bluuebunny/crossref_metadata_2025_split huggingface dataset:
Detection of cells of megakaryocyte lineage in haematological malignancies by immuno‐alkaline phosphatase labelling cell smears with a panel of monoclonal antibodies
Authors: Erber W. N. , Breton‐Gorius J. , Villeval J. L. , Oscier D. G. , Bai Y. , Mason D. Y. 
Published: March 2008

SummaryImmuno‐alkaline phosphatase staining (by the APAAP technique) has been used to identify promegakaryo‐blasts in cell smears from 10 cases of leukaemia (three acute leukaemia, seven blast transformations). In all cases pro‐megakaryoblasts were labelled by at least two anti‐platelet glycoprotein (gp) antibodies, the highest percentages being obtained with anti‐gp IIIa (antibody C17). HLA‐DR was expressed by a variable percentage of neoplastic cells in all cases, the T11 (CD2) antigen (sheep red cell receptor) in four of seven cases tested and the p150,95 antigen in three of the six cases tested. In some cases of acute myeloid leukaemia APAAP staining of blood smears revealed circulating promegakaryoblasts and micromegakaryocytes (which superficially resemble small lymphoid cells). It is concluded that immuno‐alkaline phosphatase staining of cell smears offers a convenient means of diagnosing acute megakaryoblastic leukaemia in the routine laboratory.
Chronic and subacute myelomonocytic leukaemia in the adult: a report of 60 cases with special reference to prognostic factors
Authors: Fenaux Pierre , Jouet Jean Pierre , Zandecki Marc , Lai Jean Luc , Simon Marc , Pollet Jean Paul , Bauters Francis 
Published: March 2008

SummaryWe report 60 cases of chronic and subacute myelomonocytic leukaemias (CMML and SMML) in the adult, using the FAB group criteria. The M/F sex ratio was 3·3 and the mean age 67·5 years. Splenomegaly was found in 32% of cases, hyperleucocytosis in 52% of cases and mean blood monocytosis was 4·3 × 109/l. Marrow smears showed an excess of blasts in 57% of patients, a moderate increase in monocytes in most cases and frequent myelodysplastic features. An increase in serum lysozyme and polyclonal hypergammaglobulinaemia were usual and clonal cytogenetic anomalies found in about half of the patients tested. Treatment was usually palliative and the median survival was 28 months, a blastic transformation being responsible for a third of the deaths.Prognostic factors at diagnosis were analysed retrospectively in the 46 patients who had sufficient follow up. Percentage of marrow blasts haemoglobin level and blood monocytosis at diagnosis, were subject to multivariate analysis, resulting in a discriminant ‘score’. This allowed assignment of each patient into one of two prognostic subgroups (10·9% probability of error): a poor prognosis one, with a life expectancy of less than 1 year and a high risk of acute transformation (subgroup termed SMML) and a better prognosis subgroup (termed CMML), with some CMML patients surviving over 5 years.
Electroblotting and immunohistochemical staining for identification of platelet antibodies
Authors: Rock G. , Decary F. , Tittley P. , Fuller V. 
Published: March 2008

Summary. The Western blot procedure has been adapted for use with a biotinylated antiglobulin reagent and a horseradish peroxidase complex (Vectastain ABC) to determine specific sites of antigen binding and permit discrimination between various types of platelet antibodies.Monospecific anti‐PlA1 antisera or sera containing mixtures of multispecific HLA and unidentified platelet specific antibodies were tested with PlA1 phenotyped platelets. Using monospecific anti‐PlA1, one intense band with relative molecular weight (Mr) of 88000 and corresponding to glycoprotein IIIa was seen with the PlA1+ platelets. With mixtures of antibodies, reactions were seen with platelet specific antibodies without interference from the HLA antibodies; with one serum a band of Mr approximately 135000 was identified with Baka+, but not Baka‐ platelets, indicating the presence of an anti‐Baka in the serum. The location of the Baka antigen corresponded to the area for GP IIb. With another serum, a band of Mr 88000 was revealed with PlA1‐and some PlA1+ platelets suggesting the presence of an anti‐PlA2.Two additional bands of Mr 160000 and 200000 were present on all preparations including autologous controls, probably due to the presence of non‐specific IgG. Thus, immunohistochemical staining is readily adaptable to the Western blot technique, and antibodies to platelet‐specific antigens can be easily differentiated from HLA antibodies.
Red‐cell GSH regeneration and glutathione reductase activity in G6PD variants in the Ferrara area
Authors: Anderson Barbara B. , Carandina Gianni , Lucci Marco , Perry Gillian M. , Vullo Calogero 
Published: March 2008

