bluuebunny/crossref_metadata_2025_split huggingface dataset:
An acute leukaemia augured before clinical signs by blood group antigen abnormalities and low levels of A and H blood group transferase activities in erythrocytes
Authors: Lopez M. , Bonnet‐Gajdos M. , Reviron M. , Janvier D. , Huet M. , Salmon Ch. 
Published: March 2008

SummaryA mixed field agglutination pattern with anti‐A reagents and very low levels of A and H blood group antigen specific transferase activities were found in the erythrocytes of a 4‐year‐old girl who presented no clinical signs of haematological disease. Blood and marrow examination displayed some features consistent with a moderate dysmyelopoietic state. 18 months later an acute myeloblastic leukaemia confirmed the suspected haematological malignancy.
Progesterone inhibits proliferation of human marrow colony forming cells (CFU‐GM) through increased prostaglandin production by marrow macrophages
Authors: Smith M. A. , Lucie N. P. , Ferguson M. M. , Mairs R. J. , Smith J. G. 
Published: March 2008

SummaryProgesterone inhibited proliferation of granulocyte/macrophage progenitor cells (GFU‐GM) in human bone marrow cultured in methylcellulose; the inhibition ranged from 8% at 3 x 10‐9 M, 22% at 3 x 10‐8 M to 38% at 3 x 10‐7 M progesterone in 14 non‐malignant marrows.Depletion ofmarrow monocytes/macrophages with a monoclonal antibody MO2, abolished the inhibitory effect of progestrone. However, production of colony stimulating activity (CSA0 did not appear to be affected, either in PHA stimulated peripheral blood or in bone marrow. indomethacin, a prostaglandin synthetase inhibitor, greatly reduced the degree of inhibition and the addition of prostaglandin E1 (PFE10 to culture in concentrations as low as 10‐12 M completely removed any additional inhibitory effect of progesterone. PGE1 is known to inhibit only those CFU‐GM bearing the Ia antigen; we observed that depletion of Ia positive CFU‐GM by either 24 h incubation or by monoclonal (I2) treatment, reduced or abolished progresterone mediated inhibition. this confirms that the mechanism of progesterone inhibition of CFU‐GM is mediated through increased PGE production by marrow macrophages, and subsequent inhibition by PGE of ia positive CFU‐GM.
The role of platelet membrane glycoproteins Ib and IIb‐IIIa in platelet adherence to human artery subendothelium
Authors: Sakariassen Kjell S. , Nievelstein Patricia F. E. M. , Coller Barry S. , Sixma Jan J. 
Published: March 2008

SummaryPlatelet adherence to human artery subendothelium in blood from eight normal subjects, four patients with Glanzmann's thrombasthenia (deficiency of platelet membrane glycoproteins IIb and IIIa: GPIIb‐IIIa), two patients with Bernard‐Soulier syndrome (deficiency of platelet membrane glycoprotein Ib: GPIb) and one patient with von Willebrand's disease (VWD subtype III, deficient in factor VHI‐von Willebrand factor: FVIII‐VWF) was compared at various wall shear rates (300, 500, 1000, 1800 and 2500 s‐1).
Serum sickness and haematopoietic recovery with antithymocyte globulin in bone marrow failure patients
Authors: Bielory Leonard , Gascon Pedro , Lawley Thomas J. , Nienhuis Arthur , Frank Michael M. , Young Neal S. 
Published: March 2008

SummaryWe have evaluated 3 3 patients with various bone marrow dyscrasias treated with horse antithymocyte globulin (ATG) (Upjohn) to determine the relationship of haematopoietic response to the occurrence of serum sickness. Patients received ATG intravenously over 10 or 28 d at a dose of 15 mg/kg/d. Total or partial haematological responses were noted in 12 of 33 patients. Twenty‐eight patients developed clinical signs of serum sickness 6–14 d after the first infusion of ATG, while five patients did not. Twenty‐five of these patients were evaluated by immunochemical assays for circulating immune complexes (Clq‐binding assay) and 21 patients for serum complement (C3, C4 and CH50 assay) levels. There was a direct correlation between increases in immune complex levels, decreases in serum complement levels, and the development of the clinical signs and symptoms and serum sickness. Twenty‐one of 28 patients who developed serum sickness failed to show haematological improvement. However, haematopoietic recovery occurred in all five patients who did not manifest serum sickness (P <0.05) and in four patients who failed to develop immune complexes. These findings indicate that the development of serum sickness is not required for a haematopoietic response with ATG and may indeed impair recovery.
Liberation of surface and internal platelet‐associated IgG (PA‐IgG) during platelet activation
Authors: Pfueller Sharron L. , David Rosemary 
Published: March 2008

