bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Development of Haematological Changes in Homozygous Sickle Cell Disease: a Cohort Study from Birth to 6 Years
Authors: Serjeant Graham R. , Grandison Yvonne , Lowrie Yvonne , Mason Karlene , Phillips Jennifer , Serjeant Beryl E. , Vaidya Shuba 
Published: March 2008

Summary.A cohort study of sickle cell disease from birth has allowed observations on the disease without the symptomatic selection inherent in previous series. The development of haematological indices from birth to 6 years in male and female infants with homozygous sickle cell (SS) disease is presented and compared with values in age and sex matched controls with a normal haemoglobin (AA) genotype previously presented elsewhere. In SS disease total haemoglobin levels fell rapidly from birth to a plateau at 3‐6 months before falling again to 15 months after which no age related change occurred. Mean cell haemoglobin concentration fell from birth to lowest values at 15‐18 months before increasing to reach the level present at birth by the age of 5 years. Red cell counts fell rapidly after birth to a plateau at 2 months, increased slightly to 6 months and then fell steadily throughout the remaining period of the study. The mean cell volume and mean cell haemoglobin also fell rapidly after birth reaching the lowest values by 6 months and then increased progressively. Female patients showed significantly higher MCV from 4 to 8 months and significantly higher haemoglobin levels from 15 months to 41/2 years. Compared to AA controls, SS patients manifested significantly lower levels of haemoglobin from 2 weeks, and red cell counts from 1 month, and significantly higher levels of MCHC from 4 months to 3 years, MCV from 8 months to 5 years, and serum iron levels from 1 to 4 years. Children with SS disease were partially protected from iron deficiency in early childhood, perhaps by increased intestinal absorption of iron, and the associated increase in intracellular haemoglobin concentration might be disadvantageous during this high risk period.
Deficiency of Plasma Prostacyclin or PGI2 Regenerating Ability in Sickle Cell Anaemia
Authors: Stuart Marie J. , Sills Richard H. 
Published: March 2008

Summary.We have demonstrated that patients with sickle cell disease > 2 years of age have a marked decrease in their plasma PGI2 or prostacyclin regenerating ability (0.05 ± 0.08 ng/mg of vascular tissue) when compared to normal controls (0–44 ± 0.12). This abnormality was not present in other chronic haemolytic states, and was not related to concomitant hyposplenism. Normal activity was observed in patients with sickle cell disease following a period of prophylactic red cell transfusions (0.32 ± 0.13). Since vascular prostacyclin is antiaggregatory and antithrombotic, the deficiency in the prostacyclin regenerating ability of patients with sickle cell disease may play a pathogenic role in the development of the microvascular and thrombotic complications seen in this disorder.
The Switch from Haemoglobin F to A: the Time Course of Qualitative and Quantitative Variations of Haemoglobins after Birth
Authors: Terrenato L. , Bertilaccio C. , Spinelli P. , Colombo B. 
Published: March 2008

Summary. The time course of the transition from HbF to HbA production in the postnatal period has been studied. The description is based on the time‐dependent pattern of total haemoglobin and of HbF observed in an 8 months follow‐up of 25 premature newborns. The absolute amount of HbF decreases exponentially from birth to approximately 25 weeks (from 15.56 0.48 g/dl to 0.66 0.08 g/dl), at a weekly rate of approximately 16%. On the other hand, HbA shows a biphasic pattern: first it decreases slightly from birth to 6 weeks (from 4.09 0.32 g/dl to 2.63 0.33 g/dl); afterwards it increases exponentially at a weekly rate of 5% up to a plateau value, until HbF is almost completely replaced by HbA. Therefore the total haemoglobin decreases exponentially from birth to approximately 6 weeks (from 19.65 0.46 to 9.92 0.31); it remains approximately constant for the next 6 weeks, and thereafter it increases very slowly.From these data, two distinct phases of postnatal erythropoiesis can be identified: the first (from birth to 6 weeks) is characterized by a decrease in the total amount of haemoglobin produced whereas in the second there is an alteration in the relative proportions of HbF and HbA being synthesized.
Terminal Deoxynucleotidyl Transferase Expression in Acute Non‐lymphoid Leukaemia: an Analysis by Immunofluorescence
Authors: Bradstock K. F. , Hoffbrand A. V. , Ganeshaguru K. , Llewellin P. , Patterson K. , Wonke B. , Prentice A. G. , Bennett M. , Pizzolo G. , Bollum F. J. , Janossy G. 
Published: March 2008