Summary. Red‐cell studies were carried out on three groups of G6PD‐deficient subjects with different G6PD variants from the Ferrara area of Northern Italy. Red‐cell GSH and activities of G6PD, glutathione reductase (GR), glutathione peroxidase (GPx) and superoxide dismutase (SOD) were measured. A method was developed to measure red‐cell GSH regeneration after oxidation of endogenous GSH in whole blood by diamide and only this clearly distinguished the variants from each other and from normal. Regeneration by 1 h was lowest in the Mediterranean variant, 0–10.2% in contrast to 93–98% in normal. A predisposition to a haemolytic crisis after ingestion of fava beans was not clearcut, but subjects appeared to be at risk if GSH regeneration at 1 h was less than 30% of the endogenous level, and red‐cell FAD+ was very high indicated by high in vitro GR activity and inhibition by added FAD+.It is suggested that the most informative tests in G6PD deficiency are measurements of GSH regeneration in intact red cells plus GR activity and/or red‐cell flavin compounds.
Effect of human recombinant erythropoietin on human marrow megakaryocyte colony formation in vitro
Authors: Dessypris Emmanuel N. , Gleaton John H. , Armstrong Otho L. 
Published: March 2008

Summary.The effect of human recombinant erythropoietin (EP) on the growth of human marrow megakaryocyte colony forming units (CFU‐M) in vitro was investigated by the use of a plasma clot assay. EP as a single stimulating factor or as an additional factor to optimal concentration of leucocyte conditioned medium (PHA‐LCM) had no effect on the number of CFU‐M derived colonies. However, addition of EP (O·5‐1 U/ml) to cultures with suboptimal concentrations of PHA‐LCM increased megakaryocyte colony formation by 50‐90% but had no effect on the number of granulocytic‐monocytic colonies (CFU‐GM). Exposure of marrow cells to EP for 24‐48 h in liquid suspension cultures, followed by removal of the hormone and assaying the cells for CFU‐M in plasma clots, resulted in a 50‐100% increase of megakaryocyte colony formation in vitro. The augmenting effect of EP on CFU‐M growth in vitro was abolished when EP was added to the medium after the third day of culture. The presence of factors in human serum and in PHA‐LCM was an absolute requirement for the hormone to exert its potentiating effect on human CFU‐M growth in vitro. Recombinant EP potentiates the growth of human marrow CFU‐M and this effect seems to be exerted during the early stages of CFU‐M development in vitro.
An improved acidified glycerol lysis test (AGLT) used to detect spherocytes in pregnancy
Authors: Acharya J. , Ferguson I. L. C. , Cassidy A. G. , Grimes. A. J. 
Published: March 2008

Summary.The acidified glycerol lysis test (Zanella et al, 1980) had proved unreliable in our hands. We now report the test to be stable and reliable when conducted at 37°C rather than at ambient laboratory temperature as used by earlier workers. With this modification, blood samples were tested from 167 pregnant subjects. Forty‐eight were less than 26 weeks pregnant and gave normal AGLT values (T50 > 1800 s) while of 119 more than 26 weeks pregnant 57 (48%) gave shortened T50 values indicative of the presence of spherocytes.
The role of intensive remission induction and consolidation therapy in patients with acute myeloid leukaemia
Authors: Tricot G. , Boogaerts M. A. , Vlietinck R. , Emonds M. P. , Verwilghen R. L. 
Published: March 2008

SummarySixty‐one patients with AML, 59 adults and two children, were treated with intensive remission induction and consolidation therapy. The median age was 36 years. Forty‐four (72%) patients entered complete remission (CR): 11 patients received a bone marrow transplantation. The median survival of complete remitters was 26.5 months; the probability of remaining in CR at respectively 1 and 2 years was 75% and 62%. The only factor significantly correlated with the outcome of remission induction, survival and duration of CR was age. Patients < 30 years fared significantly better than those 30 years or older; no difference in outcome was observed between patients aged 30‐50 and those over 50 years. In patients < 30 years the CR rate was 95%; 75% of them were still alive at 2 years and only one (5%) has relapsed. In contrast, in patients 30 years or older the CR rate was 60% and the median survival only 11.5 months. 50% of the complete remitters in this age group have relapsed. Morbidity from intensive consolidation therapy was considerable; more than 50% of consolidation courses were complicated by high fever, needing urgent admission; only four (3%) courses had a fatal event. It is concluded that intensive consolidation therapy may be considered as a major advance in the treatment of younger patients with AML. while its role in older individuals remains questionable. A possible explanation for the completely different outcome in younger and older patients with AML is discussed.
A randomized trial of 13‐cis retinoic acid with or without cytosine arabinoside in patients with the myelodysplastic syndrome
Authors: Clark R. E. , Ismail S. A. D. , Jacobs A. , Payne H. , Smith S. A. 
Published: March 2008

