bluuebunny/crossref_metadata_2025_split huggingface dataset:
Haemolytic Disease of the Newborn and Chronic Anaemia Induced by γβ Thalassaemia in a Dutch Family
Authors: Oort M. , Heerspink W. , Roos D. , Flavell R. A. , Bernini L. F. 
Published: March 2008

Summary. Severe haemolytic anaemia with hyperbilirubinaemia and erythroblastaemia was observed in nine newborn infants belonging to one large family. One infant was still‐born, two died shortly after birth, five recovered after receiving one or more exchange transfusions and one improved without transfusional therapy. In four out of six newborns whose bilirubin levels were determined, a mixed hyperbilirubinaemia was found with high concentrations of both free and conjugated bilirubin. At the end of the first year of life the surviving children, although in satisfactory general condition, showed a mild microcytic hypochromic anaemia with decreased red cell osmotic fragility and morphological abnormalities of the erythrocytes consisting of microcytosis, anisopoikilocytosis and target cells. The serum iron levels were normal. The same haematological picture was also present in those parents of the affected children that are members of this family; these adults had normal levels of Hbs A2 and F.Incorporation of [3H]leucine into the globin chains of two adults with this syndrome revealed a reduced β‐chain synthesis (β/α ratio of 0.49 and 0.53, respectively). In the two infants available for this investigation, reduced γ‐chain production was found shortly after birth. In parallel with the switch from fetal to adult haemoglobin, the deficient γ‐chain production was replaced by a similar reduction of β‐chain synthesis. These results suggested, therefore, a combined deficiency of γ and β‐chain production. The normal levels of Hb A2 were compatible with a defective δ‐chain synthesis as well. Analysis with restriction enzymes had shown previously a large deletion, comprising γ and δ genes, in one of the chromosomes of the affected individuals. The same procedure had established that, in spite of the defective β‐chain production, the β‐globin structural gene is intact.
Splenic Irradiation in B‐Prolymphocytic Leukaemia
Authors: Oscier D. G. , Catovsky D. , Errington R. D. , Goolden A. W. G. , Roberts P. D. , Galton D. A. G. 
Published: March 2008

Summary.Twelve patients with B‐cell prolymphocytic leukaemia (PLL) were treated with splenic irradiation at a weekly dose of 100 cGy to a maximum total dose of 1000 cGy. There was no morbidity associated with this treatment. Seven patients responded. Three achieved a good response and four a partial response. Two of the patients who had a partial response have subsequently died of unrelated causes. Four of the five patients who failed to respond have died as a result of their disease. When more than 25% of the prolymphocytes formed rosettes with mouse red blood cells (MRBC) the patients appeared to respond better to splenic irradiation. There was no correlation between response and the initial white cell count or the size of the spleen.
Biochemical Studies on Cryopreserved Granulocytes: Possible Explanations for the Defects Induced
Authors: McCarthy D. M. , Goldman J. M. , Peters T. J. 
Published: March 2008

Summary. The functional capacity of normal human granulocytes has been studied after cryopreservation by two different methods with dimethylsulphoxide as cryoprotectant. Both methods resulted in substantial disruption of organelle membranes and impaired cell function. Similar organelle disruption could be induced without cryopreservation following the addition of dimethylsulphoxide to cells, but the extent of this disruption was dependent on the temperature at which the dimethylsulphoxide was diluted out. The loss of cellular function also depended on the temperature and rate of dilution.We suggest that disruption of membrane organization is the mechanism underlying the loss of cell function induced by cryopreservation. Pending development of more successful methods of cryopreserving granulocytes, it is recommended that optimal preservation of functional activity would be obtained if the cryopreserved cells are thawed directly at 37°C and the cryoprotectant diluted carefully and slowly.
Antibodies against Platelet Membrane Glycoproteins II. INFLUENCE ON ADP‐ AND COLLAGEN‐INDUCED PLATELET AGGREGATION, CROSSED IMMUNOELECTROPHORESIS STUDIES AND RELEVANCE TO GLANZMANN'S THROMBASTHENIA
Authors: Jenkins C. S. P. , Ali‐Briggs ELIZABETH F. , Clemetson K. J. 
Published: March 2008