SummaryThe relationship between platelet‐associated IgG (PA‐IgG) of intact platelets to that of lysed platelets has been studied using a competitive ELISA. In platelets from 20 normal individuals mean surface PA‐IgG constituted 3 5% of mean total PA‐IgG and showed a significant linear relationship with total PA‐IgG. In platelets from 61 patients with various forms of thrombocytopenia including that with immune causes, 38 showed a similar proportion of PA‐IgG on the surface after storage at 2°C, as seen in normal platelets, while in 23, levels of surface and total PA‐IgG were equal. Normal platelets activated with thrombin or calcium ionophore A2 318 7, also had levels of surface PA‐IgG close‐to total PA‐IgG. Supernatants of platelet suspensions activated with aggregating agents contained increased amounts of IgG and when the platelets were washed, both total and surface PA‐IgG were decreased. Liberation of IgG from platelets was slower than that of [14C]serotonin but was decreased by release reaction inhibitors. Platelets treated at 2°C with soluble heat‐aggregated IgG, which binds to the Fc γ receptor, showed increased surface PA‐IgG, but, after incubation at 37°C, although [14C]serotonin was released, PA‐IgG levels were no longer increased.
Chronic lymphoid leukaemia and hairy cell leukaemia due to chronic exposure to benzene: report of three cases
Authors: Aksoy Muzaffer 
Published: March 2008

SummaryThree cases of chronic leukaemia, two with chronic lymphoid and one with hairy cell leukaemia are reported. These three leukaemic patients belonged to a series of 58 leukaemic patients with chronic exposure to benzene. The low percentage of chronic leukaemias in this series is discussed.
Sensitive methods for determining subclasses of IgG anti‐A and anti‐B in sera of blood‐group‐O women with a blood‐group‐A or ‐B child
Authors: Brouwers H. A. A. , Overbeeke M. A. M. , Gemke R. J. B. J. , Maas C. J. , van Leeuwen E. F. , Engelfriet C. P. 
Published: March 2008

SummaryThe determination of the subclasses of IgG antibodies against blood groups A and B is important in order to improve our understanding and predict haemolytic disease of the newborn due to IgG anti‐A or ‐B. We describe two techniques that circumvent the problem of the agglutination of A and B red cells by the corresponding IgG antibodies in saline: an antiglobulin consumption test and a modified solid‐phase micro‐immunofluorescence test. The results of the two techniques are compared with the results obtained in the indirect antiglobulin test beyond the saline agglutination titre in a microplate technique. The solid‐phase micro‐immunofluorescence test was the most sensitive for the determination of the subclasses of IgG anti‐A and ‐B. Usually sera contained IgG2 anti‐A, B in a higher titre than antibodies of other subclasses.
A myeloproliferative disease in two infants associated with eosinophilia and chromosome t(1;5) translocation
Authors: Darbyshire P. J. , Shortland D. , Swansbury G. J. , Sadler J. , Lawler S. D. , Chessells J. M. 
Published: March 2008

SummaryTwo children are described who presented at the age of 5 and 7 months with anaemia, a high white cell count with eosinophilia and thrombocytopenia. Both children had an identical balanced translocation t(1;5)(q23;q33) and no evidence of a constitutional abnormality. The response to treatment of one child was poor, the other remains well on therapy. This translocation has not been previously reported and is likely to represent a subclass of myeloproliferative disorder analogous to the monosomy 7 syndrome, although less common. The previous literature of acquired chromosome abnormalities involving chromosomes 1 and 5 is reviewed.
Response to therapy in 37 patients with HIV‐related thrombocytopenic purpura
Authors: Oksenhendler Eric , Bierling Philippe , Farcet Jean‐Pierre , Rabian Claire , Seligmann Maxime , Clauvel Jean‐Pierre 
Published: March 2008

