bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pathophysiology of Aplastic Anaemia
Authors: Heimpel H. , Kubanek B. 
Published: March 2008

Summary.Aplastic anaemia is the clinical expression of reduced influx into the compartments of morphologically identifiable proliferating and maturing haemo‐poietic cells from the stem cell compartment. The bulk of evidence, especially the results of bone marrow transplantation, favours a primary alteration of the proliferation and/or differentiation of pluripotent haemopoietic stem cells. Experimental and clinical data suggest that a stem cell compartment which is reduced in size may compensate its output by adaptation of kinetics for a limited period of time; this could explain the well‐known time lag between exposure to potentially harmful drugs or viruses and manifestation of aplastic anaemia. Humoral regulating factors, as far as have been examined, show normal reactivity. A primary alteration of bone marrow stroma is less likely. However, the basic mechanism of aplastic anaemia may be heterogenous and some cases may be due to alteration in the micro‐environment of haemopoietic cells. In many, and maybe in all cases, haemopoietic insufficiency is induced by external factors on the basis of individual hypersensitivity. Chemical, physical and viral factors are known. Direct toxicity of these factors on stem cells is more likely than mediation through immune mechanisms.
Antenatal Diagnosis of the Haemoglobinopathies
Authors: Nathan David G. , Alter Blanche P. 
Published: March 2008

Summary.The β‐chain of adult haemoglobin is expressed in fetal red cells during the first to mid‐trimester of human pregnancy. The rate of β‐chain production is approximately 8% of γ‐chain production during this period of gestation. The production of βs‐chains is readily detectable and the reduction of βA‐chain production in thalassaemia may also be observed.Acquisition of fetal cells by means of placentocentesis or placental venipuncture via a fetoscope are current sampling methods which have been shown to be useful. Thus far five attempts at such antenatal diagnosis have been made, four for β‐thalassaemia and one for sickle cell anaemia. In three of these attempts (all for β‐thalassaemia), a sample of fetal blood was obtained. In one, the correct diagnosis of a normal fetus was made. In a second fetus, the diagnosis of β‐thalassaemia trait was made; the infant was normal. In a third, the diagnosis of a normal fetus was made; the infant had β‐thalassaemia trait.These early results indicate that attempts at antenatal diagnosis of the common β‐chain abnormalities do not necessarily disturb pregnancy and can yield important information which encourages mothers at risk to retain pregnancies.
The Histopathology of Malignant Lymphoma
Authors: Lennert K. , Mohri N. , Stein H. , Kaiserling E. 
Published: March 2008

Summary.It has been possible to reinterpret non‐Hodgkin's lymphomas by applying subtle histological, cytological, and cytochemical techniques and electron microscopy as well as by using modern immunological methods and immune chemical analyses. This has led to the differentiation of four main groups of low‐grade and three main groups of high‐grade malignant lymphomas. Most non‐Hodgkin's lymphomas are derived from the B‐lymphocyte system. Only some of the lymphocytic, a small fraction of the lymphoblastic, and very few immunoblastic lymphomas originate from the T‐lymphocyte system. True reticulosarcomas are very rare and will have to be redefined.
Physiological Experiments in Haemostasis and Thrombosis
Authors: Tschopp Th. B. , Baumgartner H. R. 
Published: March 2008

Summary.An in vitro perfusion system has been developed which allows morphometric measurement of platelet adhesion and surface‐induced aggregation after exposure of subendothelium and other surfaces to flowing blood. Recent results are briefly reviewed and compared with results obtained using other methods.A number of rheologic and blood‐borne factors including flow velocity, vessel geometry, presence of red blood cells and divalent cations, affect both platelet adhesion and surface‐induced aggregation.Diminished adhesion of platelets to subendothelium of rabbit aorta but normal surface‐induced aggregation was found after exposure to blood of patients with von Willebrand's disease and the Bernard‐Soulier syndrome at blood flow rates similar to those found in arteries. In contrast, surface‐induced aggregation was defective in patients with thrombasthenia and in an inbred strain of rats with a haemorrhagic diathesis, similar to the platelet ‘storage pool disease’in patients. Similarly, normal adhesion and strikingly diminished aggregation were found with blood of rabbits after aspirin ingestion. These findings may explain the defective haemostasis in the patients and animals, and suggest a new way to study platelet function in a physiological system.
The Use of Factor IX Concentrates for Patients with Conditions other than Factor IX Deficiency
Authors: Ménaché Doris , Gunllin Marie Claude 
Published: March 2008

