bluuebunny/crossref_metadata_2025_split huggingface dataset:
Congenital Dyserythropoietic Anaemia (CDA) with Severe Gout, Rare Kell Phenotype and Erythrocyte, Granulocyte and Platelet Membrane Reduplication: a New Variant of CDA Type II
Authors: Lowenthal R. M. , Marsden K. A. , Dewar C. L. , Thompson G. R. 
Published: March 2008

Summary.A 43‐year‐old man with lifelong anaemia showed features which indicate him to have a previously undescribed variant of congenital dyserythropoietic anaemia (CDA), type II. The main clinical features—of which the first two are unique or very unusual in CDA—have been severe tophaceous gout, massive splenomegaly, gall stones necessitating cholecystectomy and haemosiderosis affecting the liver and probably the heart. At age 41 he sustained a spontaneous retinal detachment. In the peripheral blood there were large numbers of nucleated red blood cells and marked macrocytosis; otherwise the picture was typical of CDA type II. The bone marrow contained many bi‐ and multi‐nucleated erythrocyte precursors. There were increased levels of a number of red cell enzymes and a slightly raised level of HbF. Uncharacteristically, the red cells failed to lyse with acidified normal serum. The cells were strongly agglutinated by anti‐i and were of the rare Kpb‐negative phenotype. Plasma lipid analysis showed very low levels of cholesterol and vitamin E. Lipid peroxidation was markedly increased. Ultrastructural studies showed reduplication of the erythrocyte, granulocyte, and platelet cell membranes.
HLA Haplotypes and Long Survival in Childhood Acute Lymphoblastic Leukaemia Treated with Transfer Factor
Authors: Bruyère M. De , Cornu G. , Heremans‐Bracke T. , Malchaire J. , Sokal G. 
Published: March 2008

Summary.Association between HLA haplotypes and a long survival was investigated in 116 children with acute lymphoblastic leukaemia. It was found that patients with A2 B12 and/or A2 B40 haplotypes survived longer than patients without these two haplotypes. Since all children were treated with transfer factor obtained from their relatives, it is suggested that children possessing A2 B12 or A2 B40 haplotypes may respond better to this type of immunotherapy.
Lymphocyte, Granulocyte and Platelet Contamination of Blood Frozen by the Low‐Glycerol Liquid Nitrogen Technique
Authors: Amer K. A. , Pepper D. S. , Urbaniak S. J. 
Published: March 2008

Summary.The residual leucocyte and platelet content of blood frozen in 20% (v/v) glycerol was assessed by several methods. The final red cell suspension contained 6–12% of the original leucocytes in the form of intact lymphocytes, whilst intact polymorphonuclear cells and platelets could not be detected. The lymphocytes were nonviable as assessed by trypan blue dye exclusion but there was a low response to phytohaemagglutinin stimulation in culture.Using 125I cell‐surface labelling it was found that 39·5 ± 7% (SD) of the labelled lymphocyte surface material, 25·8 ± 6·6% of the granulocyte material and 2·6 ± 0·4% of the platelet surface membrane material remained in the final product after processing. The level of the remaining leucocyte derived material could be lowered by removing the buffy coat after the centrifugal wash steps and by passage through a 40 μan micropore filter; however, this was at the expense of a loss of approximately 25% of red cells.The intact lymphocytes which remained were non‐antigenic in vitro as assessed by the one‐way mixed lymphocyte reaction using the recovered cells as stimulators. However, when the antigen status of the frozen blood as a whole was studied in vivo by injection into laboratory animals, this blood stimulated lymphocytotoxic antibody production, although the levels of antibody were lower and appeared later than those induced by the infusion of a comparable volume of fresh blood. The antibody levels could be diminished further by passing the blood through a 40 μm micropore filter prior to injection.
An Agarose Plate Method for Detecting Alloantisera to Coagulant Factor IX and Factor IX Antigen
Authors: Reisner Howard M. , Strand Elizabeth A. , Chung Kuo‐San , Roberts Harold R. 
Published: March 2008

Summary.An agarose plate method for detecting human alloantibodies (inhibitors) to coagulant factor IX has been developed. The assay is based on the ability of such antisera to inhibit the coagulation of a mixture of haemophilia B plasma, normal plasma and platelet substitute in an agarose matrix. The agarose plate method was also adopted to measure levels of FIX antigen (IX:Ag) in plasma. Using this technique, 12 of 17 obligate carriers of haemophilia B demonstrated reduced levels of IX:Ag. Three of the five carriers with normal IX:Ag levels were members of kindred in which affected individuals had normal or near normal levels of IX:Ag.
Familial Haemostatic Defect Associated with Reduced Prothrombin Consumption
Authors: Parry D. H. , Giddings J. C. , Bloom A. L. 
Published: March 2008