Summary. Indirect immunofluorescence for terminal transferase enzyme (TdT) was used to study the blasts of 64 patients with acute non‐lymphoid leukaemia (ANLL). In 32 patients no TdT positive cells were seen. In 19 cases a small subpopulation of cells expressing TdT was detected; these constituted up to 5% of total nucleated cells, and it was not clear whether these TdT positive cells were part of the leukaemic process or represented residual normal bone marrow lymphoid cells.The remaining 13 patients had TdT positive cells accounting for 7–90% of the total. In two of these cases TdT was expressed on blasts with myeloid features, representing an aberrant expression of TdT by myeloid cells; in contrast, in three cases mixed populations of TdT positive lymphoid blasts and TdT negative myeloid blasts were observed. In the remaining cases it was not possible to determine whether the TdT positive cells had definite lymphoid or myeloid features. Cytogenetic analysis showed no evidence of the Philadelphia chromosome. Response to treatment was assessed in 11 of the 13 patients. Only one patient remitted with the initial choice of therapy (DAT); four failed to respond to initial regimes of vineristine and prednisone (V & P) while the other five patients did not respond to myelotoxic combinations (DAT). Only one patient subsequently entered complete remission on second line therapy (V & P). This group of patients with TdT+ ANLL had a particularly bad prognosis, and appeared to differ from cases of TdT positive acute undifferentiated leukaemia, which often respond to V & P therapy.
Methaemoglobinaemia Resulting from Heterozygosity for Two NADH‐Methaemoglobin Reductase Variants: Characterization as NADH‐Ferricyanide Reductase
Authors: Board P. G. , Pidcock M. E. 
Published: March 2008

Summary. A case of congenital methaemoglobinaemia was found to result from heterozygosity for two different forms of NADH‐methaemoglobin reductase (NADH‐MR) deficiency. One of these variant forms was found to be electrophoretically fast, and to have sufficient residual activity to allow detailed characterization. This variant was characterized by its NADH‐ferricyanide reductase activity, and was found to have a normal pH optimum and normal affinity for K3Fe(CN)6, NADH and deamino NADH. The diminished activity associated with this variant appeared to result from its decreased stability.It is suggested that the NADH‐ferricyanide reductase activity of NADH‐MR provides a convenient and standardized procedure for the characterization of NADH‐MR.
Severe Aplastic Anaemia: Correlation of in Vitro Tests with Clinical Response to Immunosuppression in 20 Patients
Authors: Bacigalupo A. , Podesta M. , Van Lint M. T. , Vimercati R. , Cerri R. , Rossi E. , Risso M. , Carella A. , Santini G. , Damasio E. , Giordano D. , Marmont A. M. 
Published: March 2008

Summary. Colony formation in agar (CFU‐c) was studied in 20 patients with severe aplastic anaemia by three different assays: (1) cultures of light density untreated marrow cells; (2) cultures of marrow cells manipulated in order to enhance colony formation (pretreatment with antilymphocytic globulin, ALG, or 6‐methylprednisolone, 6‐MPr, T cell depletion, adherent cell (AC) depletion, depletion of both AC and T cells), and (3) co‐culture of putative suppressor T cells with autologous T‐depleted marrow cells.By the first assay, all patients showed poor colony formation (1 1.5 colonies/105 cells; normal controls 46.18 colonies/105 cells). By the second assay, ALG and 6‐MPr had no significant effect on colony formation. Removal of adherent cells proved equally without effect on colony growth. On the contrary, removal of T cells enhanced significantly (P < 0.001) colony formation in 10 out of 20 patients. By the third assay, colony formation of marrow cells (deprived of T lymphocytes) was inhibited by the addition of autologous T cells in six patients studied.All patients were given high dose bolus 6‐MPr as first treatment on admission: only patients who had detectable suppressor T cells in their marrow achieved a complete autologous haematologic reconstitution after 6‐MPr or after 6‐MPr and ALG.The results of this study indicate that detection of CFU‐c/suppressor T cells correlates with responses to immunosuppressive regimens, and may thus help to identify patients with immune mediated aplastic anaemia.
Bone Marrow Transplantation in Severe Aplastic Anaemia: a Survey of the European Group for Bone Marrow Transplantation (E.G.B.M.T.) *
Authors: Gluckman E. , Barrett A. J. , Arcese W. , Devergie A. , Degoulet P. 
Published: March 2008