Summary.The increasing availability of factor IX concentrates have extended their use in conditions other than factor IX deficiency and especially in combined acquired deficiencies of vitamin K dependent clotting factors.The occurrence of complications such as post‐transfusion hepatitis type B and thromboembolic episodes have prompted the International Committee on Thrombosis and Hemostasis to constitute a Task Force on the clinical use of factor IX concentrates.The various concentrates available and the basis for their method of preparation are listed. Their use in various pathological conditions other than factor IX deficiency and also the occurrence of complications in such conditions are reviewed.The conclusions and the recommendations on the indications and contraindications formulated by the Task Force on the clinical use of factor IX concentrates as issued following the 1974 Basel Meeting are summarized.
Kinetic Studies of 51Cr and DF32P Labelled Granulocytes
Authors: Dresch C. , Najean Y. , Bauchet J. 
Published: March 2008

Summary. In vivo kinetic studies of granulocytes labelled in vitro with 51 Cr and DF32P were carried out in nine haematologically normal subjects by isolation of the cells in the blood samples by the Ficoll‐Isopaque flotation method. 51 Cr and 32P specific activity of blood samples made of 93–98% granulocytes was studied. Distribution between marginated and circulating granulocyte pools was identical for both labelled cells and the marginated pool was similar to the circulating pool, except that it was lower in one subject who had a previous splenectomy.The half‐disappearance time (T 1/2) was 16.1 ± 2.2 h for 51 Cr‐labelled and 5.4±2.1 hr for DF32P‐labelled granulocytes. In one case of a normal subject who previously received multiple transfusion homologous 51 Cr‐labelled granulocytes had a T1/2 of less than I h.
The Subcellular Localization of the Erythrostimulant Factors Present in the Kidney and Spleen of the Rabbit
Authors: Giovannini Elvio , Rosi Gabriella 
Published: March 2008

Summary. The subcellular localization of erythrostimulant factors contained in the kidney and spleen of the rabbit have been studied. This was done by subjecting homogenates to fractionation by centrifugation and assessing the erythrostimulant capacity of the hypotonic extracts prepared from the various fractions thus obtained.The results of these studies show that the erythrostimulant factors are present in the light mitochondrial and microsomal fractions of the normal kidney. In conditions of anaemia they are to be found not only in the above‐mentioned fractions but also in the mitochondrial fraction. By contrast, in the spleen such factors are present only in the mitochondrial fraction.It is likely that the different localization of the erythrostimulant factors in the kidney and in the spleen of the rabbit may be closely connected with the different functions performed by these organs in the humoral regulation of erythropoiesis.
Blood Leucocyte Enzymes
Authors: Brok‐Simoni F. , Ashkenazi Y. E. , Holtzman. F. , Ramot B. 
Published: March 2008

SUMMARY.The activities of glucose‐6‐phosphate dehydrogenase (D‐glucose‐6‐phosphate: NADP oxidoreductase, G6PD), 6‐phosphogluconatc dehydrogenase (6‐phospho‐D‐gluconate: NADP oxidoreductase, 6PGD), hexokinase (ATP: D‐hexose 6‐phosphotransferase, Hx), lactate dehydrogenase (D‐lactate: NAD oxidoreductase, LDH). glutamate oxaloacetate transaminase (L‐aspartatc: 2 oxoglutarate aminotransferase, GOT) and dihydrofolatc reductase (DHFR) were measured at 8 a.m. in leucocytes of healthy individuals and patients with chronic myeloid leukaemia (CML), chronic lymphatic leukaemia (CLL), myelofibrosis with myeloid metaplasia and polycythaemia vera. In view of the heterogeneity of the leucocyte populations in these conditions, the enzyme activities were correlated to the number of immature cells in CML and to the percentage of lymphocytes in CLL. No differences in the enzyme activities were found between the white cells of healthy individuals, myelofibrosis with myeloid metaplasia and polycythaemia vera. In CML the activities of all enzymes except GOT correlated directly with the number of immature cells; an inverse correlation with the number of lymphocytes was observed in CLL. GOT was the only enzyme whose activity correlated with the number of lymphocytes in the cell suspension. Furthermore, a significantly higher activity of this enzyme was found in Ficoll‐isolated CLL lymphocytes as compared to normal lymphocytes.
Thymoma Associated with Pure Red Cell Aplasia, Immunoglobulin Deficiency and an Inhibitor of Antigen‐induced Lymphocyte Transformation
Authors: Geary C. G. , Byron P. R. , Taylor G. , Maciver J. E. , Zervas J. 
Published: March 2008