Summary. In Glanzmann's thrombasthenia glycoproteins IIb and IIIa are missing or strongly reduced and aggregation to ADP, collagen and thrombin is impaired. Antibodies against glycoproteins IIb and IIIa did not entirely induce a thrombasthenia‐like state in normal platelets. However, they did strongly inhibit collagen‐induced aggregation and inhibited the second wave of aggregation induced by ADP.Crossed immunoelectrophoresis studies using Triton X‐100 extracts of whole platelets with these antibodies gave a single immunoprecipitate. This immuno‐precipitate was absent when similar studies were carried out with thrombasthenic platelets.Platelet antibodies gave a number of immunoprecipitates with normal platelets and differences were observed with thrombasthenic platelets, the most notable of which was a marked reduction in one of the major immunoprecipitates.These results provide further evidence that glycoproteins IIb and IIIa are involved in the later stages of platelet aggregation.
Post‐transfusion Purpura: a Serological and Immunochemical Study
Authors: Pegels J. G. , Bruynes E. C. E. , Engelfriet C. P. , Kr.von dem Borne A. E. G. 
Published: March 2008

Summary. By applying the platelet suspension immunofluorescence test (PSIFT), platelet‐specific alloantibodies responsible for post‐transfusion purpura were detected in eight patients within a period of 2 years. All patients were female and had previously received blood or had been pregnant. The platelet‐specific alloantibodies had the specificity anti‐Zwa in all the patients, who were all Zw(a ‐). In two patients the platelets were tested in the acute phase of the disease and found to be coated with IgG. In one patient an ether eluate was prepared from the platelets that reacted strongly with Zw(a +) platelets, but weakly with platelets from Zwb‐homozygous individuals. The sera of these two patients, and of two others whose platelets were not directly tested, taken in the acute phase of the purpura, reacted strongly with Zwa‐positive platelets. The four sera also reacted, however weakly, with Zwa‐negative platelets, with autologous platelets taken during remission and with platelets from patients with Glanzmann's disease.It is postulated that Zwa‐anti‐Zwa complexes, present in the eluate and the sera, caused the reaction with Zwa‐negative platelets and the patients’own platelets. Immunochemical characterization of the post‐transfusion purpura antibodies showed that in all patients these were IgG, in two combined with IgM antibodies. Antibodies of the sub‐class IgG1 were found in all patients, sometimes together with IgG3. In the indirect immunofluorescence test with anti‐complement serum, the PTP antibodies in only four sera were able to fix complement. In only two of these sera were these complement‐binding antibodies detectable in the 51Cr‐lysis technique and then in a much lower titre than in the immunofluorescence technique.
Characteristics of circulating erythroid colony‐forming cells in normal and polycythaemic man
Authors: Mladenovic J. , Adamson J. W. 
Published: March 2008

Summary.Circulating erythroid progenitors from 10 patients with polycythaemia vera, nine with secondary polycythaemia and 10 normal subjects were studied in culture to determine their frequency, requirements for erythropoietin (ESF) and physical characteristics. Increased numbers of erythroid progenitors were detected in circulation from the patients with polycythaemia vera when compared to patients with secondary polycythaemia or normals, and 19 ± 3% of these progenitors formed endogenous colonies without added ESF. Velocity sedimentation analysis showed that the majority of ESF‐dependent erythroid progenitors in all study groups sedimented at 6.46 mm/h. The circulating endogenous erythroid colony‐forming cells in polycythaemia vera patients sedimented more rapidly. Tritiated thymidine suicide experiments failed to detect significant differences between the percentages of endogenous and ESF‐dependent progenitors in DNA synthesis in polycythaemia patients and normals. Thus, differences in cell cycle state were not responsible for the more rapid sedimentation of endogenous colony‐forming cells. These results further define characteristics of circulating erythroid progenitors in polycythaemia vera and normal subjects and suggest that simple culture of peripheral blood cells may be useful in the differential diagnosis of polycythaemia.
Azure B‐eosin Y stain as the standard Romanowsky‐Giemsa stain*
Authors: Wittekind D. H. , Kretschmer Vera , Sohmer Inge 
Published: March 2008

Summary.An azure B‐eosin Y stain is described which is intended to fulfil the requirements of a standard Romanowsky‐Giemsa stain. It consists of pure azure B and eosin Y only and gives strictly reproducible staining results.
Recurrent thrombocytopenia, erythroid hypoplasia and sideroblastic anaemia associated with hypothermia
Authors: O'Brien H. , Amess J. A. L. , Mollin D. L. 
Published: March 2008