Summary. A survey of the results of bone marrow transplantation for severe aplastic anaemia in 13 European teams is reported. 159 questionnaires were analysed by univariate and multivariate analysis. The overall 1 year survival was 41.2%. The chance of survival was decreased by the presence of infection before grafting and by the use of female donors. The conditioning regimen did not influence survival. Graft rejection was influenced by the conditioning regimen and the sex of the donor. Graft‐versus‐host disease was influenced by the conditioning regimen, and the age and the sex of the recipient.This study defines prognostic indices which may be helpful in determining the likelihood of success after marrow transplantation for severe aplastic anaemia.
Serological and biochemical analysis of the PlA1 alloantigen of human platelets
Authors: Lane Judy , Brown Melissa , Bernstein Irwin , Wilcox Paula K. , Slichter Sherrill J. , Nowinski Robert C. 
Published: March 2008

A solid phase platelet antibody assay has been developed which rapidly and sensitively detects PlA1 antibodies. The three‐step assay is performed by: (1) adhering platelets to the wells of a microtitre plate, (2) incubating the platelets with test serum, and (3) adding radiolabelled Staphylococcal protein A which binds to the Fc domain of IgG antibodies. Immune reactions are detected by overnight autoradiography.Characterization of the PlA1 antigen was performed by using PlA1 antisera in immune precipitation assays. A 90 000 dalton molecular weight species was precipitated from PlA1 positive human and dog platelets.
A technique for rapid isolation of bone marrow mononuclear cells using Ficoll‐Metrizoate and the IBM 2991 blood cell processor
Authors: Gilmore M. J. M. L. , Prentice H. G. , Blacklock H. A. , Ma D. D. F. , Janossy G. , Hoffbrand A. V. 
Published: March 2008

Marrow from seven normal donors and patients has been layered onto Ficoll‐Metrizoate (FM) under pressure in the IBM 2991 blood cell processor to isolate the mononuclear cell (MNC) population prior to allogeneic transplantation or cryopreservation. This separation method, which takes less than 90 min, is a further development since our previous report detailing the use of the IBM 2991 to produce a concentrated marrow‘buffy coat’for infusion (Gilmore & Prentice, 1981). By adding FM to the system, marrow stem cells are further concentrated in a small volume with removal of unwanted granulocytes and red blood cells. This facilitates the in vitro treatment of marrow with monoclonal antibodies (Granger et al, 1982) or drugs, for either the selective elimination of malignant cells prior to autologous bone marrow transplantation (BMT), or T lymphocytes in an attempt to prevent graft versus host disease (GvHD) in allogeneic BMT.Five of the seven marrows processed by this procedure have thus far been infused into lethally irradiated recipients with engraftment (allogeneic); the other two marrows have been cryopreserved.
Plasma fibronectin in acute leukaemia
Authors: Boughton B. J. , Simpson A. 
Published: March 2008

Summary.In acute leukaemia, low plasma fibronectin was seen at diagnosis and when the disease relapsed. This was not directly related to tumour load, chemotherapy or neutropenia, but correlated well with episodes of intercurrent infection. In severe infections, fibronectin fell rapidly to very low levels, and was sometimes not restored to normal up to 2 weeks later. Preliminary attempts at replacement therapy were successful and the possible importance of this is discussed.
Joint involvement in patients with severe haemophilia A in 1957–59 and 1978–79
Authors: Helske T. , Ikkala E. , Myllylä G. , Nevanlinna H. R. , Rasi V. 
Published: March 2008

Summary.Clinical findings of joint involvement in patients with severe haemophilia A were compared between two surveys made in 1957‐59 and 1978–79. Permanent joint changes were seen in 50 (81%) of 62 patients in 1957‐59 and in 70 (86%) of 81 patients in 1978–79. The incidence of permanent changes was the same in the two surveys in all 10‐year age groups, but the degree of involvement was less severe in the two youngest age groups in 1978–79. It is concluded that effective replacement therapy can delay but not prevent completely the development of progressive arthropathy in patients with severe haemophilia A.
Serological studies in patients on platelet‐ and granulocyte‐substitution therapy
Authors: Pegels J.G. , Bruynes E.C.E. , Engelriet C.P. , Kr. vondem Borne A.E.G. 
Published: March 2008

