bluuebunny/crossref_metadata_2025_split huggingface dataset:
Studies on immunological assay of vitamin‐K dependent factors.
Authors: Mikami S. , O'Brien D. P. , Mellars G. , Goodall A. H. , Tuddenham E. G. D. 
Published: March 2008

Summary. Two‐site immunoradiometric assays (IRMAs) for factor IX antigen (IX:Ag) were developed using a monoclonal antibody (RFF‐IX/1) on the solidphase and either another monoclonal antibody (RFF‐IX/4) or a human polyclonal inhibitor antiserum as tracer (M‐M and M‐I IRMA respectively). The lower sensitivity limits of these two assays for IX: Ag in normal reference plasma were 4 × 10−4 (M‐M IRMA) and 2 × 10−4 (M‐I IRMA) units/ml.In 20 samples of normal plasma, levels of factor IX coagulation activity (IX:C) and of factor IX antigen measured by both IRMAs were highly correlated. Mean values of approximately 1·0 units/ml were obtained in all three assays. In normal serum, IX: Ag levels were lower with means of 0·84 (M‐M IRMA) and 0.83 (M‐I IRMA) units/ml. 4/25 patients with haemophilia B were CRM neg., two were CRM+ and the remaining 19 patients were CRMR variants. In two of these, IX: Ag was detectable by M‐I IRMA whilst IX:C and IX:Ag measured by M‐M IRMA were undetectable. In plasma from a fetus subsequently terminated on eugenic grounds, IX:C and IX:Ag by both M‐M and M‐I IRMA were undetectable.In warfarin‐treated plasma (n= 12), the level of IX:C was low (mean 0·39 units/ml). The levels of IX: Ag measured by M‐M IRMA (mean of 0·80 units/ml) and by M‐I IRMA (0.70 units/ml) showed a discrepancy. M‐M IRMA reflects thereal amount of IX:Ag in warfarinized plasma because both monoclonal antibodies bind to epitopes distant from the light chain carboxylated region.Western blotting of denatured factor IX demonstrated that RFF‐IX/1 binds an epitope that is lost after XIa activation. RFF‐IX/4 binds the heavy chain.
Increasing haemoglobin oxygen affinity to prevent sickling: abnormal haemoglobin variants as models
Authors: Franklin I. M. , Huehns E. R. , Rosemeyer M. A. 
Published: March 2008

Summary. TWO abnormal high oxygen affinity haemoglobins have been studied for their ability to inhibit the polymerization of deoxy‐Hb S. They were used as models to predict the effect of chemically modifying haemoglobin to increase oxygen affinity since the oxy(R)‐conformation is the most potent inhibitor known of cell sickling. The participation of these variants in deoxy‐Hb S polymers has been described quantitatively in terms of an exclusion coefficient, f, that relates their behaviour to that of deoxy‐Hb S, and qualitatively in terms of an exponent of hybridization.Hb Bethesda was a potent inhibitor of polymerization, its behaviour being similar to that of Hb F studied previously. Hb Radcliffe demonstrated atypical behaviour, with hybrid molecules of the formula α2βsβRad participating in the polymerization as effectively as Hb S, as has been shown for Hb C.These data have implications for the development of anti‐sickling agents designed to increase oxygen affinity by covalent binding to Hb S.
An evaluation of the acidified glycerol lysis test
Authors: Rutherford C. J. , Postlewaight B. F. , Hallowes M. 
Published: March 2008

SummaryThe acidified glycerol lysis test (AGLT), as a screening procedure for spherocytosis, has been assessed for sensitivity and specificity. AGLT identified 24 of 26 subjects with hereditary spherocytosis and produced 19 abnormal AGLTs out of a total of 52 normal blood donors. The claims for the test as being both 100% sensitive and specific could not be confirmed.
Immunological abnormalities in myelodysplastic syndromes II. COEXISTENT LYMPHOID OR PLASMA CELL NEOPLASMS: A REPORT OF 20 CASES UNRELATED TO CHEMOTHERAPY
Authors: Copplestone J. A. , Mufti G. J. , Hamblin T. J. , Oscier D. G. 
Published: March 2008

SummaryWe report a series of 20 patients with the myelodysplastic syndrome (MDS) each with a coexistent lymphoid or plasmacytic neoplasm. In none of the patients did chemotherapy play a part in the pathogenesis. The possible reasons for the coincidence of these conditions are discussed. The frequency and variety of associated neoplasms reinforces the idea that in MDS there are genetically unstable progenitor cells liable to clonal differentiation along a variety of pathways given appropriate stimuli.
Factor VIII concentrates contain factor VIII procoagulant antigen bound to phospholipid
Authors: Kemball‐Cook G. , Barrowcliffe T. W. 
Published: March 2008