SummaryNinety‐eight consecutive patients with myelodysplastic syndrome were randomized to a treated or a control group, both receiving conventional supportive therapy. The treated group were given 13‐cis‐retinoic acid 20 mg/d if marrow blasts were ≦ 5% or cytosine arabinoside 10 mg/d subcutaneously on 6 d /week if marrow blasts were 6‐30%, to which retinoic acid was added after 12 weeks. Serum levels of the drugs in the treated group were similar to those that would produce inhibition of CFU‐GM growth in vitro. In patients in the low blast group receiving retinoic acid. myeloid surface antigens reverted from an abnormal to a normal pattern.Log rank analysis carried out after 25 months showed no significant difference in survival between the treated and control group, either in the total patient population or in the high and low blast groups considered separately. However, analysis of 39 non‐sideroblastic patients with ≦ 5% blasts showed an increase in survival in the treated group.
Aberrant pattern of red cell membrane and cytosolic proteins in a case of congenital dyserythropoietic anaemia
Authors: Pothier Brigitte , Morlea Laurette , Alloisio Nicole , Ducluzeau Marie‐Thearegse , Feao Claude , Blanchard Dominique , Cartron Jean‐Pierre , Garbarz Michel , Chaveroche Isabelle , Boivin Pierre , Favre‐Gilly Jean , Delaunay Jean 
Published: March 2008

SummaryWe report an unusual case of congenital dyserythropoietic anaemia (CDA). The propositus is a 25‐year‐old gipsy female presenting with a recessively inherited haemolytic anaemia. The diagnosis of CDA was based on erythrokinetic data and the morphological appearance of the erythroid precursors. The direct assay of HEMPAS antigen was negative. In peripheral blood there were 15% dacryocytes. The red cell membrane protein pattern was dramatically altered, with four major aberrant bands. Band a (mol wt 86 000) was at the lower edge of band 3, band b (mol wt 82 000) was below band 3, band c (68 000) and band d (67000) were below band 4.2. In addition, there was an array of aberrant minor bands below band d. Gel densitometric determinations and immunological characterization showed that these bands did not derive from any of the major components of the membrane. In fact, membrane proteins appeared normal in many respects, although periodic acid‐Schiff staining revealed an apparent decrease of sialoglycoproteins. The major aberrant bands a, b and c occur in very low amounts in controls. These bands, as well as band d, also exist in normal cytosol and were strongly increased in the propositus.
The lectin leucoagglutinin binds specifically to human granulocytes, monocytes and tissue mast cells: further evidence for a common origin of the three cell types
Authors: Schumacher U. , Horny H. P. , Welsch U. 
Published: March 2008

SummaryThe lectin leucoagglutinin (LA) binds to the granules of all forms of granulocytes as well as mast cells and monocytes, which is considered to be a further indication for a common origin of these cells.
A specific radioimmunoassay for 5′‐deoxyadenosyl cobalamin in serum
Authors: Quadros Edward V. , Rothenberg Sheldon P. , Polu Sailaja 
Published: March 2008

A specific radioimmunoassay for 5′‐deoxyadenosyl cobalamin (ado‐Cbl) has been developed using an antiserum raised to this cobalamin (Cbl). At a 1:100 or greater dilution the antiserum did not bind radiolabelled methyl‐hydroxo‐, sulphito‐ or cyano‐Cbl and these Cbl analogues did not compete in the radioimmunoassay even at 100‐fold higher concentration.The serum concentration (range and mean ± SD) of ado‐Cbl in 30 normal subjects was 47–134 and 81 ± 16 pg/ml. The corresponding values for total Cbl in these sera were 189–610 and 355 ± 144 pg/ml, and the computed values for methyl‐Cbl were 142–476 and 274 ± 127 pg/ml. The coefficient of variation was substantially greater for methyl‐Cbl than for ado‐Cbl (46% v. 21%, respectively). The ado‐Cbl concentration was in the normal range (75–95 pg/ml) in five healthy subjects with a low normal concentration (189–217 pg/ml) of total Cbl. In two subjects with low total Cbl (118 and 170 pg/ml) and without any clinical evidence of Cbl deficiency, ado‐Cbl was normal (63 and 95 pg/ml. respectively). Thus, in this group, low methyl‐Cbl accounted for the lower total Cbl.The concentration (mean ± SD) of serum ado‐Cbl and methyl‐Cbl in six patients with low total Cbl (57 ± 25 pg/ml) and clinical evidence for Cbl deficiency was 35 ± 12 pg/ml and 22 ± 22 pg/ml, respectively. This difference from the normal mean for each cofactor was highly significant (P<0.001). The decrease in the concentration of methyl‐Cbl in Cbl deficiency was relatively greater than the decrease in ado‐Cbl (92% v. 57%, respectively) which raised the relative concentration of ado‐Cbl in Cbl deficiency to 61% of the total Cbl. Although a low serum methyl‐Cbl is a sensitive indicator of Cbl deficiency, it may not be as specific as a decrease in serum ado‐Cbl. Factor(s) other than tissue stores of Cbl may lower serum methyl‐Cbl below the 95% confidence limit in the absence of clinical Cbl deficiency.
Serum immunoreactive erythropoietin in healthy normal children
Authors: Hellebostad M. , Hågå P. , Cotes P. Mary 
Published: March 2008

