bluuebunny/crossref_metadata_2025_split huggingface dataset:
Reduced risk of non‐A, non‐B hepatitis after a first exposure to ‘wet heated’factor VIII concentrate
Authors: Kernoff P. B. A. , Miller E. J. , Savidge G. F. , Machine S. J. , Dewar M. S. , Preston F. E. 
Published: March 2008

SummaryThe risk of post‐infusion non‐A, non‐B hepatitis (NANBH) in patients receiving a first exposure to unheated or conventionally ‘dry heated’factor VIII concentrates approaches 100%. implying invariable contamination of these products. Amongst 18 patients who received a first treatment with a ‘wet heated’commercial concentrate, five (28%) developed asymptomatic NANBH, suggesting a more efficient inactivation of NANB agent(s) by this process. 2/9 (22%) of the batches of concentrate used in the study were implicated in NANBH transmission. One of these two batches. responsible for NANBH in four patients, had been prepared from a plasma pool containing an unusually large proportion of donations with high alanine aminotransferase (ALT) levels. A resulting high level of viral contamination in this batch may have been sufficient to override the effects of the sterilization process. All patients remained anti‐HIV seronegative at 17–28 months of follow‐up.
Clotting factor levels and the risk of diffuse microvascular bleeding in the massively transfused patient
Authors: Ciavarella D. , Reed R. L. , Counts R. B. , Baron L. , Pavlin E. , Heimbach D. M. , Carrico C. J. 
Published: March 2008

SummaryClotting factor activities and coagulation screening tests in 36 massively transfused patients were measured after every 12 units of blood and whenever diffuse microvascular bleeding (MVB) developed. Moderate deficiencies in clotting factors were common, but they were not associated with MVB. MVB was associated with severe abnormalities of coagulation, i.e. a fibrinogen level <0.5 g/l or clotting factor levels <20%.The quantitative relationship between the prothrombin (PT) and partial thromboplastin (PTT) times and underlying clotting factor levels was explored by multiple linear regression. Clotting factor levels accounted for only 65–85% of the variability in these tests. However, clotting factor activities less than 20% were reliably reflected by marked prolongations of the PT and PTT (values greater than 1.8 times control).Our data suggest that commonly used replacement formulas are not likely to prevent MVB, since consumption of platelets and/or clotting factors, rather than simple dilution, is a major cause of the deficiencies leading to MVB. Modified whole blood alone was sufficient replacement therapy for most patients. Guidelines for transfusion of supplemental components during massive transfusion are given.
Haemopoietic reconstitution after allogeneic bone marrow transplantation in man: recovery of haemopoietic progenitors (CFU‐Mix, BFU‐E and CFU‐GM)
Authors: Ma David D. F. , Varga Dianne E. , Biggs James C. 
Published: March 2008

SummaryHaemopoietic reconstitution was evaluated in 44 patients given HLA compatible sibling bone marrow transplants. The mean peripheral blood haemoglobin, neutrophil and platelet counts were markedly reduced early post‐graft but returned to normal by 26 weeks post transplant. Bone marrow multipotent (CFU‐Mix), erythroid (BFU‐E) and myeloid (CFU‐GM) progenitor cell reconstitution were also assessed at regular intervals up to 2 years post‐graft. The mean value of CFU‐GM increased gradually and attained a normal value by 52 weeks. The BFU‐E value did not reach a normal value until after 52 weeks post‐graft. However, CFU‐Mix growth appeared to be impaired even up to 2 years post‐transplant. The occurrence of graft versus host disease at 3 months post‐transplant was associated with significantly lower mean numbers of platelets, marrow CFU‐GM, BFU‐E and CFU‐Mix. Post‐transplant patients who were on methotrexate therapy were also shown to have lower marrow CFU‐GM and neutrophil values compared to those patients who received cyclosporin post‐transplant. This study demonstrated that although peripheral blood counts were normal after 26 weeks post‐graft, marrow stem cell reserve in these patients was reduced. This might in part explain the documented increase in risk of severe infections or thrombocytopenia in some of these patients, particularly during viral infection, graft‐versus‐host disease or immunosuppressive treatment.
Rearrangement for the T‐cell receptor gene and co‐expression of immature T‐cell markers and natural killer cell phenotype, in a patient with acute lymphoblastic leukaemia
Authors: Pizzolo G. , Trentin L. , Vinante F. , Agostini C. , Zambello R. , Ranucci A. , Luca M. , Chilosi M. , Dazzi F. , Foa R. , Caligaris‐Cappio F. , Perona G. , Semenzato G. 
Published: March 2008

