bluuebunny/crossref_metadata_2025_split huggingface dataset:
An Immunoenzyme Histochemical Technique for the Detection of Platelet Antibodies from the Serum of Patients with Idiopathic (Autoimmune) Thrombocytopenic Purpura (ITP)
Authors: Tate D. Y. , Sorenson R. L. , Gerhard J. M. , White J. G. , Krivit W. 
Published: March 2008

SUMMARY.The present report describes a simple, reproducible, semi‐quantitative immunohistochemical assay for the detection of antiplatelet antibody. Evaluation of the technique in 10 children with active idiopathic (autoimmune) thrombocytopenic purpura (ITP), seven children with thrombocytopenia due to other causes, and 12 normal individuals revealed that the assay consistently and reliably distinguished children with ITP from the other groups. Individuals who had had multiple platelet transfusions and individuals with systemic lupus erythematosus (SLE) also had antiplatelet antibodies detectable using this technique although the levels were less than those of individuals with ITP. The method can be used effectively to monitor the course of ITP and the effects of therapy.
Platelet Autoimmunity
Authors: Chanarin I. , James D. , Stafford Denise , Tidmarsh Elizabeth 
Published: March 2008

SUMMARY.Autoimmunity to platelets was looked for by complement fixation, antiglobulin consumption, transformation and migration‐inhibition techniques. Only the migration‐inhibition method gave consistently positive results. The test was positive in nine out of 14 patients with idiopathic thrombocytopenic purpura and all five patients with glandular fever.
Immunologic Characterization and Quantification of Haemoglobin A1c
Authors: Javid Jamshid , Pettis Penelope K. , Koenig Ronald J. , Cerami Anthony 
Published: March 2008

Sheep antiserum developed against the human glycohaemoglobin, Hb A1c, distinguishes this fraction from the major component Hb A0. Partial cross reactivity is observed with Hb A1a and Hb A1b, as well as with analogous glycohaemoglobins from mouse and dog haemolysates. The reactivity of the haemoglobins with this specific antiserum is abolished by the reduction of the keto group of the sugar ligand.The immunological specificity displayed provides the basis of a quantitative assay for Hb A1c, suitable for studies of clinical and experimental diabetes.
Immune Complexes in Myelofibrosis: a Possible Guide to Management
Authors: Lewis C. M. , Pegrum G. D. 
Published: March 2008

Using a fluorescent technique intracellular immune complexes were found in the peripheral leucocytes in all of 12 patients with myelofibrosis studied. Fluorescent cells varied from 12% to 81%; with no relation to the total white count or any obvious clinical finding.Three of the patients with severe refractory anaemia benefited from immunosuppressive therapy.
Unmasking Covert Folate Deficiency in Iron‐Deficient Subjects with Neutrophil Hypersegmentation: dU Suppression Tests on Lymphocytes and Bone Marrow
Authors: Das Kshitish C. , Herbert Victor , Colman Neville , Longo Dan L. 
Published: March 2008

Fifteen patients with apparently uncomplicated iron‐deficiency anaemia were studied to determine the presence of covert folate or vitamin B12 deficiency. None had morphologic or conventional biochemical evidence of folate or vitamin B12 deficiency on admission, except for neutrophil hypersegmentation in peripheral blood smears of 10 of the 15 patients, and giant metamyelocytes in some of the bone marrow aspirates.Although deoxyuridine (dU) suppression tests on pretreatment bone marrow were normal in all 15 patients, similar tests on pretreatment transformed lymphocytes were characteristic of folate deficiency in eight of 14 patients (57%), with poor transformation obscuring results in the fifteenth subject.After six patients were given 6 weeks of oral folate and iron therapy, the dU suppression test was repeated in the lymphocytes of two subjects who demonstrated folate deficiency in this test on admission. The test still showed folate deficiency, supporting our prior report that the test was useful as a marker of past folate deficiency despite intervening folate therapy.The nine remaining subjects received iron alone for 3–6 weeks, which produced no change in serum folate, red cell folate, or dU suppression tests in lymphocytes. In five of the nine subjects (56%), however, an abnormality characteristic of folate deficiency appeared for the first time in bone marrow dU suppression tests, with morphologic megaloblastosis developing frankly in four and equivocally in one. Four of these five subjects had a folate corrected defect in the lymphocyte dU suppression test before treatment, and the fifth was the subject in whom lymphocytes did not transform sufficiently to allow testing for the defect.These observations: 1. Suggest that when a patient with apparently uncomplicated iron deficiency has hypersegmentation of his granulocyte nuclei, underlying folate deficiency should be sought. It was found in nine of the 10 current such subjects. 2. Suggest that the most sensitive test to detect covert folate deficiency in contrary to the situation in severe uncomplicated deficiency of vitamin B12 or folate, in which hypersegmentation is striking and rapid scanning of a hundred neutrophils yields a minimum of five with more than five lobes.In uncomplicated iron deficiency, iron binding capacity is almost always elevated above 400 μg/dl. Such levels were seen in only four of our 15 patients, two without hypersegmentation and two with hypersegmentation. This may suggest that a patient population with relatively low total iron binding capacity, perhaps often in association with liver disease, is more likely to have covert folate deficiency with their iron deficiency.
In Vivo and in Vitro Variations of Human Erythrocyte Glutathione Peroxidase Activity as Result of Cells Ageing, Selenium Availability and Peroxide Activation
Authors: Perona G. , Guidi G. C. , Piga A. , Cellerino R. , Menna R. , Zatti M. 
Published: March 2008