SummaryFractionation of a human antibody to factor VIII :Ag by immuno‐absorption with factor VIII/phospholipid (PL) complex has produced two pools of labelled Fab’fragments which can be used in fluid‐phase immunoradiometric assays (IRMAs). One pool binds only to the PL‐binding sites on factor VIII :Ag (and thus measures only that factor VIII: Ag in a sample not bound to PL), while the second binds to other antigenic sites. Parallel assays of factor VIII‐containing materials using these two pools provide estimates of the proportion of factor VIII :Ag bound to PL in those materials. Five batches each of nine brands of factor VIII concentrates from eight different manufacturers were tested for PL‐bound factor VIII :Ag by this method: all contained substantial amounts, ranging from 28% to 54% of the total factor VIII :Ag present. In addition, varying amounts of the factor VIII: Ag present in both non‐activated and activated prothrombin complex concentrates (PCCs) from four manufacturers were PL‐bound.
Bone marrow transplantation in two multiply transfused patients with thalassaemia major
Authors: Brochstein Joel A. , Kirkpatrick Dahlia , Giardina Patricia J. , Weinberg Rona S. , Alter Blanche P. , Driscoll Catherine , Wolfe Lorraine , Shank Brenda , O'Reilly Richard j. 
Published: March 2008

SummaryBone marrow transplantation has generally been unsuccessful when applied to patients with thalassaemia major over the age of 6 years. We report here two successful transplants for this disorder in a 7‐year‐old boy and an 11‐year‐old girl following a pre‐transplant cytoreductive/immunosuppressive regimen of total body irradiation and cyclophosphamide. Complete durable engraftment of donor haematopoietic and lymphoid populations was documented through several approaches, including cytogenetic analysis, haemoglobin electrophoresis, globin chain synthetic ratios, red cell typing and DNA restriction enzyme analysis. Both patients are surviving in good health, 28 and 9 months from transplantation. The successful outcome in these patients demonstrates the feasibility of marrow transplantation for the treatment of thalassaemia in multiply transfused and, presumably, highly sensitized patients.
Normal levels of ATP, total nucleotides and activities of three enzymes related to nucleotide metabolism in fetal erythrocytes
Authors: Lestas A. N. , Nicolaides K. H. , Rodeck C. H. , Bellingham A. J. 
Published: March 2008

SummaryPure fetal blood was obtained by direct‐vision fetoscopy from 30 fetuses at 17–23 weeks gestation. The erythrocyte concentrations of ATP and total nucleotides and the activities of the enzymes pyrimidine‐5′‐nucleotide nucleosidase (Fyr5N), phosphoribosylpyrophosphate (PRPP) synthetase and adenylate kinase (AK) were analysed by established techniques to find the normal ranges for this gestational age. The ranges were relatively narrow and could serve as reference values for the prenatal diagnosis of defects in nucleotide metabolism.
The role of aggressive chemotherapy in the treatment of the myelodysplastic syndromes
Authors: Tricot G. , Boogaerts M. A. 
Published: March 2008

SummaryFifteen patients with the initial diagnosis of myelodysplastic syndrome (MDS) received aggressive chemotherapy with high dose cytarabine or with a standard acute myeloid leukaemia (AML) regime. Cases treated with aggressive chemotherapy were either younger individuals with refractory anaemia with excess of blasts (RAEB) or patients, irrespective of age in advanced stages of MDS (RAEB in transformation or after evolution to frank AML), who did not have a major infection at the time of presentation. Age seemed to be the most important factor in determining the outcome of aggressive remission induction chemotherapy in MDS: 86% of the patients <50 years entered complete remission, compared to only 25% in the older age group. In spite of intensive consolidation therapy the duration of complete remission was short. We conclude that young patients (<50 years) with excess of bone marrow blasts should be treated with aggressive chemotherapy even in the early stages of the disease. Elderly patients in advanced stages of MDS should be treated with less aggressive chemotherapy.
Alpha thalassaemia in an Italian population
Authors: Velati C. , Sampietro M. , Biassoni M. , Cappellini. M. D. , Wainscoat J. S. , Higgs D. R. , Fiorelli G. 
Published: March 2008