Summary.Ten individuals from three unrelated families suffered from an unusual inherited haemorrhagic disorder. The defect was associated with reduced prothrombin conversion as shown by a grossly abnormal prothrombin consumption index. All known plasma coagulation factors were present in adequate concentrations but in the two families tested the results were consistent with a defect of the interaction between plasma and platelets or phospholipid. Some of the affected family members exhibited a mild bleeding tendency characterized by post‐operative or post‐partum haemorrhage but none suffered spontaneous bleeding. In therapeutic trials in one of the patients both plasma and platelet transfusions were needed in order to correct the abnormality. This finding together with in vitro and other in vivo studies indicated that the abnormality is associated with an inhibitor of the interaction between plasma and phospholipid during blood coagulation. The results suggest the possibility of an abnormality of the γ‐carboxyglutamic acid residues of one or more vitamin K dependent coagulation factors but immunological and electrophoretic studies have so far failed to detect this. The findings underline the importance of a test of prothrombin conversion in screening procedures for an otherwise unexplained bleeding disorder.
Evaluation and Modification of Whole Blood Filtration in the Measurement of Erythrocyte Deformability in Pregnancy and the Newborn
Authors: Buchan Peter C. 
Published: March 2008

Summary. SUMMARY. This study was designed to investigate the deformability of erythrocytes from pregnant women and full‐term newborn infants, with healthy female adults as a comparison, using a whole blood filtration method. Delay in measurement, white cells, haematocrit, plasma viscosity, temperature and pH all significantly affected the rate of whole blood filtration and could thereby obscure or exaggerate changes in erythrocyte deformability.A new method was developed which eliminated or minimized these sources of error and the study was completed. Fetal erythrocytes were found to be significantly less deformable than adult erythrocytes which were in turn found to be significantly less deformable than erythrocytes from pregnant women. These differences appear to be related to the varying plasma fibrinogen concentrations in the three groups of subjects. The significance of these findings in the special haemodynamic situations found in the neonate and during pregnancy are discussed.
Subclassification of Acute Lymphoblastic Leukaemia in Children: Analysis of the Reproducibility of Morphological Criteria and Prognostic Implications
Authors: Viana Marcos B. , Maurer Helen S. , Ferenc Christine 
Published: March 2008

Summary. Stained smears of aspirated bone marrow obtained at time of diagnosis from 223 children with acute leukaemia were reviewed independently by three observers in a double‐blind fashion in order to assess the reproducibility and clinical significance of the French—American—British Cooperative Group Classification. In 170 cases of acute lymphoblastic leukaemia (ALL), triple agreement of 69.4% was reached in the subclassification into L1, L2 and L3 types. The closest degree of agreement between two observers was 86.8%.In children with blasts classified as L1, 57 of 61 patients (93.4%) remained in haematological remission after 12 months, as compared with 14 of 20 (70%) in children whose blasts were typed as L2 morphology, a difference which was statistically significant (P < 0.05). There was no difference when these groups were compared after 24 or 36 months. Children older than 7 years had an increased incidence of L2 type (P < 0.05).We conclude that although there may be significant variation in individual interpretations of the criteria utilized for classification, blast morphology may nevertheless be a useful prognostic factor.
Phenotypic Characterization of a Virus Producing Line Established from a Patient with Leukaemia/Lymphoma: Comparison with T‐Lymphoblastic and Myeloblastic Lines
Authors: Karpas A. , Worman C. P. , Khalid G. , Neumann H. , Hayhoe F. G.J. , Newell D. , Stewart J. W. 
Published: March 2008

Summary. The properties have been studied of a haemic cell line established from a patient with longstanding non‐Hodgkin's lymphoma after transformation to acute leukaemia. The cells are EBNA, Fc, C3 and SmIg and la‐like antigen negative. The continuous in vitro proliferation and karyotype abnormalities suggest malignancy. The cells contain only the lymphoid type of alkaline phosphatase and a high level of terminal deoxynucleotidyl transferase and as they formed E‐rosettes during the first year in culture they may be of T‐cell lineage. Since the patient's leukaemic cells have not been studied it is impossible to know whether the cultured cells represent an in vitro proliferation of the patient's malignant cells or an outgrowth of a subpopulation of cells. In the cytoplasm of many cells inclusion bodies containing virus‐like particles, similar to those observed in fresh human leukaemia cells, were often seen. As this new virus is biologically active, infection of the cells by virus may explain their continuous proliferation in vitro.
A Comparison of Methods for the Study of Platelet Hyperfunction in Thrornboembolic Disorders
Authors: Chen Yao‐Chang , Wu Kenneth K. 
Published: March 2008