Cases showing erythrocyte glutathione peroxidase (GSH‐Px) defects have been previously described. Our experiments demonstrate that a number of non genetic factors may influence the GSH‐PX activity in human erythrocytes.Selenium administration in vivo was followed in four subjects by elevation in erythrocyte GSH‐Px activity ranging from 30% to 1400%. Selenium operates mainly in the bone marrow erythroblasts by facilitating the synthesis of active GSH‐Px molecules; experiments in vitro demonstrate that, in the youngest erythrocytes, selenium can raise the enzyme activity, but by a different mechanism. The reticulocyte GSH‐Px activity appears to depend on selenium availability and may vary over a wide range. In some normal and iron deficient subjects, the GSH‐Px activity in the youngest erythrocyte fraction was equal or lower than that previously found in whole erythrocytes of patients affected by haemolytic anaemia.During erythrocyte life, GSH‐Px activity may either diminish or increase, and these variations are inversely related to the initial GSH‐Px activity in the youngest cells. In vitro experiments with the addition of acetyl‐phenyl‐hydrazine strongly suggest that elevation of GSH‐Px activity may be due to allosteric enzyme activation by activated oxygen.
Destruction of IgG‐Sensitized Erythrocytes by Human Blood Monocytes: Modulation of Inhibition by IgG
Authors: Fleer A. , van der Meulen F. W. , Linthout Els , von dem Borne A. E. G. Kr. , Engelfriet C. P. 
Published: March 2008

The in vitro interaction between monocytes and erythrocytes sensitized with non‐complement binding IgG antibodies (i.e. the Rh antibody anti‐D:EAIgG anti‐D) is completely inhibited by low concentrations of IgG (e.g. 30–100 μg/ml). However, the interaction between monocytes and erythrocytes sensitized with IgG anti‐A (EAIgG anti‐A) is not inhibited by IgG. The findings presented in this paper indicate that this difference is probably due to the difference in the number of IgG antibody molecules per EAIgG. Thus, the higher the number of IgG antibody molecules per EAIgG, the less the interaction between EAIgG and monocytes is inhibited by IgG.A second factor which proved to have a strong influence on inhibition by IgG was the number of EAIgG per monocyte. When the number of EAIgG per monocyte was increased from 1 to 32, the percentage of inhibition by a fixed amount of IgG (50 μg/ml) decreased significantly. This in vitro effect is only evident when relatively weakly sensitized erythrocytes are used and, in vivo, destruction of these weakly sensitized red cells (e.g. EAIgG anti‐D) is confined to the spleen. Since a considerable haemoconcentration occurs in this organ, it is conceivable that a high EAIgG:macrophage ratio is accomplished. The latter data are an indication that this high ratio may allow interaction between weakly sensitized erythrocytes and splenic macrophages despite the presence, in vivo, of a high concentration of IgG, and that, in this way, in the spleen, the inhibitory effect of IgG is overcome.
Studies of Erythrocyte Membrane Loss Produced by Amphipathic Drugs and in Vitro Storage
Authors: Greenwalt Tibor J. , Lau Francis O. , Swierk Ellen M. , Williams Robert E. 
Published: March 2008

