bluuebunny/crossref_metadata_2025_split huggingface dataset:
Biochemical and Histological Analysis of Bone Marrow Collagen in Myelofibrosis
Authors: Charron Dominique , Robert Ladislas , Couty Marie‐Claude , Binet Jacques‐Louis 
Published: March 2008

Summary. Total protein, collagen (hydroxyproline) and glycoproteins (hexosamine) content of control and myelofibrosis (MF) bone marrow samples was determined using a sequential extraction procedure. MF marrow extracts contained higher amounts of collagen than control extracts. The collagen content appeared to increase with the duration of the disease. In more recent MF cases (<2 years) at least 60% of the total collagen was extracted in 0.5 m NaCl. this proportion decreases to 33% in older cases (> 4 years), indicating a progressive insolubilization (crosslinking) of collagen.The hexosamine content of the extracts decreased in MF as compared to controls reflecting a decrease in glycosaminoglycans (and possibly of structural glycoproteins). The reticulin content of the same bone marrows was estimated by a quantiative morphometric procedure. There was a positive correlation between the morphometrically estimated reticulin surface and the total hydroxyproline content of the marrow samples. The slopes of the least square lines correlating increase of reticulin surface to hydroxyproline content were, however, significantly different in control and MF marrows, indicating a 44% higher increase in histochemically detectable reticulin per unit increase in hydroxyproline content than in the control marrows. This result may indicate a more efficient fibrogenetic process in MF marrow than in normal bone marrow.The above results confirm the collagenous nature of the fibrous reticulin like material deposited in MF marrows and suggests a correlation between the progression of the disease and the rate of synthesis and deposition of collagen fibres.
Response to Treatment in Acute Non‐lymphatic Leukaemia: Prognostic Value of Colony Forming and Colony Stimulating Capacities of Bone Marrow and Blood Cells Compared to Other Parameters
Authors: Beran Miloslav , Reizenstein Peter , Uden Ann‐Mari 
Published: March 2008

Summary.Growth of bone marrow and mononuclear white blood cells (MWBC) in soft‐agar cultures was studied in 26 patients with untreated acute non‐lymphocytic leukaemia (ANLL). Marrow and MWBC from 30 healthy volunteers served as controls. All ANLL patients revealed an abnormal growth in vitro. Patients with an increased number of clones in marrow cultures and large cluster predominance (‘excessive growth’) responded poorly to therapy with only one of 10 patients entering remission. On the contrary, only two of the 15 patients with a decreased clone number (‘low growth’) failed to achieve remission. The number of colonies and clusters in both bone marrow and blood cultures was significantly lower at presentation in patients who later entered remission than in those who did not. The correlations between the number of colonies and clusters in the blood and the marrow cultures were statistically significant. No significant correlations were found between prognosis or colony formation, on one hand, and the production of colony stimulating activity (CSA), by bone marrow and blood cells of ANLL patients, on the other. Nor could such correlations be found between prognosis, blood cell counts, and age. It is concluded that the growth pattern of both bone marrow and circulating colony forming cells is of value in predicting the response to cytostatic drugs and particularly in selecting patients with a high probability to respond poorly to current cytostatic regimes.
Polymorphonuclear (PMN) Function after Small Intestinal Shunt Operation for Morbid Obesity
Authors: Palmblad Jan , Hallberg Dag , Engstedt Lars 
Published: March 2008

Summary.The possible influence on blood polymorphonuclear (PMN) granulocyte functions of the small intestinal shunt operation for obesity was studied in 10 massively overweight patients. They were investigated prior to operation and for 9 months afterwards, when they had lost on average 32 kg body weight. Preoperatively they showed reduced PMN bactericidal capacity and increased PMN adherence compared with controls of normal weight. During the first 2–4 months postoperatively all patients displayed a gradually increasing bactericidal capacity, which then reached levels similar to the controls and remained so for the rest of the follow‐up period. This enhancement was more easily assessed by a new in vitro assay in which each PMN was provided with 30–40 bacteria, than by a standard assay using 2–4 bacteria per granulocyte. PMN adherence decreased during the first postoperative months and then returned to preoperative levels. The changes in PMN functions were not statistically related either to each other or to the continuous loss of body weight. Thus, impairment of PMN killing function occurring in extremely obese patients became normalized after small bowel shunt operation, while the high adherence remained unchanged.
Origin of Human Bone Marrow Fibroblasts
Authors: Golde D. W. , Hocking W. G. , Quan S. G. , Sparkes R. S. , Gale R. P. 
Published: March 2008