Summary. A case of thymoma associated with pure red cell aplasia and hypogammaglobulinemia is described in which the anaemia was of abrupt onset, following removal of the tumour. Tests of immunological function showed abnormalities of both humoral and cellular immunity. The patient was found to have a serum inhibitor of antigen‐induced lymphocyte transformation which disappeared after immunosuppressive therapy at the same time that erythroblasts reappeared in the marrow.It is suggested that the triad of thymoma, pure red cell aplasia and immunoglobulin deficiency are manifestations of ‘pluripotent’ stem cell failure; in this case the inhibitor of lymphocyte transformation may have been related to the factor which also inhibited red cell maturation.
Anti‐Wrb, and Other Autoantibodies Responsible for Positive Direct Antiglobulin Tests in 150 Individuals
Authors: Issitt P. D. , Pavone B. G. , Goldfinger D. , Zwicker H. , Issitt C. H. , Tessel J. A. , Kroovand S. W. , Bell C. A. 
Published: March 2008

SUMMARY Eluates from the red blood cells (and sera whenever free autoantibody was present) of 150 individuals with positive direct antiglobulin tests, have been studied for antibody specificity. Of 87 patients with AIHA, 64 had autoantibodies reacting with all red cell samples including Rhnull. Of these 64 anti‐dl autoantibodies, two were, and 32 contained, auto‐anti‐Wrb. Of 33 patients being treated with alphamethyldopa, who had developed positive direct antiglobulin tests, 23 had anti‐dl autoantibodies four of which contained auto‐anti‐Wrb. Of 30 haematologically normal donors with positive direct antiglobulin tests, 23 had anti‐dl autoantibodies, two of which were, and six of which contained, auto‐anti‐Wrb. The full specificities of autoantibodies, other than anti‐Wrb and anti‐dl, in the 150 patients are described, as are the natures of the protein red cell coatings that caused the positive direct antiglobulin tests. The presence of free serum autoantibody as a correlate of the three clinical conditions is reported.Several observations on auto‐anti‐Wrb are documented. The antibody can cause gross red cell destruction in vivo, but can be benign on other occasions; it occurs with approximately the same frequency in AIHA patients and ‘normal’donors with positive direct antiglobulin tests, but in fewer patients with alpha‐methyldopa induced positive direct antiglobulin tests; it does not activate complement in vivo;, and finally it may eventually provide a clue to the aetiology of AIHA.
Evidence that Collagen Releases Human Platelet Constituents by Two Different Mechanisms
Authors: AKBAR HUZOOR , Ardlie Neville G. 
Published: March 2008

SUMMARYCollagen is believed to be involved in the initial events in haemostasis and has been shown by others to cause platelet aggregation and release, and also to initiate the intrinsic pathway of coagulation. The present experiments provide evidence which suggests how these many effects of collagen may be involved in haemostasis.It is shown here that collagen releases platelet constituents by two different pathways. Collagen causes platelets washed free of loosely adsorbed coagulation factors to release constituents. This activity is, therefore, independent of the intrinsic pathway of coagulation, and is not inhibited by heparin or hirudin. Collagen also releases platelet constituents by an alternative pathway which is inhibited by heparin and hirudin and is independent of factor XII, but is dependent on factor XI, subsequent factors in the intrinsic pathway of coagulation and calcium. These results suggest that collagen‐induced release of platelet constituents is in part due to a direct effect on the platelet, and, in part, to an indirect effect involving coagulation factors and mediated by thrombin. The present results suggest that irreversible aggregation by collagen is also mediated by thrombin.The possible significance of this dual action of collagen in the haemostatic process is shown in Fig 7.
Blast Crisis of Chronic Myeloid Leukaemia (CML): I. PRESENTATION SIMULATING ACUTE LYMPHOID LEUKAEMIA (ALL)
Authors: Beard M. E. J. , Durrant Jill , Catovsky D. , Wiltshaw E. , Amess J. L. , Brearley R. L. , Kirk Barbara , Wrigley P. F. M. , Janossy G. , Greaves M. F. , Galton D. A. G. 
Published: March 2008