SummaryWe describe a patient with acute lymphoblastic leukaemia whose blasts co‐expressed immature T‐cell markers and nearly the entire phenotypic repertoire of NK cells. The T‐cell nature of the proliferating blasts was proven by the demonstration of the rearrangement for the β‐chain of the T‐cell antigen receptor. Although an abnormal phenotypic expression related to the neoplastic proliferation cannot be formally excluded, it is possible that the cells in this patient may represent the clonal expansion of a normal subpopulation of T‐cell lineage NK‐related cells frozen at an early stage of differentiation. These features provide arguments for discussing the controversial issue of the ontogeny of NK cells and their relationship to the T‐cell lineage.
The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia
Authors: Melo Junia V. , Catovsky Daniel , Gregory Walter M. , Galton David A. G. 
Published: March 2008

SummaryThe prognostic value of biological, clinical and laboratory features was analysed in a series of 265 patients with chronic lymphocytic leukaemia (CLL) and prolymphocytic leukaemia (PLL). On univariate analysis seven features were shown to influence significantly the survival of the whole group of patients: absolute prolymphocyte count (ABS PROL), percentage of prolymphocytes (%PROL), WBC, spleen size, age, intensity of surface‐membrane immunoglobulin (SmIg) and mouse (M) rosettes. Multivariate regression analysis of these features showed that only ABS PROL and spleen size had independent prognostic significance. The survival in PLL (38 cases) was significantly shorter than in CLL (227 cases) (median survival=3 and 8 years, respectively). Patients with CLL with an increased %PROL (11–55%), defined as CLL/PL, could be divided into two groups: those with ABS PROL ≤ 15 × 109/1 (26 cases) fell within the ‘standard‐prognostic risk’ for typical CLL (i.e. ≤ 10% PROL), whereas the survival outlook for the cases with ABS PROL > 15 × 109/1 (40 cases) was as bad as for PLL. A scoring system was generated with the four features that showed high prognostic significance: ABS PROL, spleen size, SmIg and M‐rosettes. The score proved to be superior to any single feature as a predictor of survival, being especially useful in the analysis of the CLL/PL group: cases with high scores (>2) had a median survival of 2·5 years, while the median has not been reached for those with low scores (≤2). We suggest that this scoring system may help to identify the cases of CLL/PL that behave as PLL, and as such may benefit from different treatment.
The spleen and haemolysis: evaluation of the intrasplenic transit time
Authors: Ferrant A. , Leners N. , Michaux J. L. , Verwilghen R. L. , Sokal G. 
Published: March 2008

SummaryThe mean intrasplenic red cell transit time (STT) and the slow mixing splenic red cell volume (SSV) have been measured in patients with hereditary spherocytosis (HS), autoimmune haemolytic anaemia (AIHA) and lymphoproliferative disease (LD). There was an inverse relationship between the mean red cell life span (MRCLS) and the STT in HS (r=–0·96, P<0·001) and in AIHA (r=–0·90, P<0·001). No such relationship existed in LD. The size of the spleen and the SSV were not related to the severity of haemolysis. Our data offer strong evidence for the conditioning effect of the spleen on HS‐ and AIHA red cells and suggest that the STT is an index of the adverse effect of the spleen on red cells in patients with HS or AIHA.
Chromatographic analysis of human erythrocyte pyrimidine 5′‐nucleotidase from five patients with pyrimidine 5′‐nucleotidase deficiency
Authors: Hirono Akira , Fujii Hisaichi , Natori Hideyo , Kurokawa Izumi , Miwa Shiro 
Published: March 2008

SummaryHuman erythrocyte pyrimidine 5′‐nucleotidase (P5N) was separated into two subclasses, P5N‐I and P5N‐II, by DEAE Bio‐Gel A column chromatography. Their enzymological properties were studied using five normal subjects and five patients with different P5N deficiencies. Study of the normal subjects showed that P5N‐I and P5N‐II have distinctive properties, and P5N‐II is similar to the 5′‐nucleotidase in rat liver cytosol. The P5N‐II from the five subjects with this deficiency had normal activity and other normal enzymological properties. However, the P5N‐I from these patients had abnormal properties, including reduced activity. These variant enzymes had a high Michaelis constant for substrate cytidine 5′‐monophosphate and were heat stable. The optimum pH was shifted towards the acidic side in two patients, towards the basic side in one, and was unchanged in the other two. These results strongly suggest that the main cause of P5N deficiency is an abnormality of P5N‐I, probably arising from a structural gene mutation.
A preparation for calibrating the assay of the blood group antibody anti‐c
Authors: Phillips P. K. 
Published: March 2008