Four amphipathic drugs, primaquine, propranolol, chlorpromazine and tetracaine, were used to cause endocytosis in glucose‐depleted red cells, and the relative reduction of membrane surfaces was measured by the toluidine blue (TB) method. The TB measurements correlated well with the observed electron microscopic alterations. In vitro blood storage studies indicated loss of cell membrane during storage correlated with decreasing uptake of TB by the red cells. Regeneration of cellular ATP with adenosine did not always restore TB uptake by the cells.Loss of red blood cell membrane either by exocytosis or endocytosis may occur during normal in vivo or in vitro ageing, and may be increased in pathological states or by the action of drugs.
Release of β‐Thromboglobulin from Human Platelets by Therapeutic Intensities of Ultrasound
Authors: Williams A. R. , Chater B. V. , Allen K. A. , Sherwood M. R. , Sanderson J. H. 
Published: March 2008

SUMMARY The effects of therapeutic intensities of ultrasound on human platelets in whole blood were investigated by monitoring the release of the platelet specific protein β‐thromboglobulin (β‐TG). More β‐TG was released as the intensity of the ultrasound was increased and also as the driving frequency was decreased from 3.0 to 0.75 MHz. Some β‐TG was released at spatially‐averaged intensities as low as 0.6 W/cm2 at 0.75 MHz, a value significantly lower than that observed for the onset of aggregation of platelet rich plasma (obtained from the same volunteer) in the same exposure system.Liberation of β‐TG by ultrasound was diminished but not abolished in the presence of inhibitors which rendered the platelets functionally inert. Our data suggests that β‐TG is liberated in two ways, firstly as a result of platelet disruption by cavitation, and subsequently by potent aggregating agents, liberated in parallel with β‐TG, inducing the physiological release reaction in adjacent platelets.The low therapeutic intensities and short exposure times (30 s or less) necessary to liberate β‐TG from normal human platelets in vitro, suggests that patients with abnormally sensitive platelets and/or ‘hypercoagulable state’ could be at risk if subjected to high therapeutic intensities of ultrasound.
Cell Studies in Prolymphocytic Leukaemia
Authors: Singh A. K. , Vaughan‐Smith S. , Sawyer Barbara , O'Connor T. W. E. , Thomson A. E. R. , Wetherley‐Mein G. 
Published: March 2008

Summary. A case of prolymphocytic leukaemia, showing several features not yet reported in this disease, is reported.The majority of lymphocytes in the peripheral blood and bone marrow had markers of both B‐ and T‐lymphocytes. The simultaneous presence of receptors for sheep RBC and surface immunoglobulins on individual cells was demonstrated and the endogenous origin of these markers was established.The lymphocytes had some of the functional characteristics seen in chronic lymphocytic leukaemia (CLL). In vitro cell death in the presence of colchicine (colchicine ultrasensitivity) and polystyrene bead column retention were of the same order as seen in CLL. In contrast with the findings in CLL, these cells were markedly radioresistant in vitro.The dominant clinical features‐anaemia and constitutional symptoms‐appeared to be related to hypersplenism associated with massive splenomegaly. The relevance of these findings is discussed.
The Regulation of Erythropoiesis in Protein‐Energy‐Malnutrition
Authors: Fondu Pierre , And Per Hågå , Halvorsen Sverre 
Published: March 2008

SummaryThe erythropoietin (ESF) content of plasma and urine has been studied in children with protein‐energy‐malnutrition (PEM) living in the Kivu province at an altitude of 1467–2000 m.On admission, packed cell volume (PCV) was moderately reduced; after 2 months of refeeding PCV had increased but was still lower than in the controls. Plasma ESF was increased on admission and in patients refed for 2 months. The expected positive correlation between serum and urine ESF levels was found after refeeding but not on admission; the last finding could not be explained by the presence of erythropoiesis inhibiting factor(s) in the urine.In spite of the normal 2,3‐DPG and P50 previously described in PEM in Kivu, the anaemia associated with this disease is not merely an adaptation to lowered oxygen consumption and there must be some disturbances in the responsiveness of bone marrow to ESF. The high ESF values observed after 2 months of refeeding confirm that the restoration of the red cell volume proceeds slowly.
Effect of Antilymphocyte Globulin on Granulocyte Precursors in Aplastic Anaemia
Authors: Faille A. , Barrett A. J. , Balitrand N. , Ketels F. , Gluckman E. , Najean Y. 
Published: March 2008