SummaryThirty‐seven patients belonging to the major AIDS‐risk groups and with serum antibodies to the human immunodeficiency virus (HIV), were evaluated and treated between 1983 and 1986 because of profound chronic thrombocytopenia with clinical bleeding. Fifteen out of 26 patients achieved a significant response while on prednisone therapy. Six of them sustained their platelet count above 50 × 109/1 following cessation of steroids. High dose intravenous immunoglobulin therapy gave a satisfactory initial response rate in 12 of 17 patients. Only two of 18 patients responded to danazol. Splenectomy was successful in 10 of 14 patients.
Relationship between platelet aggregating factor and von Willebrand factor in thrombotic thrombocytopenic purpura
Authors: Murphy William G. , Moore Jane C. , Barr Ronald D. , Pai Mohan K. R. , Kelton John G. 
Published: March 2008

SummaryThe pathophysiology of the platelet thrombotic disorder, thrombotic thrombocytopenic purpura (TTP), is not well understood. Two apparently unrelated laboratory abnormalities have recently been described in patients with TTP: a platelet aggregating factor and abnormalities in von Willebrand factor (vWF). Although an interaction between these two abnormalities has been postulated to participate in the disease, this has not been proved. In this report we describe studies on a patient with relapsing TTP. These studies demonstrate that a consistent relationship exists between the platelet aggregating factor present in the patient's serum and vWF. The patient had chronic low‐grade thrombocytopenic and schistocytic haemolytic anaemia that could be temporarily cured by infusions of plasma and certain other blood products. During acute exacerbations of the illness, a platelet aggregating factor was detectable in the patient's serum and this was associated with the loss of the larger multimers of vWF. During remissions of the illness, abnormally large multimers of vWF were present. The results of this study support the concept that a platelet aggregating factor plus large multimers of vWF participate in the acute platelet thrombi that characterize TTP.
Characterization of a new α° thalassaemia defect in the South African population
Authors: Vandenplas S. , Higgs D. R. , Nicholls R. D. , Bester A. J. , Mathew C. G. P. 
Published: March 2008

Summary.A new a thalassaemia defect has been detected in the South African population. Restriction mapping of the α globin gene cluster in affected individuals has established that the defect is associated with the removal of 22·8·23·7 kb of DNA, including the pSα1, pSα1, pSα2, α2, and α1 globin genes. The 5’endpoint of the deletion has been localized between the 2 and pSα1 globin genes, and the 3’endpoint lies 4–5 kb 3’to the α1 globin gene. We have called the deletion ––SA in order to distinguish it from α° thalassaemia defects described in other populations.
Erythroid and myeloid maturation patterns related to progenitor assessment in the myelodysplastic syndromes
Authors: Dörmer P. , Schalhorn A. , Wilmanns W. , Hershko C. 
Published: March 2008

Summary. Patterns of erythropoiesis and granulopoiesis were studied in 15 patients with myelodysplastic syndromes and in one with smouldering leukaemia by correlating CFU‐C, BFU‐E and CFU‐E cloning efficiency with erythroid and myeloid maturation indices derived from quantitative 14C‐autoradiography and with ferrokinetics. Maturation index of a cell lineage was defined as the ratio of cell production rate increase from the first to the last proliferative compartment over the corresponding normal value. The myeloid maturation index was reduced in all cases, but CFU‐C progenitor frequency was increased by a factor of 3. Erythroid maturation index was also reduced in most cases, and BFU‐E progenitor frequency was reduced by a factor of 2. Similarly, CFU‐E cloning efficiency corrected for the erythroid maturation index was 2.5–10‐fold lower than normal. Comparison of the erythroid maturation index with ferrokinetics revealed a constant ratio of ineffective erythropoiesis with a maturation disturbance in the proliferative pool twice that in the non‐proliferative pool.These findings indicate basic qualitative and quantitative abnormalities and a difference in the patterns of production of erythroid and myeloid cells in the myelodysplastic syndromes: Myeloid progenitors are increased in number but their maturation is grossly abnormal. On the other hand, the apparent reduction in erythroid progenitors and the reduced erythroid maturation index are attributable to both premature cell death of the more mature erythroblasts and an increased proerythroblast proliferation, i.e. extra divisions in the proerythroblast compartment at the expense of maturation.
Bone marrow transplantation in patients with leukaemia previously transfused with blood products from family members
Authors: Ho Winston G. , Champlin Richard E. , Winston Drew J. , Feig Stephen A. , Gale Robert Peter 
Published: March 2008

