bluuebunny/crossref_metadata_2025_split huggingface dataset:
Haematological aspects of life with an ileostomy
Authors: Kennedy H. J. , Callender Sheila T. , Truelove S. C. , Warner G. T. 
Published: March 2008

Summary. The haematological aspects of life with an ileostomy have been studied in 51 patients, of whom 39 had had ulcerative colitis and 12 had Crohn's dissease. The findings in these patients have been compared with those in 39 healthy volunteers who were matched for age and sex with the 39 patients who had had ulcerative colitis.There was evidence of a mild degree of iron deficiency in the patients with an ileostomy. This was partly due to a pre‐existing iron deficiency resulting from their preceding illness and operation, as the abnormality was less pronounced in the patients in whom the ileostomy had been established for more than 3 years. There was some evidence of excessive iron loss and a controlled trial of ferrous fumurate showed that the iron deficiency was largely corrected by this means. Circulating levels of vitamin B12 were normal, but it is relevant that some of the Crohn's disease group were receiving parenteral supplements. The absorption of vitamin B12 was low in the patients with Crohn's disease who had had an ileal resection of more than 17 cm. The absorption of vitamin B12 in the patients who had had ulcerative colitis was increased and possible mechanisms are discussed. All but one of the patients had normal levels of plasma folate and in all the red cell blood folate was normal, which can be taken as an indication of a good dietary intake and adequate absorption.
Studies on the partition of transferrin‐donated iron in rabbit reticulocytes: I. THE KINETICS OF IRON DISTRIBUTION BETWEEN STROMA AND CYTOSOL
Authors: Egyed A. 
Published: March 2008

Summary. The distribution of transferrin‐donated iron between the stroma and cytosol compartments of rabbit reticulocytes was studied. In cells with inhibited haem synthesis iron accumulates in the stroma fraction. The stromal accumulation of iron was found to be reversible and dependent on the coupled state of mitochondrial respiration. Iron in cytosol, however, proved to be more available for haem synthesis than iron accumulated in stroma.A hypothesis concerning mitochondrial iron uptake is proposed, by which the experimental and pathological changes in the intracellular distribution of iron can be explained.
Heterogeneity of haemophilia A: a study with three different antisera
Authors: Muller Hein P. , Van Tilburg Nico H. , Bertina Rogier M. , Veltkamp Jan J. 
Published: March 2008

Summary. One human and one rabbit antibody against VIII:C—in a fluid‐phase, inhibitor neutralization assay (INA)—and one human antibody—in a solid‐phase, immunoradiometric assay (IRMA)—were used to investigate a group of 59 patients with severe, moderate and mild haemophilia A.Patients were classified as haemophilia A+ when the VIII:C/VIIICAG ratio was less than 0.4 while the absolute VIII:C antigen (VIIICAG) value exceeded 0.25 u/ml. Three haemophiliacs (from two pedigrees) were classified as A+ in all three assay systems. 50% of the patients were classified as haemophilia A+ on the basis of at least one assay. The other 50%, including 66% of the patients with severe haemophilia, were shown to be A‐ by all three assay systems. Combining the data obtained with the three different antibodies four different categories could be distinguished, in addition to the classification based on severity.
Erythroblast iron metabolism in sideroblastic marrows
Authors: May A. , Souza P. de , Barnes K. , Kaaba S. , Jacobs A. 
Published: March 2008

Summary. The uptake of iron by bone marrow erythroblasts and its intracellular distribution have been studied in 23 patients with primary sideroblastic anaemia (SA), five patients with secondary SA and one patient with only non‐ringed sideroblasts. EM of erythroblasts from 18 cases showed both mitochondrial iron deposits and cytoplasmic ferritin aggregates in all cases except the patient with only non‐ringed sideroblasts.Iron uptake by erythroblasts in whole bone marrow was normal but there was a decreased incorporation into haem and an increased incorporation into cell stroma. Age matching of erythroblasts using Percoll density gradient centrifugation indicated that stromal iron incorporation was high at all stages of erythroblast development even before haem synthesis had become a major metabolic activity and in intermediate and late erythroblasts a real decrease in haem synthesis appeared less certain. These observations, together with the inability to correct the abnormality in vitro with either pyridoxal phosphate or amino‐laevulinic acid suggest that the primary defect in SA may be an abnormality of mitochondrial iron metabolism rather than an abnormality of haem synthesis.
Ferritin and iron uptake by reticulocytes
Authors: Blight G. D. , Morgan E. H. 
Published: March 2008