Summary. Granylocyte colony forming units (CFU‐C) were studied in 22 patients with severe aplastic anaemia before and after treatment with antilymphocyte globulin (ALG). Nine patients showed a clinical response to ALG characterized by a rise in the granulocyte count to over 1 × 109/1 within 30 d. These patients were distinguished in vitro from non‐responders by an increase in CFU‐C numbers after incubation of bone marrow cells with ALG, and by the presence of inhibitors of normal CFU‐C in the serum in six out of seven patients tested. In responding patients bone marrow CFU‐C rose while most non‐responding patients showed no change or a fall in CFU‐C after treatment. In addition in three out of four responding patients examined serum inhibitors disappeared after treatment. The horse ALG used in this study also stimulated normal CFU‐C in vitro. This evidence is contrary to the hypothesis that ALG stimulates CFU‐C in aplasia by inactivating an abnormal suppressor lymphocyte population.The nature of the stimulation by ALG remains unclear. But in practice the effect of ALG on bone marrow cells and study of CFU‐C inhibitors in serum could be used to select patients likely to respond to ALG treatment.
Studies in Desferrioxamine and Ferrioxamine Metabolism in Normal and Iron‐Loaded Subjects
Authors: Summers M. R. , Jacobs A. , Tudway D. , Perera P. , Ricketts C. 
Published: March 2008

Summary. Plasma concentrations of desferrioxamine and ferrioxamine were measured following bolus injections of desferrioxamine and during 24 h infusions of the drug. [59Fe]ferrioxamine clearance and urinary iron excretion were also measured.Higher plasma ferrioxamine concentrations are found in iron loaded subjects and higher desferrioxamine concentrations in subjects with normal iron loads. There is a correlation between the circulating concentration of ferrioxamine during an infusion and the 48 h urinary iron excretion. The data suggests that the amount of iron chelated in vivo is related to an increase in the size of an intermediate chelatable pool rather than the total amount of the iron load. The well‐recognized delay in urinary iron excretion appears to be related to active tubular reabsorption of ferrioxamine.
Hereditary Acanthocytosis Associated with the McLeod Phenotype of the Kell Blood Group System
Authors: Symmans W. A. , Shepherd C. S. , Marsh W. L. , ØYen R. , Shohet S. B. , Linehan B. J. 
Published: March 2008

Summary. Some boys with X‐linked chronic granulomatous disease (CGD) have red cells of the rare McLeod phenotype in the Kell blood group system. Only one example of this phenotype has previously been described in a non‐CGD subject. We have studied a 10‐year‐old boy and a maternal brother who do not have CGD and whose red cells are of the McLeod type. The boy presented as a haematological problem with red‐cell abnormalities. These were acanthocytosis, anisocytosis and ‘tailing’ in the osmotic fragility curve, changes now known to occur with the McLeod phenotype. Subsequent studies revealed his rare blood group.A family study has established that an uncle also has acanthocytic red cells and the McLeod phenotype. In addition the boy's sister, mother and maternal grandmother all show red‐cell mosaicism with double populations of McLeod acanthocytes and normal red cells of common Kell type. The gene that determines inheritance of the McLeod phenotype is X‐linked and the mosaicism present in female carriers is believed to result from X chromosome inactivation by the Lyon effect. The study provides further evidence that the McLeod phenotype arises by inheritance of a variant X‐linked modifying gene and not through inheritance of a variant gene at the Kell autosomal locus. It also represents the first occasion that a person of rare blood group has been recognized because of an associated anomaly in red cell morphology.
The Ph1 Chromosome in Childhood Leukaemia
Authors: Chessells J. M. , Janossy G. , Lawler S. D. , Walker L. M. Secker 
Published: March 2008