Summary. Transfusions are withheld, whenever possible, from patients with aplastic anaemia who are potential bone marrow transplant recipients because of the increased risk of graft failure associated with transfusions prior to transplantation. Family members are specifically excluded as blood product donors to reduce the likelihood of sensitizing the recipient to antigens shared by the blood and bone marrow donor. This policy of not using family members, particularly the HLA‐matched bone marrow donor, to provide blood products prior to transplantation has been extended to leukaemia as well.To evaluate this policy we reviewed the outcome of bone marrow transplantation in 18 patients with leukaemia transfused prior to transplantation with platelets and/or leucocytes from related family members. In 15 cases in which the outcome could be evaluated, engraftment was rapid and graft failure did not occur. Transfusion of blood products from related family members to patients with leukaemia prior to transplantation does not appear, therefore, to increase the risk of graft rejection.
Evaluation of splenectomy in large granular lymphocyte leukaemia
Authors: Loughran Thomas P. , Starkebaum Gordon , Clark Eoward , Wallace Paul , Kadin Marshall E. 
Published: March 2008

SummaryWe performed splenectomy in four patients with severe neutropenia (<0.5 × 109/1), recurrent infections, and splenomegaly associated with large granular lymphocyte leukaemia. Prior to splenectomy, elevated levels of neutrophil‐reactive IgG were detected in sera of all three patients tested. In all patients, enlargement of the spleen was due to a characteristic lymphoid infiltration of red pulp cords. Splenectomy resulted in an increased neutrophil count >0.5 × 109/l in all patients; this response was sustained in two patients who benefited clinically by a dramatic reduction in frequency of infections. Poor clinical response was associated with elevated levels of antineutrophil antibody post‐splenectomy. All four patients had an increase in number of circulating large granular lymphocytes post‐splenectomy; one patient who had attained a sustained neutrophil response died of an accelerated lymphoproliferative disorder 19 months post‐splenectomy. We conclude that splenectomy may be of value in correcting severe neutropenia and reducing infections in some patients with large granular lymphocyte leukaemia. However, splenectomy appeared to be of no value in treatment of the underlying lymphoproliferative disorder.
Mechanisms and prognostic value of cell kinetics in the myelodysplastic syndromes
Authors: Dörmer P. , Hershko C. , Wilmanns W. 
Published: March 2008

SummaryCell kinetics were studied by quantitative autoradiography in 29 patients with myelodysplastic syndromes (MDS) and in four with smouldering leukaemia (SML) in a prospective 6–year study. Cellular turnover in all FAB groups was characterized by reduced labelling index and prolonged DNA synthesis time indicating reduced proliferative activity. Two more parameters were derived from the measurements. (1) The relative cell production rate, which is increased in proerythroblasts and myeloblasts in most cases. This could be attributed to an increased number of divisions in early recognizable precursors in the absence of maturation, and (or) an increased influx from the progenitor compartments. (2) The maturation index of a cell lineage was determined by the ratio of relative cell production rate increase from the first to the last proliferative compartment over the corresponding normal value. It showed a high degree of abnormality in all FAB subgroups, indicating premature cell death in the bone marrow. None of the kinetic parameters proved to be of value in predicting the evolution of leukaemia. In contrast, an excellent correlation was found between the myeloid maturation index and survival (P<0.0001). No correlation, however, was found between survival and the erythroid maturation index, which was relatively normal in the worst prognostic group. These findings indicate that althoughin vitrocell kinetic studies can not predict the evolution of overt leukaemia, the myeloid maturation index is a useful predictor of survival in patients with MDS and SMI.
Quinine‐ and quinidine platelet antibodies can react with GPIIb/IIIa
Authors: Christie Douglas J. , Mullen Patrick C. , Aster Richard H. 
Published: March 2008