Summary.We investigated the origin of bone marrow fibroblasts in three bone marrow transplant recipients with aplastic anaemia and leukaemia who received grafts from HLA‐identical siblings of opposite sex. The patients were conditioned for transplantation with high doses of cytotoxic drugs and 300–1000 rads total body irradiation. After transplantation, bone marrow cells wrere cultured in T flasks for 3 weeks and the adherent cells were then trypsinized and passaged weekly. After several passages the cells had the typical morphology and growth pattern of fibroblasts. Metaphases from these cells were all of recipient sex type. In contrast, haematopoietic cells and lymphocytes obtained at the same time were of donor sex type. Our findings indicate that the human bone marrow fibroblast is not derived from a precursor common to haematopoietic cells or lymphocytes. The bone marrow fibroblast appears to be a mesenchymal cell, unrelated to haematopoietic stem cells, which is capable of in vitro proliferation after as much as 1000 rads of total body irradiation.
Polycythaemia Vera: in Vitro Studies of Circulating Erythroid Progenitors
Authors: Lacombe C. , Casadevall N. , Varet B. 
Published: March 2008

Summary.Peripheral blood mononuclear cells from healthy subjects, patients with secondary polycythaemia and patients with polycythaemia vera (PV) were cultured using the plasma clot technique, and large erythroid colonies or bursts were scored after 14 d. Erythroid bursts appeared without addition of erythropoietin in all 20 patients with PV but never in normal subjects (n=10) nor in patients with secondary polycythaemia (n=6). The erythropoietin dose‐response curves in six PV patients were characterized by an initial plateau followed by a nearly normal response. This suggests the coexistence of two populations of erythroid progenitors in PV, one abnormally sensitive, the other normally responsive to erythropoietin. In nine patients the ratio of spontaneous bursts to bursts induced by high doses of erythropoietin from the blood on day 14 was identical with the ratio of spontaneous colonies to colonies induced by high doses of erythropoietin from the bone marrow of the same patients on day 7. This argues against the hypothesis that spontaneous erythroid bursts are derived from erythroid progenitors (BFUE) abnormally sensitive to erythropoietin. Moreover blood mononuclear cells from healthy subjects were found to give rise to erythroid bursts when erythropoietin addition was delayed until 6 d in culture. This is consistent with the concept that erythropoietin is not normally required in vivo for the differentiation of early (BFUE) to late (CFUE) erythroid progenitors. These findings suggest that in PV all BFUE are normally responsive to the physiological factor(s) different from erythropoietin and responsible for their differentiation into CFUE, and that the abnormal response to erythropoietin, distinctive of a population of erythroid progenitors, is expressed only at the most mature (CFUE) level.
Terminal Transferase Enzyme Assay and Immunological Membrane Markers in the Diagnosis of Leukaemia: a Multiparameter Analysis of 300 Cases
Authors: Janossy G. , Hoffbrand A. V. , Greaves M. F. , Ganeshaguru K. , Pain Carol , Bradstock K. F. , Prentice H. G. , Kay H. E. M. , Lister T. A. 
Published: March 2008

Summary.Multiparameter analyses have been carried out with recently developed enzyme and membrane markers in 300 patients with various leukaemias including ALL, AML, but excluding Ph1 positive leukaemias.TdT enzyme levels were particularly valuable in the differential diagnosis of adult acute lymphoid and myeloid leukaemias. The levels were raised in 108 (94%) of the 115 patients who were considered to be non‐T, non‐B ALL on membrane marker and morphological analysis; all seven cases giving negative TdT results in this group were young children. Unexpectedly high levels were seen only in three (4.1%) of 73 cases of acute myeloid leukaemia verified by histochemistry and membrane markers. Anti‐ALL serum was a most useful reagent in childhood leukaemias but blasts from 19 patients (10% of childhood ALL cases and 29% of adult ALL cases) failed to react with the serum in spite of TdT positivity. Strongly ALL+ blasts were seen only in non‐T, non‐B ALL and some undifferentiated leukaemias. Weakly ALL+ blasts were seen in seven of 32 cases of thymic ALL (Thy‐ALL) but in other respects these blasts expressed Thy‐ALL features, such as strong reactivity with anti‐T cell (HuTLA) serum, negativity with anti‐Ia‐like serum and raised TdT. The combination of tests was particularly useful in 32 cases of undifferentiated leukaemia: in 10 of these cases TdT positivity indicated the probable ‘lymphoblast’, nature of blast cells: the remaining 22 cases remained unclassifiable with the markers used. The analysis revealed other interesting variant forms of leukaemias.
Lymphocyte Density Distribution Profile and Spontaneous Transformation Related to the Stage of Hodgkin's Disease
Authors: De Pauw B. E. , Th. Wagener D. J. , Smeulders J. B. J. M. , Geestman E. J. M. , Wessels J. M. C. , Haanen C. 
Published: March 2008