Summary. Ph1 positive leukaemia was diagnosed in eight of 123 (6.5%) consecutive new cases of childhood leukaemia. Four patients presented as typical chronic granulocytic leukaemia (CGL) and four as acute leukaemia, two of the four children with CGL have since developed blast crisis. Morphological and immunological characterization of the blasts in these six acute cases was suggestive of a lymphoid crisis in three, a mixed crisis in two, and a myeloid crisis in one. Remission was achieved in five of the six patients but lasted more than a year in only two; in one of these two patients the blasts at the time of crisis lacked the Ph1 chromosome and in the other, who presented in myeloid crisis, remission ended in a lymphoid crisis. Cytogenetic studies showed unusual translocations in two of the eight children. Serial examination showed that in three children the proportion of Ph1 positive cells in the marrow was reduced to less than 6% at the time of remission from acute leukaemia.We conclude that Ph1 positive leukaemia in childhood presents frequently as acute leukaemia and may even mimic ‘good risk’ acute lymphoblastic leukaemia. The response to treatment cannot be predicted by morphological or immunological characterization of blast cells. Storage of bone marrow for possible autologous transplantation should be considered in children who achieve remission.Ormond Street, London WC1N3JH.
Serial Changes in Coagulation and Viscosity during Sickle‐Cell Crisis
Authors: Richardson S. G. N. , Matthews K. B. , Stuart J. , Geddes A. M. , Wilcox R. M. 
Published: March 2008

Summary. Coagulation activity and whole‐blood viscosity were measured in the steady state, and serially during painful crisis, in eight patients with sickle‐cell anaemia. Platelet and coagulation activation occurred in the steady state and became more pronounced early in crisis. Whole‐blood viscosity increased during crisis in parallel with plasma fibrinogen.Similar changes were found in a parallel study of 20 patients with localized bacterial or viral infection who did not have sickle‐cell anaemia. Reports of platelet activation, hypercoagulability, and hyperviscosity during painful crisis therefore reflect secondary changes arising from vascular stasis, precipitating infection, and an acute‐phase protein reaction. Although secondary, these changes may contribute to vascular occlusion by an additive effect in vessels already partially occluded by sickled cells.
Inherited Lack of Transcobalamin II in Serum and Megaloblastic Anaemia: a Further Patient
Authors: Burman J. F. , Mollin D. L. , Sourial N. A. , Sladden R. A. 
Published: March 2008

Summary. We have studied a patient, unrelated to the patients previously described, with inherited lack of the vitamin B12 binding protein Transcobalamin II. Severe haematological abnormalities were found within a few weeks of birth and responded to treatment with both vitamin B12 and folic acid. He was maintained in partial remission with such treatment until adolescence, except for a time in early childhood when folic acid alone was given and he suffered severe neurological deterioration. At the age of 18 years he was admitted to hospital because of convulsions; the deoxyuridine suppression test showed intracellular deficiency of B12 despite a normal serum B12 and normal haemoglobin concentration.His serum failed to promote the uptake of radioactive B12 by bone marrow cells, and analysis of serum B12 binding proteins demonstrated the lack of Transcobalamin II. Treatment with injections of 1000 μg of B12 three times weekly corrected the abnormality shown in the deoxyuridine suppression test; following this treatment, together with changes in anticonvulsive therapy, he remains healthy without occurrence of further convulsions, and is haematologically normal.
Effects of 5‐Fluorouracil on Mouse Bone Marrow
Authors: Radley J. M. , Scurfield G. 
Published: March 2008

Summary.5‐Fluorouracil (150 mg/kg) was injected intravenously into 11‐week‐old male mice. Its effects on marrow structure over the following 9 d were assessed using light microscopic examination of semi‐thin transverse sections of decalcified humeri.The extravascular compartment became markedly depleted of cells and decreased in size, reaching a minimum on day 5 post‐treatment. Proliferating haematopoietic cells disappeared within 2 d. Post‐replicative erythrocytic cells underwent extra‐vascular phagocytosis by resident macrophages, whilst mature granulocytes continued to pass into the circulation. Repopulation was underway by day 6, and re‐expansion of the extravascular compartment by day 7 when megakaryocytes, of larger than normal average size, occupied most of the extravascular compartment. Megakaryocyte dominance gave way over days 8 and 9 as other haematopoietic cells proliferated and the extravascular compartment continued to enlarge. The significance of the results is discussed in relation to marrow micro‐circulation, egress of haematopoietic cells into the circulation, and cell proliferation in the marrow. Endotoxin, administered 4 h after 5‐fluorouracil, accelerated depletion of the marrow and recovery of haematopoiesis by about 1 d.