Summary.A distinct and previously undescribed haematological picture was noted in three patients with hypothermia. During hypothermia there was anaemia with reduced normoblastic erythropoiesis, marked sideroblastic change and thrombocytopenia in the presence of a normal number of megakaryocytes. In two patients as the body temperature returned to normal these changes were slowly reversed. Evidence both from observations in patients and from animal experiments is given why the haematological changes are due to the hypothermia.
Stroma free human platelet lysates potentiate the in vivo thrombogenicity of factor Xa by the provision of coagulant‐active phospholipid
Authors: Giles Alan R. , Nesheim Michael E. , Hoogendoorn Hugh , Tracy Paula B. , Mann Kenneth G. 
Published: March 2008

Summary.A stroma free platelet lysate supernatant (PLS) was prepared by repeatedly freezing and thawing a human platelet suspension separated from platelet rich plasma by gel filtration. The material was potently thrombogenic in a stasis model in rabbits, but only when combined with a purified preparation of factor Xa. The dose of factor Xa used was not thrombogenic when given alone. Initial evaluation suggested the presence of a factor V activator when PLS shortened the clotting time of normal but not a V deficient plasma. Subsequent evaluation, in a system employing purified components of the prothrombinase complex and a marker of thrombin generation, dansyl‐arginine‐N‐(3‐ethyl‐1,5‐pentanediyl)amide (DAPA), failed to confirm the presence of a factor V activator. Further evaluation in this system demonstrated that the procoagulant activity related to the content of coagulant‐active phospholipid. Thin layer chromatography confirmed the presence of phospholipids known to have coagulant activity. These components were isolated by a lipid extraction procedure and the phospholipid replacing activity in the in vitro assay with DAPA was quantitatively retained. The extracted material was non‐thrombogenic in vivo but augmented the thrombogenicity of a purified preparation of Xa.
Results of immunosuppression in 170 cases of severe aplastic anaemia
Authors: Gluckman E. , Devergie A. , Poros A. , Degoulet P. 
Published: March 2008

Summary.This is a report of the European Group of Bone Marrow Transplant (EGBMT) on the influence of immunosuppression (IS) on survival of patients with severe aplastic anaemia (SAA). Fourteen teams participated in this survey involving 170 cases treated from 1974 to December 1980. The 1 year survival was 62.7%. Three types of treatment were used: (1) anti‐thymocyte globulin (ATG) alone, (2) anti‐thymocyte globulin and haplo‐identical related bone marrow infusion, and (3) high dose bolus‐6‐methyl‐prednisolone (b‐6 MePr). There was no statistical difference in survival between these three groups. Androgens did not modify survival. Blood counts before treatment had a significant prognostic value. Patients with less than 0.2 x 109/l granulocytes and less than 10 x 109/l reticulocytes had 40% 1 year survival; the others had more than 70% 1 year survival. Patients with complete or partial reconstitution had the same good prognosis. In contrast, patients with no improvement after IS had a 27% 1 year survival. Several successive courses of IS improved the prognosis of non‐responding patients. This survey confirms that IS improves the survival of patients with SAA. A prospective study will be performed to define the best and safest form of IS and to correlate clinical results with in vitro tests.
The Wiskott‐Aldrich syndrome: studies on a possible defect in mitochondrial ATP resynthesis in platelets
Authors: Akkerman J. W. N. , van Brederode W. , Gorier G. , Zegers B. J. M. , Kuis W. 
Published: March 2008

Summary.A possible defect in mitochondrial ATP resynthesis in platelets has been used for detection of Wiskott‐Aldrich syndrome carriers (Shapiro et al, 1978). The detection was based on an abnormal adrenaline‐induced platelet aggregation under conditions that only the mitochondria provide metabolic energy. We evaluated the test and found false negative and false positive results which raised doubts about the applicability of the test and the nature of the underlying defect. Direct analysis of mitochondrial ATP regeneration in patients and carriers showed an impaired mitochondrial energy production which was insufficient to maintain ATP homeostasis when glycolytic energy production was inhibited. Also abnormal was the fall in metabolic ATP concentration during stimulation with adrenaline and especially with thrombin when the platelets were incubated in glucose‐free medium. These data provide direct evidence for a regulation defect in mitochondrial ATP resynthesis in platelets of patients and carriers with Wiskott‐Aldrich syndrome.
The morphology and cytochemistry of human T‐cell subpopulations defined by monoclonal antibodies and Fc receptors
Authors: Armitage R. J. , Linch D. C. , Worman C. P. , Cawley J. C. 
Published: March 2008