Summary.To gain insight into the interrelationship of various methods for the study of platelet hyperfunction, we compared plasma β‐thromboglobulin (β‐TG), circulating platelet aggregates (CPA), and ADP‐induced platelet aggregation in 119 patients with thromboembolic disorders and 28 control subjects. CPA correlated fairly well with β‐TG, suggesting that both methods measure similar in vivo platelet activation and aggregation. CPA had an excellent correlation with platelet survival half‐time. In contrast, there was a poor correlation between in vitro platelet aggregation and CPA or β‐TG. These results confirm the notion that both CPA and β‐TG procedures are suitable for determination of in vivo platelet activation whereas platelet aggregation technique has serious limitations to test in vivo platelet activity. In order to have an accurate assessment of platelet hyperfunction, a battery of tests are needed.
Patterns of Factor‐VIII Related Antigen on Crossed Immunoelectrophoresis and Large Pore Polyacrylamide Gel‐Crossed Immunoelectrophoresis in von Wilkbrand's Disease
Authors: Fukui H. , Mikami S. , Takase T. , Fujimura Y. , Nishino M. , Yoshioka A. 
Published: March 2008

Summary.Plasma samples from patients with various types of von Willebrand's disease were subdivided into six patterns according to the electrophoretic mobility and shape of VIIIR: Ag on crossed immunoelectrophoresis (CIE): pattern 1 no precipitation arc, pattern 2 normal mobility with low arc, pattern 3 intermediate mobility with low arc, pattern 4 faster anodal mobility with low arc, pattern 5 normal mobility with normal arc height, pattern 6 faster anodal mobility with normal arc height. Of 62 patients, 14 had pattern 1, 6 pattern 2, 16 pattern 3, 12 pattern 4, 9 pattern 5, and 5 pattern 6.Large pore polyacrylamide‐agarose gel‐crossed immunoelectrophoresis (PAAG‐CIE) of crude factor VIII fraction from cryoprecipitate revealed no arcs in patients with pattern 1, three arcs of reduced height in the patients with patterns 2 and 3, four arcs very similar to normal control in patients with pattern 5, and two arcs with fast anodal migration in the patients with patterns 4 and 6. Crude factor VIII fractions from normal cryosupernatant showed one low fast anodally migrating arc corresponding to the fourth arc of normal cryoprecipitate. No peak was seen in patients with pattern 1, and one low fast anodal arc similar to normal control was present in the patients with patterns 2, 3, 4, 5 and 6.
Binding and Release of Factor VIII/von Willebrand's Factor by Human Endothelial Cells
Authors: Wall Robert T. , Coums Richard B. , Harker Laurence A. , Striker Gary E. 
Published: March 2008

Summary.The uptake and release of factor VIII/von Willebrand's protein by cultured human umbilical vein endothelial cells have been examined using highly purified 125I‐factor VIII poswssing von Willebrand's factor activity. 125I‐factor VIII/vWF was taken up by the cells, reaching maximum binding within 4 h with a tl‐2 of binding of 15 min. Endothelial cell binding of 125I‐factor VIII/vWF reached saturation at a concentration of 1.5 mg/l. Binding was inhibited by coincubation of excess unlabelled factor VIII/vWF. Most of the cell‐associated radioactivity was released by treatment of the cells with trypsin. Internalization of bound protein was evidenced by the incorporation into the cells of radioactivity which could not be released by trypsin. Human vascular smooth muscle cells did not bind 125I‐factor VIII/vWF. Addition of 0.1 μm epinephrine to the 125I‐factor VIII/vWF labelled endothelial cultures induced the release of cell bound, protein‐associated radioactivity into the medium. Propranolol inhibited completely epinephrine‐induced release, whereas phenylephrine had no effect.Endothelial cells maintained in medium partially depleted of factor VIII/vWF by tricalcium citrate cellulose treatment of plasma did not release factor VIII antigen into the culture medium during subsequent incubation. Although [3H]proline was incorporated into proteins released by endothelial cells under these experimental conditions, specific incorporation of label into factor VIII/vWF antigen was not detectable by a sensitive solid‐phase immunoradiometric assay. We conclude that factor VIII/vWF binds to endothelial cells and that this cell‐bound protein is mobilized by epinephrine through beta‐adrenergic stimulation.
C‐Group Chromosome Abnormalities in Bone Marrow Cells of Three Children with Dyshaematopoiesis of Unknown Origin
Authors: Hagemeijer A. , Hählen K. , Smit E. M. E. , Zanen G. E. van 
Published: March 2008