SummaryThe incidence of α‐thalassaemia in an Italian population has been determined by a survey of random cord bloods for the presence of Hb Bart's. 144 out of 4730 (3%) had detectable amounts of Hb Bart's. Furthermore, α‐globin gene analysis of 100 random cord bloods showed that five out of 100 had the common type of α‐thalassaemia caused by a single α‐globin gene deletion (—α). The molecular basis of α‐thalassaemia was also determined in a selected group of 34 newborns with detectable levels of Hb Bart's. 25 of these cases had the —α3.7 deletion type of α‐thalassaemia and nine had nondeletion types of α‐thalassaemia in four of which the molecular defect was detectable directly by restriction enzyme analysis.
The myelosuppressive effect of recombinant interferon γ in short‐term and long‐term marrow cultures
Authors: Coutinho L. H. , Testa N. G. , Dexter T. M. 
Published: March 2008

SummaryThe effect of a highly purified human gamma IFN (r‐γ‐IFN) on the growth of haemopoietic progenitors was examined in soft agar assays and in long‐term bone marrow cultures. r‐γ‐IFN reduced colony formation by progenitor cells of the granulocyte/macrophage lineage (GM‐CFC). the eryth‐roid lineage (BFU‐E) and multipotent cells (GEMM‐CFC), and suppressed haemopoiesis in long‐term culture in a dose‐related fashion. At high doses (1000 units/ml) r‐γ‐IFN appeared to be toxic to the stromal cells of the bone marrow.
Immunohistological localization of factor VIII in placental endothelial cells
Authors: Stel Herbert V. , Oudejans Cornelis B. M. , Veerman Enno C. I. , Meijer Chris J. L. M. 
Published: March 2008

SummaryDNA‐recombinant studies have established that factor VIII‐mRNA is present in liver and placental tissue. In a previous immunohistological study using monoclonal antibodies we have localized factor VIII in liver sinusoidal endothelial cells. In this paper we demonstrate that also the endothelial cells lining the fetal vessels in human placenta contain factor VIII. Based on the combined results of the present study and a previous study we favour‐the concept that factor VIII is synthesized in placental endothelial cells.
Immunohistochemical study of basement membrane proteins and type III procollagen in myelofibrosis
Authors: Apaja‐Sarkkinen Meeri , Autio‐Harmainen Helena , Alavaikko Martti , Risteli Juha , Risteli Leila 
Published: March 2008

SummaryIn this study the distribution of type IV collagen in the marrow is compared with that of laminin, another basement membrane protein. In addition, incompletely processed type III procollagen is identified with specific antibodies.
Failure of transferrin to enhance iron absorption in achlorhydric human subjects
Authors: Bezwoda W. R. , Macphail A. P. , Bothwell T. H. , Baynes R. D. , Derman D. P. , Torrance J. D. 
Published: March 2008

SummaryThere is evidence in experimental animals that transferrin, produced either by gastrointestinal cells or derived from bile, mediates the luminal absorption of iron. The applicability of these findings to human subjects was tested by administering diferric transferrin labelled with 3 mg 59Fe to seven patients with pernicious anaemia. Achlorhydric subjects were chosen to ensure that the iron transferrin complex did not dissociate in the stomach. The geometric mean absorption of 1.4% was similar to that of 3 mg iron given as ferric chloride (1.9%) and much less than that of ferrous ascorbate (18.9%). These findings suggest that transferrin does not play a physiological role in the absorption of iron in human subjects.
Congenital plasminogen deficiency associated with venous thromboembolism: therapeutic trial with stanozolol
Authors: Mannucci P. M. , Kluft C. , Traas D. W. , Seveso P. , D'Angelo A. 
Published: March 2008

SummaryWe describe the case of a 34‐year‐old man with a history of venous thrombotic disease and demonstrate that his plasma plasminogen was reduced to about half‐normal levels but was functionally intact. The deficiency was inherited as an autosomal trait and the propositus and his sister are heterozygotes. The propositus, but no other family member, had an associated deficiency of plasminogen activator activity, probably due to heightened plasminogen activator inhibition in his plasma. A therapeutic trial with the anabolic steroid stanozolol transiently normalized plasminogen and plasminogen activator levels.
Alteration of red cell membrane organization in sickle cell anaemia
Authors: Choe Hye‐Ryun , Schlegel Robert A. , Rubin Elizabeth , Williamson Patrick , Westerman Maxwell P. 
Published: March 2008