Summary. A serological follow‐up study was undertaken in 47 patients with bone‐marrow failure, who were repeatedly transfused with random donor granulocytes and/or platelets. Sera, obtained at regular intervals, were investigated in the leucoagglutination test, the lymphocytotoxic test and the immunofluorescence test on paraformaldehyde‐fixed platelets, granulocytes and lymphocytes. The frequency of alloimmunization was high (73%). Not only HLA antibodies, but also blood‐cell‐specific alloantibodies were detected in the sera of the alloimmunized patients, e.g. lymphocyte‐specific, platelet‐specific and granulocyte‐specific antibodies.The immunofluorescence test on platelets was also used as a crossmatch, and when this test was positive it was always found that after platelet transfusion the increment value was nil.
Relationship between platelet aggregation, thromboxane synthesis and albumin concentration in nephrotic syndrome
Authors: Jackson C. A. , Greaves M. , Patterson A. D. , Brown C. B. , Preston F. E. 
Published: March 2008

Summary. Increased platelet aggregability on stimulation with sodium arachidonate (NaAA), collagen and ADP was found in a group of 14 patients with nephrotic syndrome when compared with age and sex matched controls. Five of the group also exhibited spontaneous platelet aggregation (SPA), associated with synthesis of thromboxane B2 (TxB2), and which appeared to correlate with a markedly increased serum triglyceride concentration.Thromboxane B2 generation in response to NaAA was increased and reflected both the low serum albumin concentration and the increased platelet aggregation response to this agent. Addition of albumin in vitro decreased the amount of TxB2 generated for a given dose of NaAA and increased NaAA and collagen‐induced platelet aggregation thresholds. However, albumin had no significant effect on collagen‐induced TxB2 production.The results suggest that the hypoalbuminaemia and associated reduced binding of arachidonic acid and increased synthesis of TxA2 account in part for the increased platelet aggregability seen in the nephrotic syndrome but that other mechanisms are also involved.
HLA‐DA monoclonal antibodies inhibit the proliferation of normal and chronic granulocytic leukaemia myeloid progenitor cell
Authors: Goldman John M. , Hibbin Jill , Kearney Lyndal , Orchard Kim , Th'ng K. H. 
Published: March 2008

Summary. We studied the capacity of murine monoclonal antibodies with HLA‐DR specificity to inhibit the proliferation in vitro of erythroid (BFU‐E and CFU‐E) and granulocyte‐macrophage (CFU‐GM) progenitor cells in normal bone marrow and the blood of patients with chronic granulocytic leukaemia (CGL). Two IgG2 antibodies (CA 2.06 and L243) inhibited the proliferation of normal BFU‐E and CFU‐GM at relatively high dilution; a third antibody, DA2, had no effect on either progenitor cell. A complement‐king monoclonal antibody with T‐cell activity (OKT3) produced only minor reduction in progenitor cell proliferation. Further studies with L243 showed that BFU‐E and CFU‐GM from the blood of patients with CGL were inhibited to the same degree as normal marrow progenitor cells. The inhibition of progenitorcell proliferation by a given antibody was always complement dependent and was therefore presumed to be due to a direct cytotoxic effect. The inhibitory effect of monoclonal antibodies is a valuable approach to the characterization of antigenic determinants on myeloid progenitor cells and the differential cytotoxicity of selected monoclonal antibodies might be exploitable for therapy.
Treatment of aplastic anaemia with antilymphocyte globulin (ALG)
Authors: Fairhead S. M. , Chipping P. M. , Gordon‐Smith E. C. 
Published: March 2008

Summary.Thirty‐one patients with aplastic anaemia were treated with antilymphocyte globulin (ALG) followed by anabolic steroids. All patients were reviewed at least 1 year after receiving treatment. Twenty‐one patients were considered to have a severe form of aplasia. ALG from three sources was used during the trial. Sixteen patients were alive at the time of analysis, eight who had had severe aplastic anaemia (38%) and five of these no longer require blood support. No difference in response was found according to type of ALG used. Patients with a long history of aplasia before treatment fared worse than those with a short history. These results were better than those obtained with anabolic steroids alone in historical controls. It is concluded that ALG treatment is worth giving to patients with aplastic anaemia but that the optimum way of giving this immunosuppression may be different from that used in this presentation.
Studies of the heterogeneity of antithrombin III concentrates
Authors: Barrowcliffe T. W. , Eggleton C. Anne , Mahmoud M. 
Published: March 2008