SUMMARY. Seven patients presenting as an acute leukaemia caused difficulty in diagnosis. The lymphoid appearance of the blast cells either initially or during treatment suggested acute lymphoid leukaemia (ALL). In each case the Philadelphia chromosome was shown to be present thus suggesting that these cases were examples of chronic myeloid leukaemia (CML) presenting in blast crisis without a detectable chronic phase.The implications of these findings are discussed and the difficulty in achieving a precise diagnosis in the acute leukaemias is emphasized. Cytogenetic analysis should be carried out whenever the type of acute leukaemia present is of critical importance.
Alpha‐Thalassaemia Trait in Various Racial Groups in the United Kingdom: Characterization of a Variant of Alpha‐Thalassaemia in Indians*
Authors: Walford Diana M. , Deacon Rosemary 
Published: March 2008

SUMMARY. Patients whose red‐cell indices are suggestive of thalassaemia trait, but who have a normal haemoglobin electrophoretic pattern, may be carriers of αthalas‐saemia. A diagnosis of αthalassaemia trait was made in 44 such patients, using the incorporation of [3H]leucine by reticulocytes to measure the relative rates of synthesis of the α and β‐chains of adult haemoglobin. Patients with αthalassaemia trait had a reduced rate of synthesis of the αchains, with a mean α/β specific activity ratio of 0.79 ± SD 0.07. The mean α/β specific activity ratio of 20 control subjects was 1.06 ± SD 0.08. The diagnostic value of the haemoglobin H (Hb H) preparation was assessed in proven αthalassaemia heterozygotes of various races. A high proportion of ‘false negative’results in Indian and Negro heterozygotes indicated that the Hb H preparation is a highly unreliable screening test for use in a multi‐racial population. There was no significant difference in the mean level of Hb A2 in αthalassaemia heterozygotes (2.0 ± SD 0.6) compared with that of the control group (2.1 ± SD 0.5). Comparison of data from patients with α and β‐thalassaemia traits showed that αthalassaemia trait is the milder disorder, in terms of its effects on red‐cell morphology, red‐cell indices and degree of globin chain imbalance. Amongst individual patients with αthalassaemia trait, there was no correlation between the α/β specific activity ratio and the red‐blood‐cell (RBC) count, mean corpuscular volume (MCV) and mean corpuscular haemoglobin (MCH). This suggests that the α/β ratio cannot be used to distinguish between carriers of a mild gene (‘silent carriers’) and carriers of a more severe disease.This is the first study to characterize αthalassaemia trait in Indians, in whom it appears to be a common disorder. Haematologically, αthalassaemia trait in Indians is milder than that seen in Chinese and the fact that haemoglobin Bart's hydrops fetalis does not occur in Indians makes it likely that the genetics of Indian αthalassaemia differ from those of the Chinese disease. A possible genetic model for Indian αthalassaemia is discussed and the identification of the homozygote is seen as the first step in the determination of the underlying molecular defect.
Factor VIII and Human Platelet Aggregation
Authors: Bottecchia D. , Vermylen J. 
Published: March 2008

SUMMARY Bovine factor VIII aggregates human platelets either in a strong single wave at high concentration (10 ug/ml platelet suspension) or in two waves at low wave at low concentration 2–1 ug/ml). The strong single wave of aggregation is not associated of [14C]serotonin or β‐glucuronidase; the high concentration retraction of reptilase‐clotted platelet‐rich plasma. With the low release of [14C]serotonin is observed just prior to the onset of the aggregation; release of β‐glucuronidase does not occur at any low concentration of bovine factor VIII induces moderate retraction of platelet‐rich plasma, which is inhibited by acetylsalicylic acid, in and apyrase, indicating that it is a consequence of release of platelet phosphate.It has previusly been suggested that carbohydrate groups are involved in the bovine factor VIII interaction, since galactose oxidase and periodate the platelet aggregating activity of bovine factor VIII. The present these oxidizing substances also induce a degradation of bovine the exact role of carbohydrate groups in the aggregation process established.
Physiology and Ultrastructure of the Blood Platelet following Exposure to Hydrogen Peroxide
Authors: Rodvien Robert , Lindon Jack N. , Levine Peter H. 
Published: March 2008