Summary.Clinical and cytogenetic findings in three children with dyshaematopoiesis and bone marrow aneuploidy are described. Monosomy 7 was found in immature cells of one 10‐year‐old boy with myelofibrosis following a 3 years evolution of severe thrombocytopenia and anaemia. Trisomy 8 was found in 80% of the bone marrow metaphases of a 5 1/2year‐old girl with aplastic anaemia and Australia antigen positivity. During a 3 year observation period the number of cells with trisomy 8 regressed and eventually disappeared. Improvement of her clinical condition is present but still limited. Trisomy 8 was also found in all bone marrow cells of an 8‐year‐old girl with an undefined myeloproliferative disorder. Her disease was apparently related to collagen‐vascular disorders like periarteritis or other necrotizing angiitis and presented bvith periods of exacerbation and periods of chronic evolution. Periods of exacerbation were accompanied by excessive myeloid proliferation. Repeated bone marrow cytogenetic analysis during the acute and chronic phases showed trisomy 8 in all the metnphases analysed. During the last episode of acute illness, further clonal evolution was observed, characterized by a translocation (8; 17).
Binding of Serum Ferritin to Concanavalin A: Patients with Hornozygous β Thalassaemia and Transfusional Iron Overload
Authors: Worwoon M. , Cragg S.J. , Jacobs A. , McLaren C. , Rickeits C. , Economidou J. 
Published: March 2008

Summary.Serum ferritin concentrations have been measured in 124 patients with homozygous β thalassaemia who were between 2 and 21 years old, had received 11–504 units of blood but had not undergone splenectorny. There were highly significant correlations between serum ferritin concentration and both the amount of blood transfused and alanine amino‐transferase (ALT) activity. However, multivariate analysis showed that units of blood and ALT activity together only accounted for about 30% of the variation in serum ferritin concentration. Little of the remaining variation could be explained by other variables related to iron nietabolism or liver damage. The concentration of concanavalin A binding ferritin increased rapidly with the number of units of blood up to 100 units but thereafter showed no further increase with number of transfusions. The concentration of non‐binding ferritin was more closely related to transfusion load. These results suggest that the secretion of glycosylated ferritin from rcticuloendothelial cells reaches a maximum with increasing iron accumulation, perhaps reflecting a maximum rate of synthesis. Ferritinaemia in patients with transfusional iron overload therefore seems to be the result of the combined effects of increased ferritin synthesis and the release of intracellular ferritin from damaged cells. A simple relationship between serum ferritin and iron stores cannot be assumed when ferritin concentrations exceed 4000 μg/l or in patients who have received more than 100 units of transfused blood.
Management of Relative Polycythaemia: Studies of Cerebral Blood Flow and Viscosity
Authors: Humphrey P. R. D. , Michael J. , Pearson T. C. 
Published: March 2008

Summary.Cerebral blood flow has been shown to be reduced in a group of patients with high packed cell volume due to a reduced plasma volume (relative polycythaemia). 500 ml of intravenous Dextran 40 resulted in a significant fall in PCV and whole blood viscosity with a concomitant rise in cerebral blood flow (CBF). This effect, however, lasted less than 24 h. Oral fludrocortisone therapy (0.2 mg daily) resulted in no definite change in either PCV, viscosity or CBF. Venesection brought about a significant fall in PCV and whole blood viscosity with a significant rise in CBF. Of the methods used, venesection was the only practical long‐term method of lowering PCV in this group of patients.
ADCC (K‐Cell) Lysis of Human Erythrocytes Sensitized with Rhesus Alloantibodies III. COMPARISON OF IgG ANTI‐D AGGLUTINATING AND LYTIC (ADCC) ACTIVITY AND THE ROLE OF IgG SUBCLASSES
Authors: Urbaniak S. J. , Greiss M. Ayoub 
Published: March 2008

