.The significance of the fairly regular oscillations was analysed on the basis of evidence on the various parts of the haemopoietic system. The nature of these and other oscillations observed led to the suggestion that they could be understood in terms of interactions of a four‐component system: the stem cell population; a stem cell specific mitotic inhibitor, termed chalone, which is produced in proportion to the stem cell population and catabolized in proportion to its own level; the differentiated cell population; and erythropoietin. The features of the various oscillations in terms of the four‐component system, and the effects of the parameters on these features, are discussed.
Relationship between Membrane Function and Permeability II. THE INTERRELATIONSHIP OF COPPER ATTACHMENT, GLYCEROL TRANSPORT AND THROMBOPLASTIN AVAILABILITY IN THE INTACT HUMAN ERYTHROCYTE
Authors: Cohen Phin 
Published: March 2008

Glycerol in trace amounts appears to enhance exponentially the attachment of trace amounts of copper to human erythrocytes, while its own penetration of the erythrocytes is unaffected. At higher concentrations of glycerol its effect in enhancing copper attachment is abolished. At higher concentrations of copper, glycerol transport is delayed as shown by the PF‐3 availability test in which glycerol is used as the probing agent and the erythrocytes as the source of PF‐3 activity. The copper effect on the glycerol‐induced PF‐3 availability may be surmounted by a surfeit of glycerol.Using these findings, along with some assumptions based upon other data in the literature, a new theory interrelating glycerol and copper transport and thromboplastin availability in the intact erythrocyte has been presented.
Increased Agglutinability by Anti‐i of Red Cells in Sideroblastic and Megaloblastic Anaemia
Authors: Cooper A. G. , Hoffbrand A. V. , Worlledge S. M. 
Published: March 2008

Agglutinin titres have been performed using anti‐I and anti‐i cold antibodies on red cells from patients with sideroblastic, iron deficiency and megaloblastic anaemias, as well as on normal adult and cord blood red cells.Raised i antigen titres were found using the red cells of 13 of 15 patients with sideroblastic anaemia and of seven of eight patients with megaloblastic anaemia but in none of eight patients with iron deficiency anaemia and in one of 17 patients with megaloblastic anaemia in remission. Those patients who had an elevated i antigen titre usually also had an elevated I antigen titre.The raised i and I antigen titres of the red cells of a patient with pernicious anaemia were shown to decrease markedly within 3 months of the onset of B12 therapy, indicating that the alteration probably persists for the life‐span of the red cell but that the cause of the alteration is reversible once the underlying blood disorder is corrected.It is believed that disordered erythropoiesis results in membrane alterations which lead to the increased agglutinability, but the cause is not known. It does not appear that a shortened red cell maturation time is a necessary factor.
The Mechanism of Action of Human Placental Lactogen on Erythropoiesis
Authors: Jepson J. H. , Friesen H. G. 
Published: March 2008

A purified preparation of human placental lactogen (HPL) increased the incorporation of 59Fe into erythrocytes of polycythaemic mice, an effect which was abolished by its incubation with anti‐HPL but not with anti‐sheep erythropoietin (anti‐ESF).The erythropoietic‐stimulating capacity of HPL was abolished by increasing the endogenous concentration of anti‐ESF prior to injection of HPL into plethoric mice, indicating that some ESF was required for the effect of HPL to become apparent. The main action of HPL appeared to be through an augmentation of the erythropoietic effect of ESF in intact and hypophysectomized plethoric and nonplethoric mice through its specific and general anabolic action on erythropoiesis. A similar effect may occur during human pregnancy.
The Site of Abnormal Erythrocyte Destruction during Experimental Fever
Authors: Karle Hans 
Published: March 2008

Measurement of the 51Cr activity in the whole body, blood, and reticulo‐endothelium system organs in rabbits, exposed to a period of fever by injection of bacterial pyrogen after labelling of the erythrocytes, showed:(1) 51 Cr, liberated by normal or abnormal erythroclasis, is temporarily deposited in an extravascular site. Under normal conditions the spleen and bone marrow are the main organs where the deposition occurs.(2) The abnormal erythrocyte breakdown during fever immediately gives rise to an increased excretion of 51Cr in the urine. Simultaneously, there is a significant increase in the activity in the spleen. Five days after a 5‐day period of fever, about 40 per cent of the abnormal 51Cr loss from the blood was recovered in the spleen. There were no signs of intravascular haemolysis.(3) The presence of increased haemoglobin in the splenic tissue of the fever‐exposed rabbits and the absence of abnormal erythrocyte loss during pyrexia in splen‐ectomized rabbits indicate that the spleen plays a direct role in the abnormal loss. The pathogenesis of the cell loss is discussed.
Observation of Four Age‐Related Parameters in the Red Cells of Various Disease States
Authors: Sabine Jean C. , Kwok Lillian W. 
Published: March 2008

Simultaneous determinations of anaerobic glycolysis, hexokinase activity (HxK), glucose‐6‐phosphate dehydrogenase activity (G6PD) and acetyl‐cholinesterase activity (AChE) were made in the whole red cell populations of patients and of healthy subjects, and in the layers of the centrifuged cell packs of selected patients to give regression lines for each parameter plotted against each of the others. Apparently normal interrelationships were found in blood loss and in haemolytic disease of various origins.Abnormalities were revealed which would escape notice if a single parameter were assayed. These were relative, not absolute, deficiency of anaerobic glycolysis in a case of congenital nonspherocytic haemolytic disease with no deficiency of G6PD, HxK or pyruvate kinase; of HxK in a case of unexplained anaemia; and of AChE in a case of Coombs positive haemolytic disease.In a case of paroxysmal nocturnal haemoglobinuria AChE was severely deficient while glycolysis, HxK and G6PD were extraordinarily high and appeared normally interrelated. The patient had been transfused just before these studies, and calculations based on the enzyme data led to the hypothesis that in this case the donor cells were rapidly destroyed.Observations in normal blood showed that values for G6PD and AChE occur as two independent normal distributions. The same was true for glycolysis and AChE. Glycolysis and G6PD were correlated but did not approach a perfect functional relationship.There is evidence that high G6PD unrelated to red cell age occurs in malignant disease, but it is not conclusive.