Summary.The biological activities of antithrombin III (At III) concentrates, prepared by several manufacturers for clinical use, have been compared by three assay methods, and their heparin‐binding properties studied by crossed immunoelectrophoresis and heparin affinity chromatography. Concentrates from two of the four manufacturers showed discrepancies between assay methods, with concentrations by heparin co‐factor assays significantly lower than those by immunological and progressive antithrombin methods. Heterogeneity was also found by heparin binding studies, with about half the total At III antigen in these concentrates being unable to bind to heparin. These results confirm previous findings of heterogeneity in At III concentrates and show that some concentrates contain substantial amounts of altered At III molecules in which the heparin‐binding site has been denatured but the thrombin‐neutralizing site left largely intact.
Biosynthesis of ferritin subunits from different cell lines of HL‐60 human promyelocytic leukaemia cells and the release of acidic isoferritin‐inhibitory activity against normal granulocyte‐macrophage progenitor cells
Authors: Dörner Marianne H. , Broxmeyer Hal E. , Silverstone Allen , Andreeff Michael 
Published: March 2008

Summary.Biosynthesis of acidic isoferritins was investigated in human promyelocytic HL‐60 cells, characterized by diploid (2C), tetraploid (4C) and mixed diploid–tetraploid (2C–4C) DNA cell lines. The three cell lines were studied for the biosynthesis of ferritin and its subunits and for the release of acidic isoferritin‐inhibitory activity against normal CFU‐GM before and after addition of DMSO. While the tetraploid and mixed diploid–tetraploid cell lines synthesized more H‐ (Mr= 21) than L‐subunits (Mr= 19) after induction, the tetraploid line synthesized more H‐subunit before and after induction, compared to the diploid line. The release of acidic isoferritin‐inhibitory activity was greater before than after induction in both cell lines, but the tetraploid cell line released more acidic isoferritin‐inhibitory activity consistent with its greater production of Mr= 21 subunit. However, after induction no inhibitory activity could be detected from the diploid cells and much less activity was detected with the tetraploid cells, suggesting that differentiation caused a decrease in production of acidic isoferritin‐inhibitory activity.
Turnover of 131I‐human spleen ferritin in plasma
Authors: Cragg Susan J. , Covell Ann M. , Burch Anna , Owen Glynn M. , Jacobs Allan , Worwood Mark 
Published: March 2008

Summary.Human spleen ferritin was labelled with 131I and injected into two normal men. The labelled ferritin left the plasma rapidly. The experimental clearance curve could be fitted with accuracy into two single exponential functions. The first component, T1/2= 9 min, accounted for the clearance of about 90% of the labelled ferritin. Surface counting showed uptake of 131I by the liver but not by the spleen. Such a rapid plasma turnover is similar to that found after injection of tissue ferritins into experimental animals but contrasts with the slow turnover previously found for 131I‐labelled human plasma ferritin. Differential clearance of isoferritins from the plasma is an important factor explaining the biochemical and immunological differences between tissue and plasma ferritins.
Serum transcobalamin levels as an early indicator of bone marrow engraftment following transplantation
Authors: Naparstek E. , Rachmilewitz B. , Rachmilewitz M. , Fuks Z. , Slavin S. 
Published: March 2008

Summary.Three B12 binding proteins, the transcobalamins TCI, TCII and TCIII, were determined serially in the serum of five patients who underwent bone marrow transplantation. The increase in TCII, followed by the increase in TCI, proved to be an early indicator of bone marrow regeneration, reaching a peak of up to twice its normal levels at least 5 d prior to the rise of the peripheral white blood cell count.
Eosinophilic blast crisis in a case of chronic myeloid leukaemia
Authors: Marinone G. , Rossi G. , Verzura Paola 
Published: March 2008

Summary.A case of typical chronic myeloid leukaemia (CML) is described, of which the terminal blast crisis was characterized by an impressive proliferation of atypical eosinophils and by the simultaneous complete deficiency of neutrophilic myeloperoxidase. Since eosinophilic cells preserved a strong peroxidase positivity, a high number of immature non‐granular eosinophilic precursors could be recognized among the leukaemic blast cells, thus supporting the diagnosis of eosinophilic blast crisis of CML.
A good prognosis group in childhood acute lymphoblastic leukaemia
Authors: Stockley Robert J. , Ahlquist Patricia Y. , Mott Martin G. 
Published: March 2008

Summary.The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 were reviewed. The overall relapse free survival rate was 50%. However, girls presenting with a total white blood cell count of 20 × 109/1 or less (35% of all patients) had a particularly favourable prognosis with an 80% relapse‐free survival rate at 12 years. The difference in prognosis between the sexes is confirmed but this difference is confined to the low initial white cell count group. We suggest that girls in this group should be excluded from the more intensive arms of new treatment protocols.