Summary. Density distribution profiles of peripheral lymphocytes were made in 41 untreated patients with Hodgkin's disease and in 14 normal controls. The fraction of lymphocytes with a low specific gravity in patients was significantly increased in comparison to normal controls (P= 0.0007). These low density lymphocytes in Hodgkin's disease patients showed an elevated spontaneous 3H‐thymidine incorporation compared to normal controls (P < 0.0001). In 78% of the patients the spontaneous DNA synthesis of the low density lymphocytes was above the normal range. A positive correlation (P= 0.002) was found between the spontaneous 3H‐thymidine uptake and the stage of the disease.
Haematological Aspects of Antenatal Diagnosis for Thalassaemia in Britain
Authors: Matsakis M. , Berdoukas V. A. , Angastiniotis M. , Mouzouras M. , Ioannou P. , Ferrari M. , Modell B. , Fairweather D. V. I. , Ward R. H. T. , Loukopoulos D. , Sakarellou N. 
Published: March 2008

Summary.The results are described of 200 antenatal diagnostic tests for haemoglobinopathies performed on samples of fetal blood obtained during the second trimester of pregnancy. Haemoglobin A synthesis in the fetus was measured by incorporation of tritiated leucine in vitro and separation of the globin chains on CM23 columns. The range of HbA synthesis detected was 3.5–8.0% in normal fetuses, 2.0–5.0% in fetuses with thalassaemia trait, and less than 1.6% in fetuses with thalassaemia major. There were eight cases in which other haemoglobinopathies were diagnosed. 29% of the pregnancies were terminated because thalassaemia major was diagnosed, and 9.5% of the remaining healthy fetuses were lost for obstetric reasons. Follow up has been possible for 96% of the 124 surviving babies and three misdiagnoses have come to light; one false positive (0.5%) and two false negatives (1%). These figures represent a first effort at antenatal diagnosis for haemoglobinopathies and it is likely that they will improve with the passage of time.
Five Families with Homozygous δ‐Thalassaemia in Japan
Authors: Yasukawa Masaki , Saito Shuji , Fujita Shigeru , Ohta Yoshiro , Ikeda Kyoichi , Matsumoto Isao , Kobayashi Yuzuru 
Published: March 2008

Summary.Five families with δ‐thalassaemia discovered in Ehime, Japan, are presented. The δ‐thalassaemia was associated with a slight elevation of the level of Hb F in two families and with normal Hb F levels in three. Complete absence of HbA2 was found in the homozygous probands. No abnormal clinical or haematological findings were noted in the individuals with δ‐thalassaemia.
Clinical and Immunologic Spectrum of Plasmacytoid Lymphocytic Lymphoma without Serum Monoclonal IgM
Authors: Levine Alexandra M. , Lichtenstein Alan , Gresik Mary V. , Taylor Clive R. , Feinstein Donald I. , Lukes Robert J. 
Published: March 2008

Summary.As first suggested by Lukes & Collins (1979, and recently confirmed in the literature, the pathological lesion associated with Waldenström's macroglobulinaemia can be found in patients with serum IgG or IgA monoclonal proteins. To expand upon the clinical and immunologic spectrum of this lesion, we reviewed the records of 12 patients with identical tumour morphology, though lacking an IgM paraprotein. The median age of the patients was 62 years. In five patients the tumour secreted monoclonal IgG, while one secreted monoclonal IgA. One patient had normal serum protein electrophoresis, although monoclonal light chains were demonstrated on urinary protein electrophoresis. The remaining patients had no detectable monoclonal protein. Marker studies demonstrated the U cell origin of tumour; these findings were consistent with the monoclonal protein secreted. Clinically, these cases varied, from peripheral blood involvenient resembling chronic lymphocytic leukaemia, to typical lymphomatous processes, to presentations resembling multiple myeloma, reflecting the similarity of all of these conditions as varying expressions of neoplasia of the B cell system.
Decreased Association of 45Calcium with Platelets Unable to Aggregate due to Thrombasthenia or Prolonged Calcium Deprivation
Authors: Peerschke Ellinor I. , Grant Robert A. , Zucker Marjorie B. 
Published: March 2008