SummaryA freeze‐dried, working calibration preparation (84/628) has been assayed against the International Incomplete anti‐c Reference Serum (67/160) in a multi‐centre study. The use of 84/628 will avoid the between‐laboratory variation, inherent in the use of local preparations, in the reporting of assays of anti‐c using the AutoAnalyzer. A value of 13 IU/ampoule has been assigned to the anti‐c content of preparation 84/628 which is available from the Blood Group Reference Laboratory, Oxford.
Demonstration of abnormal factor VIII multimers in acquired von Willebrand's disease associated with a circulating inhibitor
Authors: Ball J. , Malia R. G. , Greaves M. , Preston F. E. 
Published: March 2008

SummaryWe have studied the factor VIII multimeric structure in four patients with acquired von Willebrand's disease associated with a circulating inhibitor to the factor VIII complex. In three of the four patients tested, the high and medium molecular weight bands were absent when assessed by sodium dodecyl sulphate–agarose electrophoresis. Plasma from the fourth patient contained all the factor VIII multimeric forms, although the high molecular weight bands were markedly decreased in concentration. Intravenous infusion of 1‐deamino‐(8‐d‐arginine)‐vasopressin (DDAVP) resulted in the appearance of a full complement of multimers in the plasma of the two patients tested. This response was, however, transient and a return to pre‐infusion multimeric composition occurred within 2–4 h.In the patients studied, an inhibitor to the factor VIII complex has induced an acquired variant von Willebrand's disease which transiently corrects after the infusion of DDAVP.
Changes in the distribution and organization of platelet actin induced by diamide and its functional consequences
Authors: Spangenberg P. , Till U. , Gschmeissner S. , Crawford N. 
Published: March 2008

Summary. Exposure of blood platelets to diamide (azodicar‐boxylic acid‐bis‐dimethylamide) results in oxidation of sulphydryl groups present in the cytoskeleton and other proteins. This results in dramatic changes in functional behaviour of the cells.The distribution and level of organization of the major cytoskeletal protein actin has been studied analytically by the DNase‐I inhibition assay and morphologically by electron microscopy (EM) of Triton X‐100 treated platelets adherent to EM grids. Exposure to diamide results in a redistribution of actin within the cell reflected in an increase in cytoskeletal F‐actin and a concomitant decrease in cytosolic actin. The magnitude of these changes depends upon the concentration of diamide and the time of exposure. Diamide also alters platelet aggregatory functions in response to certain stimuli.Treatment of normal human platelets with 0.1 mM diamide proceeds via disaggregation (5 min exposure to diamide), inhibition of aggregation (30 min exposure), to finally a normalization of the aggregation response after 60–120 min incubation with diamide. In parallel with the return to full functional response the distribution of F‐actin between the cytoskeleton and cytoplasmic compartments returns to the control pattern. Incubation of the platelets with 0.5 mM diamide for 60 or more minutes leads to total inhibition of the aggregatory ability. In these cells the cytoskeleton associated F‐actin remains significantly elevated and the structural organization of the cytoskeleton is markedly altered. In contrast to the network of filaments subadjacent to the surface membrane seen in unstimulated platelets, the cytoskeleton now shows electron dense zones in the more central parts of the cytoplasm. This diamide‐induced structural reorganization of platelet cytoskeletal elements, associated with the inhibition of functional responses, emphasizes the dynamic nature of the membrane‐cytoskeletal axis and its importance in the expression of shape changes and aggregatory phenomena in response to surface stimuli.
Familial myelodysplasia: progressive disease associated with emergence of monosomy 7
Authors: Paul B. , Reid M. M. , Davison E. V. , Abela M. , Hamilton P. J. 
Published: March 2008

Summary. Two brothers developed hypoplastic anaemia with the development in one of refractory anaemia with excess blasts (RAEB) accompanied by emergence of monosomy 7. Both brothers have a constitutional inversion of chromosome 1. Neither shows the increased chromosomal fragility of Fanconi's anaemia or its variants. This family is the third reported in which monosomy 7 has been found when leukaemic or preleukaemic transformation has occurred in patients with familial hypoplastic anaemia.
Bone growth and haemopoiesis: steroid reversible anaemia, myelofibrosis and increased bone formation in a child
Authors: Macdougall Lorna G. , Pettifor John M. , Patel Jayvant M. 
Published: March 2008