SummaryBee venom phospholipase A2 and the fluorescent probe merocyanine 540 were used to examine plasma membrane phospholipid organization in the spicules released by deoxygenation and reoxygenation of sickle red cells, as well as in reversibly and irreversibly sickled erythrocytes. Digestion of phosphatidyl ethanolamine in spicules was comparable to that of phosphatidyl choline, and these structures were stained by the fluorescent probe. Both assays suggest that membrane lipid asymmetry is disrupted in spicules. The residual cells, from which the spicules were derived, retain the normal asymmetry in phospholipid distribution between the outer and inner leaflets of the plasma membrane bilayer. Comparable experiments with cell fractions enriched in irreversibly sickled cells revealed a partial enhancement of phosphatidyl ethanolamine digestion, confirming the similar experiments of Lubin et al (1981). Staining of these cells with merocyanine 540, however, did not reveal a subfraction of stainable cells, indicating that this increase in phosphatidyl ethanolamine digestion is not due to the presence of a small fraction of cells which have completely lost their membrane asymmetry.
Variable differentiation patterns of acute myelogenous leukaemia blasts in liquid suspension cultures
Authors: Baer Maria R. , Dessypris Emmanuel N. , Stein Richard S. , Cousar John B. , Krantz Sanford B. 
Published: March 2008

SummaryTo study the ability of acute myelogenous leukaemia blasts to spontaneously differentiate in vitro, bone marrow and/or blood mononuclear cells from 63 patients with acute myelogenous leukaemia were incubated in liquid suspension cultures containing human serum, without addition of chemical inducers of differentiation. Cultures were examined weekly for disappearance or persistence of blasts, and for appearance of morphologically recognizable granulocytes and macrophages. Culture outcomes were extremely variable, ranging from lack of appearance of differentiated cells to complete disappearance of blasts with replacement by mature cells. In 50 cases an increase (25–185%) in the absolute number of differentiated cells in culture was noted during the culture period. Full differentiation was seen exclusively in cultures from 13/48 (27·1%) patients studied at diagnosis, as compared to 0/20 patients studied at relapse (P<0·01). The ability to fully or partially differentiate in culture was lost to a significant degree at relapse (13/20 patients) as compared to diagnosis (48/48 patients, P= 0·0001). At diagnosis full differentiation in culture was associated with a significantly higher remission rate than partial differentiation (89% versus 40%. P<0·02). Origination of mature cells from leukaemic rather than normal precursors was suggested by the appearance of Auer rods in mature cells in seven cases, by the correlation of types of differentiated cells seen in culture with the FAB class of leukaemia and by cytogenetic data in one case.
Heterogeneity of large granular lymphocyte proliferations: morphological, immunological and molecular analysis in seven patients
Authors: Lauria F. , Foa R. , Migone N. , Giubellino M. C. , Raspadori D. , Buzzi M. , Casorati G. , Gobbi M. , Tazzari P. L. , Tura S. 
Published: March 2008

SummaryThe clinical, morphological, immunological and molecular features of seven patients with a stable picture of chronic granular lymphocytosis, observed over a period of up to 4 years, were studied. Mild splenomegaly was detected in one patient, while lymphoadenopathy and hepatomegaly were absent. Surface marker analysis showed in five patients the common membrane phenotype of granular T‐cell lymphocytosis (T3 +, T4‐, T8 +, Leu‐7 +); of the remaining two, one presented an unusual phenotype (T3 +, T4 +, T8 +) and the other showed a marked positivity with the Leu‐11 and M1 monoclonal antibodies, but lacked the T3, T4, T8 antigens. Three cases had a low (<30%) expression of the T1 antigen. Functional studies showed that the proliferative response to PHA and the NK function were reduced in four of the seven cases. Molecular analysis, performed in six cases, revealed a monoclonal rearrangement of the T‐cell receptor β‐chain gene in three, a polyclonal T‐cell configuration in two and a germ‐line arrangement in the last. All three monoclonal cases showed a depressed NK activity and two a reduced PHA response. The results of this study document the heterogeneity of granular lymphocyte expansions and suggest that the clonal or reactive nature of these often indolent proliferations, suspected on the basis of immunologic functional studies, may be recognized at the DNA level.
Comparative effect of heparin and heparan sulphate on two abnormal antithrombin III type 3 variants
Authors: Fischer A. M. , Beguin S. , Sternberg C. , Dautzenberg M. D. 
Published: March 2008