To provide reference data for normal children, serum immunoreactive erythropoietin (siEp) was estimated by radioimmunoassay in samples from 130 healthy children, 57 girls and 73 boys, with ages between 1 month and 16 years. In 128 of the children the (geometric) mean siEp was 15.8 miu/ml (iu: international units) with 95% range (the range within which 95% of the observations are predicted to fall) 9.1–27.6 miu/ml. There was no relation between siEp and the variables haemoglobin (Hb), PCV, age and sex. There were two outliers, both girls, aged 9.5 and 9.8 years. in whom siEp was >256 miu/ml. In both, Hb and PCV were normal and we are unable to account for these atypical findings.Estimates of siEp in the 128 children were not significantly different from those in 22 healthy adults investigated simultaneously (mean 16.2 miu/ml, with 95% range 11.2–23.3 miu/ml).
Radioimmunoassay for quantitative measurement of factor VIII‐heavy chain
Authors: Nordfang O. , Ezban M. , Nilsson P. , Knudsen J. B. 
Published: March 2008

Summary An immunoassay has been developed for the measurement of factor VIII heavy chain (FVIII‐HC). IgG from a human inhibitor plasma with specificity for FVIII‐HC and FVIII‐light chain (FVIII‐LC) was coated to microplates with loose wells. After washing, the plates were incubated with sample and after another wash 125I‐FVIII‐HC was added so that the amount of bound tracer was dependent on the amount of FVIII‐HC in sample. When EDTA was included in the assay buffer the assay response was increased 3‐fold for normal human plasma. This indicated that the antibody was reactive with a determinant hidden in the FVIII‐HC/FVIII‐LC complex as EDTA splits the complex. The sensitivity of the assay was 0.004 units/ml (1 unit/ml in normal human plasma pool).Together with a previously published assay for FVIII‐LC it has now become possible to measure the relative amounts of FVIII‐HC and FVIII‐LC in haemophilia A plasma and to study the separate expression of FVIII‐HC and FVIII‐LC by recombinant DNA technology. Patients with severe haemophilia A had FVIII‐HC levels below 0.01 units/ml. However, inhibitor patients in high dose FVIII‐treatment showed up to 50 times higher levels of FVIII‐HC than FVIII‐LC and FVIII:C, indicating the presence of FVIII/anti‐FVIII‐LC immune complexes. Thus, dependent on assay specificity plasma samples can show very variable content of FVIII:antigen.
In vivo studies on the relationship between intestinal iron (Fe3+) absorption, hypoxia and erythropoiesis in the mouse
Authors: Raja K. B. , Simpson R. J. , Pippard M. J. , Peters T. J. 
Published: March 2008

Summary The effect of hypoxia and changes in erythropoiesis on the absorption of 59Fe3+ from in situ tied‐off duodenal segments was studied in the mouse. Hypoxia led to an increase in mucosal uptake within 6 h, whilst mucosal transfer was unaffected for about 20 h, suggesting independent regulation of these two processes. Hypoxia (3 d) stimulated erythropoiesis and resulted in a 2–3‐fold increase in the total mucosal uptake of 59Fe. Conversely, hyperoxia (100% O2) caused a decrease in reticulocyte counts and the total mucosal uptake. The changes in the transfer of 59Fe from the mucosa to the body were more marked than changes in uptake in both hypoxia and hyperoxia.Mice subjected to subtotal nephrectomy showed a normal increase in the total mucosal uptake of 59Fe3+ following hypoxic exposure, despite the absence of any changes in the reticulocyte count. Obliteration of the erythroid tissue of animals by splenectomy and 89Sr treatment was accompanied by a marked decrease in the transfer of 59Fe from mucosa to the carcass. However, exposure of splenectomized 89Srtreated mice to hypoxia resulted in an increase in the total mucosal uptake and carcass transfer of 59Fe, without any change in erythropoiesis. These results indicate that hypoxia enhances mucosal iron uptake by a mechanism which is independent of stimulated erythropoiesis, but that changes in the rate of erythropoiesis have an additional effect, particularly on the transfer phase of iron absorption.
Production of a multipotential cell (CFU‐S) proliferation inhibitor by various populations of mouse and human macrophages
Authors: Pojda Z. , Dexter T. M. , Lord B. I. 
Published: March 2008