Summary.Leu 2a+ (suppressor/cytotoxic) and Leu 3a+ (helper/inducer) T‐cell subsets of normal peripheral blood lymphocytes, purified by fluorescence‐activated cell sorting, respectively showed 50 ± 22% (range 31‐92%) and 82 ± 9% (range 69‐93%) dot non‐specific esterase (NSE) and 48 ± 20% (range 31‐91%) and 79 ± 10% (range 64‐92%) dot acid‐phosphatase (APase) staining. Mature T cells defined by monoclonal antibody (UCHT1) displayed 81% and 79% dot NSE and APase positivity, while E‐rosetting cells not staining with UCHT1 showed only 15% and 10% dot‐positivity and were generally larger cells with more abundant cytoplasm. Examination of Fc‐rosetting cells within the different T‐cell subsets showed that dot positivity was not directly related to μFc receptor (μFcR) expression; particularly among Leu 2a+ lymphocytes, many μFcR+ cells lacked dot staining.It is concluded that dot staining for hydrolytic enzymes is a marker of true T‐cells (as defined by monoclonal antibodies), although a minority of such cells (±20%) lack this staining pattern. Although Leu 3a+ cells display a higher percentage of dot positivity than do Leu 2a+ cells, the difference is not clinically useful, and the two cell populations are not readily distinguishable on morphological grounds.The previously reported association between dot staining and μFc receptor expression is shown to be an indirect one, attributable to the fact that most T‐cells, defined by monoclonal antisera, possess μFcR whereas most E+ UCHT1− cells lack μFcR.
The effects of deoxycoformycin and deoxyadenosine on deoxyribonucleotide concentrations in leukaemic cells
Authors: Sylwestrowicz T. , Piga A. , Murphy P. , Ganeshaguru K. , Russell N. H. , Prentice H. G. , Hoffbrand A. V. 
Published: March 2008

Summary.Intracellular deoxynucleoside triphosphate pools of normal bone marrow, thymocytes and cells from patients with ALL and AML were measured after 2 h incubation with deoxycoformycin (dCF) 10−5 M and deoxyadenosine (AdR) 10−4 M in vitro and after another 30 min incubation in the absence of dCF and AdR (‘chase’ experiment). Incubation with dCF and AdR resulted in a significant rise of dATP concentrations in all groups (the highest rises occurring in the leukaemic groups particularly in AML and Thy‐ALL). The concentrations of the other three deoxyribonucleoside triphosphates fell in all groups. The dATP level fell during the‘chase’period but in Thy‐ALL and thymocytes this fall was insignificant and slower than in the other groups. This suggests that not only intracellular build‐up of dATP but also the capacity of the cell to degrade dATP is important for in vivo cytotoxicity of dCF treatment. These results help to explain the differences in response to dCF of the different leukaemias.
Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrand's disease
Authors: Tuddenham E. G. D. , Lane R. S. , Rotblat F. , Johnson A. J. , Snape T. J. , Middleton S. , Kernoff P. B. A. 
Published: March 2008

Summary. Factor VIII was purified from cryoprecipitate by ion exchange chromatography on solid phase polyelectrolyte E‐5 (PE‐E5). The product was highly purified (3.5 u VIII: C/mg protein) compared to conventional concentrate (0.3 u VIII: C/mg protein) with low fibrinogen, low isoagglutinin titre, and a ratio of factor VIII coagulant activity (VIII: C) to factor VIII related antigen (VIIIR: Ag) of 16:1.Trial infusions of this material (PE VIII) were given to three patients with severe haemophilia A and one patient with homozygous von Willebrand's disease. These patients also each received separate infusions of intermediate purity concentrate (IPC) for comparison. There were no adverse effects. The mean half life of VIII: C after PE VIII infusion in the haemophiliacs was 10.9 h and after IPC was 12.1 h, a statistically insignificant difference. The survival of factor VIII coagulant antigen (VIII:CAg) was similar to that of VIII:C. In contrast, the half life of VIII:C and of VIII:CAg was very short after infusion of PE VIII in the patient with von Willebrand's disease (2.4 h). IPC when infused in this patient produced a typical secondary rise of VIII:C. Two bleeding episodes in severe haemophiliacs were satisfactorily treated with PE VIII. PE‐E5 deserves further study as a means of preparing clinical concentrates of factor VIII.
A comparative study of carrier detection in haemophilia A by linear discriminant function
Authors: Shen Ming‐Ching 
Published: March 2008