SummaryFactors regulating the interaction between bone marrow haemopoietic cells, stromal elements and bone growth are poorly understood. Disturbance in the equilibrium between these elements can occur as the result of metabolic bone disease, haematologic disorders, neoplasia and infections. The present report concerns a child with myelofibrosis, hypoplastic/dyserythropoietic anaemia, osteo‐blast proliferation and increased bone formation. A positive tuberculin skin test and elevated EB virus titre indicated previous exposure toMycobacterium tuberculosisand Epstein‐Barr virus. No active focus of infection was identified and no improvement occurred following anti‐tuberculous therapy. A dramatic improvement occurred on corticosteroid therapy. Reticulocytosis was followed by an increase in haemoglobin and platelets and a decrease in ESR. Bone marrow fibrosis resolved and the marrow was repopulated with normal haemopoietic tissue. The bone abnormalities improved both radiologically and histomorphometrically. Relapse occurred when steroids were discontinued. Bone marrow tissue culture supernate from the patient during the active phase of the disease inhibited colony formation by normal marrow mono‐nuclear cells. This was reversed by steroid therapy. It is postulated that EB virus may have triggered osteoblast proliferation with resultant bony and haematologic changes. Response to corticosteroids could be explained on the basis of suppression of osteoblast activity and correction of fibroblast mediated suppression of haemopoiesis.
Stromal stem cells (CFU‐f) in yolk sac, liver, spleen and bone marrow of pre‐ and postnatal mice
Authors: Van Den Heuvel Rosette L. , Versele Sigrid R. M. , Schoeters Greet E. R. , J. Vanderborght Oscar L. 
Published: March 2008

SummaryOur results demonstrate that in yolk sac, liver, spleen and femoral bone marrow of mice at ages ranging between 11 d of gestation and adult life, important changes in the stromal stem cell population (CFU‐f assay) occur which are correlated with haemopoiesis. (1) In the liver, spleen and bone marrow, high numbers of CFU‐f precede high haemo‐poietic stem cell values. (2) As haemopoiesis starts in the spleen. CFU‐f numbers in fetal liver are low. Similarly, CFU‐f numbers decrease in the spleen as bone marrow haemopoiesis starts. This suggests the existence of a migration stream of stromal stem cells. (3) In spleen and bone marrow, CFU‐f numbers decrease towards adult life as these organs maintain a stable haemopoietic activity.
Flow cytochemical patterns of white blood cells in human haematopoietic malignancies
Authors: Drewinko B. , Bollinger P. , Brailas C. , Moyle S. , Wyatt J. , Simson E. , Johnston D. , Trujillo J. M. 
Published: March 2008

SummaryPeripheral blood samples from 118 patients with acute leukaemia (68 untreated; 50 treated) were measured with the Technicon H‐6000 automated haematology analyser. This instrument provides, in addition to measurements of the classical haematology parameters (i.e. cell counts, haemoglobin concentration, etc.), a differential count on 104 WBC effected by means of flow cytochemistry (peroxidase content) and volume (light scatter) discrimination. Disregarding RBC and platelet counts and their volume distribution profiles, the most important diagnostic parameters for leukaemic disease were the WBC count, the WBC differential count, and the proportions of large unstained cells (LUC) and high peroxidase (HPX) cells obtained by the automated differential count as well as the mean value of the WBC peroxidase content distribution (MPA). Granulocytic leukaemias had lower MPA than normal and lymphocytic leukaemias had MPA values above normal. M1 leukaemias were also characterized by large proportions of LUC and low fractions of HPX, while M2 leukaemias showed low LUC with high HPX. M3 leukaemias had low LUC and very high HPX. M4 leukaemias had large LUC and‘monocytic’components and a modest fraction of HPX. M5 leukaemias had very large numbers of LUC,‘monocytes’and‘lymphocytes’and a normal HPX. For M1 leukaemia, the presence of less than 7% LUC following induction treatment was related to morphological changes of normal cells induced by chemotherapy while LUC above 10% usually indicated unsuccessful induction associated with the presence of residual blasts. If treatment was successful, M2 and M3 leukaemias characteristically decreased their HPX population. All M4 leukaemias studied by us failed to enter remission and continued to display high proportions of HPX and LUC. Similarly, most M5 leukaemias had a poor response to treatment and always showed a very high proportion of LUC. Untreated lymphocytic leukaemias demonstrated high LUC, normal HPX and a high proportion of‘lymphocytes’. Hairy cell leukaemias showed almost equal proportions of‘lymphocytes’and LUC. Successful chemotherapy of all lymphoid leukaemia entities was associated with rapid decreases in LUC. slower decrements of‘lymphocytes’and moderate and transient increments in HPX. Thus, flow cytochemistry can assist not only in the segregation of acute leukaemias along with FAB classification with nonmorphologic criteria, but also in the follow up of patients with these diseases.
Pattern of fibrinolytic parameters in patients with gastrointestinal carcinomas
Authors: Kirchheimer Johannes C. , Huber Kurt , Wagner Oswald , Binder Bernd R. 
Published: March 2008