SummaryTwo families were found with an antithrombin III that was unresponsive towards heparin (type 3 AT III variants). The abnormal species were purified using affinity chromatography on Sepharose bound anti‐AT III antibodies. This yielded active proteins, as judged by their progressive antithrombin activities. In an attempt to explain the throm‐botic tendency observed in this abnormality we compared the effect of heparin and heparan sulphate on these abnormal AT III, since, unlike heparin, heparan sulphate is a naturally occurring anticoagulant in the human. In normal plasma the heparan sulphate used in this study had a heparin‐like activity of 50 U/mg by anti‐F.XA and anti‐F.IIa amidolytic assays. Full expression of the heparin cofactor activity in normal plasma could be obtained at a final concentration of 0·024 mg/ml of heparan sulphate (equivalent to 0·007 mg/ml of heparin). At this concentration of heparan sulphate the two abnormal AT III still exhibit a heparin cofactor activity below 10%. This absence of binding of heparan sulphate to abnormal AT III of type 3 could explain why some patients with this abnormality suffer from thrombo‐embolic episodes while their AT III acts normally in the absence of heparin.
Immunohistological diagnosis of a case of composite lymphoma
Authors: Salter D. M. , Sheehan T. , Krajewski A. S. , Ludlam C. A. 
Published: March 2008

SummaryA case of composite lymphoma consisting of Hodgkin's disease and B cell lymphocytic lymphoma is described. A diagnosis of lymphocyte predominant Hodgkin's disease was made initially by histology. The presence of a composite lymphoma was only appreciated once immunohis tochemistry was available. The findings illustrate the importance of the use of immunological markers for the accurate diagnosis of lymphoma.
Inhibition of platelet aggregation by myeloid leukaemic cells demonstrated in vitro
Authors: Fauldt R. , Ankerst J. , Zoucas E. 
Published: March 2008

SummaryThe effect of myeloid leukaemic cells, cells of the promyelocytic cell line HL‐60 and normal polymorphonuclear granulocytes (PMN), enclosed in dialysis tubes, on the aggregation of allogeneic normal platelets after induction with arachidonic acid (AA) and with adenosine diphosphate (ADP) was investigated in vitro. Inhibition of aggregation was seen after preincubation of the platelets with leukaemic blood or bone marrow cells from 7/14 patients belonging to various FAB groups and with HL‐60 cells, but not with normal PMN (14/14 cases). A dose‐dependent inhibition was seen after lysis of some leukaemic cells with anti‐human rabbit antiserum and active complement. Lysis of normal PMN inhibited platelet aggregation slightly and inconstantly and only at higher cell concentrations. Platelet inhibitory activity was not related to a higher rate of metabolism of the leukaemic cells which inhibited platelet aggregation since they did not differ from the cells not inhibiting aggregation with respect to heat production. Neither was a non‐specifically increased cell membrane permeability the cause of the release of platelet inhibitory factor(s) since the release of 51Cr‐labelled leukaemic cells was not related to the inhibition of platelet aggregation.
Flow cytochemical patterns of white blood cells in human haematopoietic malignancies: II, CHRONIC LEUKAEMIAS
Authors: Drewinko B. , Bollinger P. , Brallas C. , Wyatt J. , Simson E. , Trujillo J. M. 
Published: March 2008

SummaryPeripheral blood samples from 73 patients with chronic leukaemia were measured with the Technicon H‐6000 automated haematology analyser to provide flow cytochemical (peroxidase content) and volume (light scatter) discriminated scattergram patterns. For chronic granulocytic leukaemia (CGL). these patterns were so reproducible and distinct that they allowed an immediate diagnosis even without the benefit of microscopic examination, Relative and absolute basophilia was an invariable feature, and remained detected by the H‐6000 even when the patient was in haematologic and cytogenetic remission or progressed into blast crisis (BC). Meet patients in BC also demonstrated an inordinately high number oflarge unstained cells (LUC) and high proportions of ‘lymphocytes’(small blasts with no peroxidase content by visual inspection). Thus, for patients with CGL, LUC values above 10%, and/or steady increments in the proportion of ‘lymphocytes’, merit concern as these changes may herald an accelerated phase of disease.The scattergram pattern of untreated chronic lymphocytic leukaemia (CLL) showed a dense accumulation of data points within the lymphocytic ‘box’with a small cluster of granulocytic elements. Most patients also had a frankly abnormal proportion of LUC. Sixteen patients with CLL were compared for ratios of LUC to lymphocytes and stage of disease; patients with the most advanced stage (IV) had the highest, statistically significant values, than the patients with more benign disease. Thus, it is possible that follow up with this instrument of patients with CLL will also allow early detection of an impending prolymphocytoid transformation (accelerated phase) of this disease.