SummaryQuinine‐ and quinidine‐dependent antiplatelet antibodies are generally believed to bind to the membrane glycoprotein complex, GPIb/IX. However, we and others have found that some drug‐dependent antibodies bind to platelets from patients with the Bernard‐Soulier syndrome which lack these glycoproteins. We therefore studied the reactions of a group of these antibodies with normal and Bernard‐Soulier platelets and their membrane proteins.As indicated by rosette formation of the sensitized platelets around protein A‐Sepharose beads, two quinine‐ and two quinidine‐dependent antibodies reacted with both normal and Bernard‐Soulier syndrome platelets at a high (300 μM) concentration of drug. At a pharmacologic drug concentration (10 μM), all four antibodies reacted with normal platelets, but only the two quinine‐induced antibodies reacted with Bernard‐Soulier platelets.Immunoprecipitation studies with solubilized, tritiumlabelled normal platelets, at both high and low drug concentrations, showed that each of the four antibodies precipitated proteins corresponding to GPIb and GPIX. Fainter bands corresponding to glycoproteins IIb and IIIa, which do not label well with tritium, were also detected. With radioiodinated normal platelets, it was found that each of the four antibodies was capable of precipitating GPIIb/IIIa, but only in the presence of drug.The four antibodies also promoted drug‐dependent precipitation of GPIIb and GPIIIa from lysates of radioiodinated Bernard‐Soulier platelets. The two quinine‐dependent antibodies precipitated these glycoproteins at both high and low drug concentrations, while the quinidine‐dependent antibodies reacted much more strongly at the higher drug concentration. Precipitation of GPIb/IX was not observed with BSS platelets.Absorption of a quinine‐induced antibody with Bernard‐Soulier platelets in the presence of drug eliminated its ability to precipitate GPIIb and GPIIIa. However, the absorbed antibody retained the ability to precipitate GPIb from solubilized normal platelets. Thus, at least two drug‐dependent antibodies were present, one specific for GPIb/IX and the other for GPIIb/IIIa.These findings indicate that glycoproteins IIb and/or IIIa, in addition to the GPIb/IX complex, can serve as targets for drug‐dependent antibodies in both intact and detergent‐solubilized platelet preparations.
Gerbich reactivity in 4.1(—) hereditary elliptocytosis and protein 4.1 level in blood group Gerbich deficiency
Authors: Sondag Danièle , Alloisio Nicole , Blanchard Dominique , Ducluzeau Marie‐Thérèse , Colonna Pierre , Bachir Dora , Bloy Christian , Cartron Jean‐Pierre , Delaunay Jean 
Published: March 2008

SummaryThe membrane polypeptide composition and the blood group Gerbich phenotype of red cells from 4.1(–) hereditary elliptocytic patients and from Gerbich‐negative donors, who display two unrelated genetic abnormalities, were compared. In homozygous 4.1(–) hereditary elliptocytosis where the primary defect was presumably the absence of the membrane skeletal protein 4.1, there was approximatively a 70% reduction in the minor sialoglycoproteins β and γ. This was associated with a severe reduction of blood group Gerbich reactivity as determined with both murine monoclonal and human anti‐Gerbich antibodies. In the heterozygous state in the presence of one haploid set of protein 4.1 gene there was only a modest decrease in glycoproteins β and γ and the Gerbich serological reactivity was within normal limits.In homozygous Gerbich‐negative red cells which lack glycoproteins β and γ but do not display elliptocytic red cells, the levels of protein 4.1 was repeatedly found within or just below the lowest values of normal controls. In the heterozygous Gerbich‐negative conditions, glycoproteins β and γ were present in reduced amounts but the blood group Gerbich reactivity fell within normal limits since the anti‐Gerbich reagents used were unable to detect a dosage effect. The amount of protein 4.1 was normal.These results add further support to the view that protein 4.1 and the sialoglycoproteins β and γ are physically linked in vivo which in some way serve to maintain red cell shape and integrity. Of interest was the finding that absence of protein 4.1 had a greater influence on the level of membrane glycoproteins β and γ than did the absence of β and γ glycoproteins on band 4.1.
Anti‐granulocyte opsonic activity in sera from patients with systemic lupus erythematosus
Authors: Hadley A. G. , Byron M. A. , Chapel H. M. , Bunch C. , Holburn A. M. 
Published: March 2008

SummaryNeutropenia is common in patients with systemic lupus erythematosus (SLE) but mechanisms of cell depletion remain obscure. To investigate the possible autoimmune aetiology of neutropenia in SLE, sera from 31 patients with this disorder were tested for anti‐granulocyte activity. Granulocyte‐binding immunoglobulins were detected by indirect immunofluorescence, and the ability of patient sera to opsonize granulocytes was determined by measuring the chemiluminescent response of human monocytes to granulocytes sensitized by test sera.Sera from 22 of the 31 patients bound IgG to granulocyte cell membranes and/or to nuclei, but only membrane‐binding antibodies opsonized the cells for recognition by monocytes. There was no correlation between neutrophil count and the level of granulocyte‐binding IgG as measured by indirect immunofluorescence. In contrast, opsonic activity and neutrophil count were inversely correlated (r= 0·5; P < 0·05). However, opsonic activity was present in sera from most non‐neutropenic patients. In patients with SLE, impaired reticuloendothelial system function may allow sensitized granulocytes to remain in the circulation.