Summary. Blood samples collected from 37 definite carriers, 31 normal women and 64 possible carriers were examined for factor VIII‐related activities. Five variables: factor VIII coagulant activity (VIII:C) (X1), factor VIII‐related antigen (VIIIR:Ag) (X2), ristocetin co‐factor (VIIIR:RC) (X3), ratio of VIIIR: Ag to VIII:C (A/C) (X4) and ratio of VIIIR: RC to VIII:C (R/C) (X5), and 28 combinations of one to five variables were used to derive 28 linear discriminant functions. The calculation of discriminant function coefficient, individual discriminant score and cut‐off point, and the identification of normals or carriers were processed by the computer package of a biomedical computer program used at University of California at Los Angeles.A comparison of 28 linear discriminant functions for carrier detection in haemophilia A has indicated that the best and simplest is y=0.11668X1 (VIII:C)‐0.06042X2 (VIIIR:Ag), (cut‐off point =2.03742). It identified 94.6% of carriers without a single misclassification in the normal group, and the overall identification rate was 97.1%. Of 29 daughters of definite carriers who had no haemophilic sons, 13, or 44.8%, could be identified as carriers.It was found that VIIIR:RC was not as good as VIIIR:Ag for carrier detection; however, it may be concluded from this study that VIIIR:RC can be used as a supplement, if not a substitute, for VIIIR:Ag in the carrier detection of haemophilia A.
Acute lymphoblastic leukaemia cells in the diffusion chamber: change of surface phenotype during the culture period
Authors: Jäger G. , Lau B. , Pachmann K. , Dörmer P. 
Published: March 2008

Summary. Peripheral blast cells from patients with acute lymphoblastic leukaemia were cultured in the diffusion chamber system. On different harvesting days the cALL antigen, surface immunoglobulin and T‐cell antigen were investigated by direct immunofluorescence. Each cell type investigated showed a different response throughout culture, the commonest change observed being development into the B‐cell axis.Our data demonstrate that the differentiation stop of a leukaemic cell can be overcome by using the diffusion chamber system. The data therefore give some insight into the stage of developmental arrest in acute lymphoblastic leukaemia.
Haematopoietic stimulators in the serum of patients with severe aplastic anaemia
Authors: Adams J. A. , Barrett A. J. 
Published: March 2008

Summary. Recent studies on sera from patients with aplastic anaemia show it to contain both stimulators and inhibitors of granulopoiesis. We have examined the serum of patients with aplastic anaemia in a short‐term liquid culture system of human bone marrow which permits CFU‐C proliferation.Increases in CFU‐GM numbers over control values were observed with aplasia sera. All sera produced increased megakaryopoiesis, but no alteration in growth of the fibroblasts and macrophages of the adherent stromal layer. With one exception the sera had no significant direct colony stimulating activity when assayed in agar culture, but increased colony numbers were observed when the sera was incorporated into a sub‐optimal feeder layer of peripheral blood leucocytes. When aplastic serum was added to adherent cell‐depleted liquid cultures, the CFU‐C stimulating property was lost, but increased megakaryopoiesis still occurred.These results indicate the presence of a direct‐acting thrombopoietin and an indirect‐acting stimulator of CFU‐C proliferation in the serum of patients with aplastic anaemia, which may be related to the pathophysiology of the condition.
Characterization of T‐lymphocyte subsets in hairy‐cell leukaemia (HCL) by monoclonal antibodies: comparison with Fcγ, Fcμ receptors and correlation with disease activity
Authors: Lauria F. , Foa R. , Gobbi M. , Pulvirenti A. , Raspadori D. , Giubellino M. C. , Tura S. 
Published: March 2008

Summary. T lymphocytes from 22 patients with hairy‐cell leukaemia (HCL) were assessed on the basis of their ability to bind the Fc receptors for IgM (Tμ) or IgG (Tγ), and by the capacity to react with OKT monoclonal antibodies. T‐cell subsets defined by the presence of Fc receptors for IgM or IgG showed an overall increase in the proportion of Tγ cells and a non‐significant decrease of Tμ cells, regardless of the clinical state of the disease. Results with monoclonal antibodies showed that in patients with HCL in clinical remission T‐cell subsets were normally balanced, while in patients with active disease the distribution and absolute number of T‐cell subpopulations appeared markedly impaired, with a significant increase of OKT8 positive cells (suppressor/cytotoxic) and a significant reduction of OKT4 positive cells (helper/inducer) compared both with active disease patients and with normal controls. The OKT4+/OKT8+ ratio was also significantly reduced in patients with active disease compared with those in clinical remission and with controls (0.96 v 1.63 and v 1.94, respectively).Our findings confirm the heterogeneity of T‐cell subset positivity defined by monoclonal antibodies and by Fcμ and Fcγ receptors and suggest that in patients with HCL the distribution of OKT4 and OKT8 positive cells is closely correlated to the clinical state of the disease.