Summary. 45Calcium uptake was studied with aspirin‐treated platelets that were gel‐filtered through a column of Sepharose 2B equilibrated with divalent cation‐free modified Tyrode's solution to remove readily exchangeable surface‐associated calcium. These platelets aggregated almost immediately when exposed to ADP, fibrinogen and at least 30 μm CaCl2. At this calcium ion concentration, 108 platelets took up 36.6 ± SEM 2.7 pmol of 45calcium within 1–2 min. The presence of ADP and fibrinogen did not affect the amount of calcium bound. Over 90% of this platelet‐associated calcium was removed by EDTA in 5 min suggesting that it was surface‐bound. Calcium uptake increased rapidly for 10 min, then more slowly for up to 2 h. At 60 min, maximal uptake was approached at CaCl2 concentrations between 250 and 300 μm when an average of 276 ± SEM 18 pmol of calcium was associated with 108 platelets. Only 50–60% of this calcium could be removed by EDTA in 5 min, and about 70% in 20 min, suggesting that some of it had been internalized. Platelets from two patients with thrombasthenia that were unable to aggregate took up 50% less calcium than platelets from normal volunteers. Similarly, platelets that had been incubated with EDTA at 37°C, pH 7.8 for 8 min lost the ability to aggregate despite recalcification and took up 40–60% less calcium than CaEDTA‐treated controls. Platelets from a patient with the Bernard‐Soulier syndrome aggregated and bound calcium normally. Thus the platelets’ ability to take up calcium after removal of surface‐associated calcium correlates with their ability to aggregate. Since thrombasthenic platelets and platelets rendered incapable of aggregating after prolonged calcium deprivation with EDTA do not bind fibrinogen, we postulate that some of the surface‐associated calcium normally binds to the fibrinogen receptors.
Immunochemical Analysis of Active and Inactive Antithrombin III
Authors: McKay E.J. 
Published: March 2008

Summary.Antithrombin III (AT) levels from normal and AT deficiency persons were measured by electroimmunoassay (EIA) and the rewlts compared with a chromogenic assay (S2238). Discrepant results were obtained when plasma and serum were compared using one antiserum, and therefore did not always relate to functional activity. Another antiserum, however, when used was capable of differentiating active AT from inactive AT complexed with its proteases and demonstrated close correlation with all samples tested (r=0.97). The specificity of the antisera and consequent anomalous results were elucidated when purified human thrombin was added to plasma samples and subsequently reanalysed. Quantitative differences observed when serum samples were compared by single radial immuno‐diffusion and electroimmunoassay with one antiserum, illustrates the fundamental principle differences between the two methods. These results give some insight as to why previous anomalies with AT immunoassays have occurred. They also indicate that plasma and not serum should be used as clinical test material. AT antisera should be capable of recognizing and distinguishing free AT from AT/protease complexes if the results obtained by electroimmunoassay are to correlate with functional activity.
Ultrastructural Studies of Erythropoiesis in β‐Thalassaemia Trait
Authors: Wickramasinghe S. N. , Hughes Madeleine 
Published: March 2008

Summary.The erythropoietic cells of six cases of β‐thalassaemia trait were studied by electron microscopy and electron microscope autoradiography. Intracytoplas‐mic and intranuclear α‐chain precipitates were found in some late polycliromatic erythroblasts and intracytoplasmic precipitates were found in several marrow reti‐culocytes. This provides direct morphological evidence of unbalanced globin chain synthesis in the marrow. Several of the polychromatic erythroblasts and marrow reticulocytes contained autophagic vacuoles and showed a variety of other dysery thropoietic changes. Erythroblast profiles containing moderate quantities of precipitated α‐chains usually suffered from a marked depression of protein synthesis. The proportions of marrow cells containing α‐chain precipitates and displaying dyserythropoietic changes varied considerably from patient to patient. It is proposed that this variation largely reflects variations in the proteolytic capacity of the erythropoietic cells in different individuals and leads to different degrees of ineffective erythropoiesis in β‐thalassaemia trait.
Platelet‐Activating Factor (PAF‐Acether) Secretion from Platelets: Effect of Aggregating Agents
Authors: Chignard M. , Couedic J. P. Le , Vargaftig B. B. , Benvenisie J. 
Published: March 2008