Summary.Conventional manual (enzyme and antiglobulin titres) and Auto‐Analyser quantitation of anti‐D sera were compared with the ability of the same sera to mediate lysis of Rh(D) positive red cells in an ADCC assay. Auto Analyser (AA) quantitation correlated significantly with manual titration methods, although there were wide discrepancies between individual sera. The ADCC activity of the anti‐D sera did not correlate with any of the conventional assays (AA, enzyme, antiglobulin). There were significant differences between anti‐D sera obtained from females immunized during pregnancy and male volunteers immunized by deliberate injection. The female sera contained significantly less anti‐D when assessed by AA, yet were significantly more active in ADCC activity at equivalent concentrations. These functional differences in anti‐D activity could not be attributed to the absence of IgG subclasses (IgG1 and IgG3) known to induce ADCC. However, there was a relationship between ADCC and IgGI/IgG3 titres in that high ADCC activity was seen where the IgG3 titre was higher than the IgG1 titre. In the male anti‐D sera IgGl titres were ≥ IgG3 titres and ADCC activity was very low.
Drug Dependent Red Cell Antibodies and Intravascular Haemolysis Occurring in Patients Treated with 9 Hydroxy‐Methyl‐Ellipticinium
Authors: Criel A. M. , Hidajat M. , Clarysse A. , Verwilghen R. L. 
Published: March 2008

Summary.Eleven patients were treated weekly with a new cytostatic drug, 9 hydroxy‐methyl‐ellipticinium (9 HME). Eight were treated for longer than 4 weeks and three of these developed a drug dependent antibody reacting with normal red cells. In two of these patients acute intravascular haemolysis occurred, one with oliguric renal failure; in the third patient the drug was stopped as soon as the antibody was detected. In all three patients the antibody developed after 4 weeks of treatment. It was IgM, agglutinated normal red cells and bound complement only in the presence of the drug. No antibodies could be detected in the patient's serum reacting with normal platelets in the presence of the drug. The incidence of haemolysis with this drug is much higher than seen with other drugs causing immune‐complex haemolysis. Studies done with closely related substances suggest that the antigenic site of the drug is related to the group attached to carbon atom 9.
Globin Chain Synthesis in Haemoglobin New York (β 113 Valine→Glutamic acid
Authors: Todd D. , Chan Vivian , Schneider Rose G. , Dozy Andree M. , Kan Y. W. , Chan T. K. 
Published: March 2008

Summary.The presence of Hb New York was confirmed in a Chinese family in which affected members have occasional red cells with Hb H‐like inclusions and a relative decrease in α chain synthesis, suggestive of a coexisting α thalassaemia trait. However, globin gene mapping and DNA hybridization revealed no deletion of the α genome. Timed‐incubation experiments showed that the rate of synthesis of βNY chain was greater than that of normal β chain in the early periods. Chromatographic separation of Hb NY and Hb A before chain analysis revealed preferential binding of newly synthesized α chains to βNY, with a four‐fold increase in specific activity of the αHbny chains. It is concluded that βNY chain is being synthesized more rapidly and its increased turnover may account for this presentation of apparent a chain deficiency.
The Physiopathological Significance of Benign Monoclonal Gammopathy: a Study of 64 Cases
Authors: Carter Anna , Tatarsky Ilana 
Published: March 2008

Summary.Sixty‐four patients with monoclonal protein in serum but initially without evidence of multiple myeloma, macroglobulinaemia, amyloidosis or lymphoma, were studied. Fifty patients (78%) were observed for a period exceeding 3 years. Based on the follow‐up data the patients were classified into the following four groups: Group 1=patients with transient monoclonal gammopathy: 4.7%; Group 2 = patients without significant increase in monoclonal serum protein: 75%; Group 3 = patients with more than 50% increase in monoclonal serum protein: 14.1%; Group 4=patients in whom multiple myeloma developed: 6.2%. The mean interval from discovery of the serum monoclonal protein to evolution to multiple myeloma was 61 months. Retrospective analysis of age, sex, blood count, bone marrow picture, antigenic type and size of serum monoclonal proteins, presence of small amounts of homogeneous light chain in the urine, serum albumin level, levels of residual immunoglobulins, did not help to distinguish initially the patients in whom the monoclonal gammopathy evolved to multiple myeloma from patients in whom the disease remained benign and stable. The evolution to multiple myeloma had occurred abruptly after long periods of stable condition; and until this progression the follow‐up data were similar to the patients with benign disease. The possible physiopathology of occurrence and evolution of benign monoclonal gammopathy is discussed.