SummaryChanges in the plasma levels of components of the fibrinolytic system have been investigated in 80 patients suffering from gastrointestinal carcinomas. Urokinase antigen (RIA), tissue‐type plasminogen activator antigen (ELISA) and plasminogen activator inhibitor (functional assay) were determined. patients with pancreatic and colorectal carcinoma and metastases as well as those without metastases revealed significantly increased plasma urokinase levels. Those with gall bladder or gastric carcinoma did not show significantly elevated urokinase antigen levels compared to age‐matched controls. Determination of tissue‐type plasminogen activator antigen in all four carcinoma groups did not reveal significant differences when compared to an age‐matched healthy control group. The concentrations of plasminogen activator inhibitor were significantly increased in all carcinoma groups: there being no differences between the patient groups with or without metastases. No correlations between the different parameters of the fibrinolytic system could be obained.
Pyrimidine nucleoside monophosphate kinase hyperactivity in hereditary erythrocyte pyrimidine 5′‐nucleotidase deficiency
Authors: Lachant Neil A. , Zerez Charles R. , Tanaka Kouichi R. 
Published: March 2008

Summary.The pyrimidine nucleoside triphosphates (CTP, UTP) increase in the pyrimidine 5′‐nucleotidase (P5N) deficient red blood cell (RBC) to a greater degree than do the pyrimidine nucleoside monophosphates (CMP, UMP). Pyrimidine nucleoside monophosphate (PNMP) kinase phosphorylates CMP and UMP to their respective phosphodiesters. We tested the hypothesis that increased PNMP kinase activity contributes to the disproportionate increase in CTP and UTP in the P5N deficient RBC. CMP and UMP kinase activities were increased in high reticulocyte (4.4±2.1 and 8.5±3.3 μmol/ml RBC per minute) compared to normal RBC (2.8±1.0 and 6.0±2.5 μmol/ml RBC per minute). P5N deficient RBC (n= 2) had significantly increased CMP and UMP kinase activities (14.0 and 26.5 ±mol/ml RBC per minute). UMP and CDP‐ethanolamine were able to increase the activity of CMP kinase in crude haemolysate and the activity of partially purified enzyme. Since the Km for CMP of CMP kinase was 33 μmol/1 in P5N deficient RBC and since the CMP concentration is 25‐90 μmol/1 in the P5N deficient RBC, the enzyme should be nearly saturated with CMP in the P5N deficient RBC. Thus, PNMP kinase hyperactivity appears to contribute to the disproportionate increase in CTP and UTP in the P5N deficient RBC.
Splenic haematocrit and the splenic plasma pool
Authors: Zhang B. , Lewis S. M. 
Published: March 2008

Summary.A method was established for estimation of plasma volume and splenic plasma pool by quantitative scanning using 113mIn labelled transferrin (TF). The method was used in 12 patients with various haematological disorders and degrees of splenomegaly. 113mIn‐TF consistently gave about 6% over‐estimation of plasma volume by comparison with the standard 125I HSA method. The splenic plasma pool ranged from 1‐2% to 11.4% of the total plasma volume. By concurrent measurement of splenic red cell pool the splenic haematocrit (SHct) was obtained: mean 0.51. SD 0.08: the SHct/PCV ratio was 1.21 (SD 0.31) and the SHct/body Hct ratio was 1.30 (SD 0.30). SHct was independent of PCV and body Hct but there was a trend to a lower SHct in cases where splenomegaly was more marked. Direct measurement of splenic plasma pool may help to elucidate the cause of increased total plasma volume in such patients.