SummarySome bone marrow macrophages from a variety of animal species synthesize a proliferation inhibitor which specifically inhibits the flow of murine CFU‐S into DNA‐synthesis. Macrophages from other sources have now been assayed for their capacity to produce this inhibitor. Macrophages from mouse blood, marrow, spleen and peritoneum and human blood were cultured and their ability to produce inhibitor assayed against proliferating CFU‐S by the tritiated thymidine suicide assay. Inhibitor was observed in all mouse and most human preparations though with varying degrees of activity, showing functional heterogeneity in the development of macrophages in different tissues. Peripheral blood from both mouse and human showed activity approaching that of fresh marrow. This confirms that mouse assay systems can be used to study the human counterpart of the mouse CFU‐S proliferation inhibitor and that blood is a convenient source of material for purification and characterization studies.
Dendritic reticulum cell pattern as a microenvironmental indicator for a distinct origin of lymphoma of follicular mantle cells
Authors: Manconi Riccardo , Poletti Alessandro , Volpe Rachele , Sulfaro Sandro , Carbone Antonino 
Published: March 2008

SummaryFollicular dendritic reticulum cells (DRCs) are known to be normally present in primary follicles and both follicular centres and mantle zones of secondary follicles of peripheral lymphoid tissue. Involved frozen biopsy tissue specimens from eight cases of intermediate lymphocytic lymphoma/mantle zone lymphoma (ILL/MZL); eight cases of follicular centre cell lymphomas (FCCL) of the centroblastic/ centrocytic type; and seven cases of well‐differentiated lymphocytic lymphoma (WDLL) consistent with chronic lymphocytic leukaemia (CLL) were analysed immunohistologically with R4/23 (DRC‐1) monoclonal antibody reactive with ‘bystander’ DRCs. As opposed to FCCL and most WDLL/CLL cases, the DRCs consistently formed a loose, ill‐defined meshwork with a radiating or blurred outline in all MZL cases and one ILL. On the basis of the observed findings as well as from those reported in literature, the hypothesis is proposed that ILL/MZL originates from the follicular mantle zone and represents a distinct lymphoma entity owing to its peculiar immunostaining pattern of DRCs that allows it to be separated from both FCCL and WDLL/CLL. Moreover, the absence of DRCs in the microenvironment of other B‐cell malignancies such as prolymphocytic leukaemia and hairy cell leukaemia, analogously with most CLL cases, would speak in favour of their different—possibly extrafollicular— compartment of origin within lymphoid tissue.
Acquired type II von Willebrand's disease: demonstration of a complexed inhibitor of the von Willebrand factor‐platelet interaction and response to treatment
Authors: Goudemand J. , Samor B. , Caron C. , Jude B. , Gosset D. , Mazurier C. 
Published: March 2008

SummaryAn acquired von Willebrand's disease developed in two patients in association with a monoclonal gammopathy plus a Sjögren's syndrome and a chronic lymphocytic leukaemia (CLL). In both cases a plasma inhibitor to von Willebrand factor (vWf) was suspected and characterized after plasma gel filtration. The inhibitor was shown to be entirely complexed with vWf and was only demonstrated after complex dissociation by heating. The inhibitor was able to inhibit the binding of125I‐vWf to platelets in the presence of ristocetin in both cases and to thrombin‐stimulated platelets in one case. In the two patients, the highest molecular weight multimers (HMWM) of vWf were absent when assessed by sodium dodecyl‐sulphate agarose plasma electrophoresis. Intravenous infusion of 1‐deamino‐(8‐D‐arginine) vasopressin (DDAVP) resulted in the appearance of the HMWM in both cases and of the satellite bands of each multimer subunit which were lacking prior to the infusion in one patient. After transfusion of a VIII/vWf concentrate containing a significant amount of HMWM, there was a rapid plasma clearance of the vWf‐related activities and of the HMWM when compared to that seen in a patient with type III constitutional vWD. We conclude that in the two patients studied the coagulation defect was related to the presence of a circulating inhibitor to vWf which could be responsible for the disappearance of the HMWM from plasma.