Summary.Platelet‐activating factor is a 1.0‐alkyl‐2 acetyl analogue of phosphatidylcholine (PAF‐acether) which triggers platelet aggregation independently from ADP release and thromboxane A2 (T×A2) formation. PAF‐acether was described initially as being secreted by rabbit basophils during an immunological challenge. We have now found that it is also formed by washed rabbit platelets stimulated by the calcium ionophore A23187, thrombin and collagen. These three agents are known to trigger platelet aggregation independently from the release of ADP and from the formation of T×A2. By contrast, ADP and arachidonic acid, the precursor of T×A2, which do not share these properties, and PAF‐acether itself, were unable to induce PAF‐acether formation. Our results suggest that PAF‐acether may be the mediator responsible for AIIP and T×A2 independent‐aggregation.
Impaired Thromboxane Production by Newly Formed Platelets after Aspirin Administration to Thrombocytopenic Rats
Authors: Dejana Elisabetta , Barbieri Brunelia , Cerletti Chiara , Livio Manuela , Gaetano Giovanni de 
Published: March 2008

Summary.The in vivo inhibitory effect of aspirin on platelet cyclo‐oxygenase is irreversible and lasts for the entire platelet life‐span. Reappearance of cyclo‐oxygenase activity in blood after aspirin has been proposed as a measure of the formation of new platelets and as an indirect indicator of platelet survival. A delay of 24–72 h in recovery, however, has been observed and it has been suggested that aspirin might also inhibit megakaryocyte cyclo‐oxygenase. To test this possibility, aspirin (100 mg/kg) or saline were administered i.p. to rats made thrombocytopenic 2 h later (platelet count less than 5% of basal value) by a specific antiplatelet antiserum. Malondialdehyde (MDA) and thromboxane B2 (T×B2) production by platelets was measured by spectrophotometry and radioimmunoassay respectively, during the period of platelet count restoration. By 24 h after thrombocytopenia was induced, platelet count was about 15% of basal values in control and aspirin‐treated rats. However, while in controls MDA and T×B2 production was restored to about 20% of basal values, in aspirin‐treated rats less than 5% return of activity was detected. A marked difference between the two groups was still found 96 h after induction of thrombocytopenia, when platelet count restoration was similar. Since aspirin disappeared very rapidly from the circulation, the delay in recovery of cyclo‐oxygenase activity supports the hypothesis of a megakaryocyte effect of this drug.
Non‐spherocytic Haemolytic Anaemia in Mother and Son Associated with Hexokinase Deficiency
Authors: Newman P. , Muir A. , Parker A. C. 
Published: March 2008

Summary.The case history, laboratory findings and properties of the enzyme in a patient with hexokinase deficiency are reported. The mother had a haemolytic disorder of similar severity. In these cases the expression of the disease in both mother and son suggests a dominant mode of inheritance. This family further illustrates the necessity of taking the reticulocyte level into account when interpreting red cell enzyme levels.
Maturation Index: a Contribution to Quantification in the FAB Cla.ssification of Acute Leukaemia
Authors: Rhenen D. J. van , Verhulst J. C. , Huijgens P. C. , Langenhuijsen M. M. A. C. 
Published: March 2008

Summary.The French—American—British co‐operative study group (FAB) criteria were used to classify acute leukaemia in 194 adult patients. The dividing lines between FAB subgroups M1‐M2 and M5A—M5B appeared to be less well defined. In an effort to improve subtyping we introduce the maturation index (MI).MI=promyelocytes/promonocytes/blast cells + promyelocytes/promonocytes/×100%.Clearly distinct subgroups emerged after arrangement of cases according to MI values. Immature and rather mature cases in both myeloblastic and monoblastic leukaemia were divided at a MI of 4%. Assignment to M3, which is based essentially on morphological grounds, could be made on numerical grounds too, MI being at least 85% in all of our cases. A few cases showing M2 morphology and a positive α‐naphthylacetate esterase (ANE) staining should probably be categorized as M4. Application of the original FAB criteria resulted in disagreement of 14% between three investigators, while with our additional criteria the discrepancy was only 2%.