Summary.Hydrogen peroxide in micromolar concentrations can induce shape change in human blood platelets, and can modify the aggregation and release reaction of these cells as induced by ADP or thrombin. In larger (millimolar) concentrations, H2O2 produces fusion of platelets with distortions in platelet morphology unlike those normally caused by aggregating agents. The production of H2O2in vivo by granulocytes or other cells could influence the processes of haemostasis or thrombosis.
Prophylaxis in Haemophilia: A Double‐blind Controlled Trial
Authors: Aronstam A. , Arblaster P. G. , Rainsford S. G. , Turk P. , Slattery M. , Alderson M. R. , Hall D. E. , Kirk P. J. 
Published: March 2008

Summary.A double‐blind controlled trial of prophylactic factor VIII therapy has been carried out on nine severe haemophiliacs at the Lord Mayor Treloar College. Infusions were given once weekly and calculated to give a post‐infusion plasma concentration of at least 0.25 I.U./ml of factor VIII. This regime reduced the overall bleeding frequency by 15%. The bleeding frequency in the first 3 days post‐infusion was reduced by 66%. A moderate overall reduction in morbidity was also achieved. It is calculated that to reduce the incidence of bleeding in severe haemophiliacs by 15% would require a 73% increased usage of therapeutic materials. More than twice this amount of material is likely to be needed to reduce the bleeding frequency of the same group by 66%.
Lymphocyte Response to Blood Transfusion in Man: a Comparison of Different Preparations of Blood
Authors: Hutchinson R. M. , Sejeny S. A. , Fraser I. D. , Tovey G. H. 
Published: March 2008

Summary.The appearance of atypical lymphocytes in post‐transfusion blood, their incorporation of tritiated thymidine in tissue culture and the elimination of cytotoxic antibody production, have been used as markers to show that frozen red cells are the least immunogenic when compared with dextran sedimented blood and whole blood donations. The absence of atypical lymphocytes and failure to produce lympho‐cytotoxic antibodies after transfusion of frozen cells is highly significant (P<0.001) when compared with whole blood donations.
Detection of Monocyte Specific Antigen on Human Acute Leukaemia Cells
Authors: Baker M. A. , Falk R. E. , Falk J. , Greaves M. F. 
Published: March 2008

Summary.Heteroantisera with specificity for human monocyte and macrophage antigens (HuMa) have been tested for activity against peripheral blood cells from patients with acute leukaemia. Using indirect immunofluorescence, blast cells from 25/27 patients with acute myelomonocytic leukaemia were reactive with antiHuMa antisera, whereas blasts from 19/20 patients with acute myeloblastic leukaemia and 13/13 patients with acute lymphoblastic leukaemia were not reactive. The F(ab1)2 fragment of the antibody retained specific activity. Activity of whole serum against myelomonocytic blast cells was specifically removed by absorption with macrophages. These observations indicate that circulating blast cells from patients with acute myelomonocytic leukaemia may carry differentiation antigens characteristic of monocytes and macrophages.
The Use of a Single Venous Blood Sample to Assess Oxygen Binding to Haemoglobin
Authors: Lichtman Marshall A. , Murphy Marion , Pogal Meredith 
Published: March 2008

Summary.The measurement of pH, Po2, Pco2 and So2 in a single venous blood sample can be used to determine the P50 at standard or at in vivo conditions. This technique makes it feasible for a physician, firstly, to make an assessment of the net adaptation of the red cell to reductions in blood oxygen content or flow and, secondly, to make an initial assessment of whether a haemoglobin with altered affinity for oxygen is present in subjects with polycythaemia or anaemia.
Induction of Haemolytic Anaemia by Substituted Phenylhydrazines
Authors: Itano H. A. , Hosokawa K. , Hirota K. 
Published: March 2008

Summary.The induction of anaemia and reticulocytosis by arylhydrazines was influenced by substituents on the benzene ring of phenylhydrazine. Arylhydrazines with ortho substituents, which would hinder the binding by haemoglobin of a ligand derived from the arylhydrazine, resulted in the least anaemia and reticulocytosis. These results are consistent with a previous finding that hydrazinobenzoic acid, which did not produce a ferrihaemochrome from ferrihaemoglobin, did not induce anaemia. The parallelism between the formation of a ferrihaemoglobin compound and the induction of anaemia supports the hypothesis that destabilization of haemoglobin by the binding of a ligand derived from the aryl portion of an arylhydrazine is an essential step in arylhydrazine‐induced haemolytic anaemia.