Summary.The uptake of liver ferritin labelled with 125I or 59Fe by guinea‐pig reticulocytes has been studied to investigate the characteristics of the uptake process, compare it with transferrin uptake and determine whether ferritin‐iron is utilized by the cells in haem synthesis. The results confirmed that guinea‐pig reticulocytes, but not mature erythrocytes, take up liver ferritin by a saturable, time‐ and temperature‐dependent process. Up to 70% of the iron taken up by the cells was utilized in haem synthesis and competed directly with iron derived from transferrin. Scatchard analysis of the binding parameters indicated that 30–130 × 103 ferritin molecules were bound per cell to high affinity specific membrane receptors (Ka: 1·77 × 107 M−1). In contrast, rat took up much less ferritin than guinea‐pig reticulocytes and the process was entirely non‐specific. Release experiments with guinea‐pig reticulocytes at 37°C showed that a maximum of about 70% of the cell‐associated 125I‐ferritin was released from the cells of which up to 15% was trichloroacetic acid‐soluble.We suggest that ferritin uptake by guinea‐pig reticulocytes involves receptor‐mediated endocytosis. The endocytotic vesicle fuses with a lysosome, iron is removed from the protein and enters a cytosolic pool in which it competes directly with transferrin‐derived iron to provide iron for mitochondrial haem synthesis. Some of the ferritin is catabolized and the rest is returned to the extracellular medium during membrane recycling.
Deposition of the terminal C5b‐9 complement complex on erythrocytes by human red cell autoantibodies
Authors: Salama A. , Bhakdi S. , Mueller‐Eckhardt C. , Kayser W. 
Published: March 2008

Summary.A radioassay for the detection of complement activating autoantibodies (autohaemolysins) in patients with autoimmune haemolytic anaemia (AIHA) is described. The method is based on immunoradiometric quantitation of the cytolytic C5b‐9 complement complex following its antibody‐dependent deposition on red blood cells (RBC). The use of affinity‐purified, radiolabelled antibodies directed against the neoantigens of the C5b‐9 complex ensured specificity of the test which proved more sensitive than conventional haemolytic assays and was not subject to disturbances by haemolytic sera.The results obtained in 70 patients with various forms of AIHA (warm type (N= 45); cold type (N= 22); Donath‐Landsteiner type (N= 3)) support the prevailing assumption that autohaemolysins can trigger the complement cascade to completion. Since intact RBC from patients with detectable autohaemolysins carried C3/C4 components but never C5b‐9, it is inferred that regulatory mechanisms operate in vivo at the level of C4b/C3b inactivation to arrest the cascade and rescue the autologous cells.
Tissue ferritin in scorbutic guinea‐pigs
Authors: Roeser H. P. , Sizemore D. J. , Nikles A. , Cham B. E. 
Published: March 2008

Summary.Tissue ferritin metabolism was compared in control and ascorbic acid (AA) deficient guinea‐pigs. Concentrations of ferritin protein in the liver (0·98±0·61 mg/g wet weight) and spleen (0·48±0·23 mg/g) of control animals did not change after tissue depletion of AA. Iron dextran (75 mg/kg weight, i.m.) caused a 4–5‐fold increase in tissue ferritin concentrations in controls whereas no increase in tissue ferritin occurred in scorbutic animals. The rise in tissue total iron concentration was similar in the two groups. Liver ferritin synthesis was similar in control and scorbutic animals. After stimulation with iron (8 mg/kg iron dextran i.v.), ferritin synthesis rose in both groups of animals. However, the pattern of response differed. At 24 h after iron dextran, ferritin synthesis in controls was still significantly elevated (P< 0 · 001) and liver ferritin protein continued to rise, whereas in scorbutic animals, ferritin synthesis had declined to pre‐iron injection levels, and no rise in ferritin protein values occurred. It is concluded that the ferritin synthetic apparatus in AA deficient tissues remained intact and capable of responding to added iron. The absence of a sustained elevation in tissue ferritin protein after an iron load appeared to be due to inadequate stimulation of ferritin synthesis by intracellular iron. It is suggested that AA has a physiological role in the reduction of intracellular iron and that it is the reduced form of iron which stimulates ferritin synthesis. Abnormalities of iron metabolism occur in AA depleted tissues when the quantity of Fe3±us;entering cells exceeds the residual reducing capacity of those cells.
Erythrocyte membrane skeleton abnormalities in hereditary spherocytosis
Authors: Burke Brian E. , Shotton David M. 
Published: March 2008

Summary. Erythrocyte ghosts from eight individuals with hereditary spherocytosis have been compared with respect to their protein compositions as judged by SDS gel electrophoresis, their ease of spectrin extractability, and their freeze‐etch electron microscopic appearance after incubation in conditions designed to promote aggregation of the intramembrane particles. Four of these HS cases were unrelated, while the other four represented two generations from a single family, including a pair of identical twins, one of whom had not undergone splenectomy when this investigation was initiated.Of the four unrelated cases, one showed no departures from normal under the conditions of this investigation, whereas the other three exhibited features which suggested a membrane skeleton lesion. In one of these there was a reduced proportion of spectrin tetramers relative to dimers in 4°C extracts, while the two remaining cases exhibited abnormal intramembrane particle aggregation. The four related cases had almost identical variations from normal. Spectrin was not extractable from their ghost membranes during a mild extraction incubation which removed spectrin from normal control ghosts. However, the intramembrane particle aggregation subsequently induced in these ghosts was of a degree unobtainable in normal ghosts without such spectrin extraction. In addition the ghosts from one twin, the only one of these patients who had not undergone splenectomy at the start of this investigation, showed a reduced amount of band 4.2. However, when this patient's blood was re‐tested after splenectomy, this protein was found to be at normal levels.Our results support the view that hereditary spherocytosis is not a single disease, but is rather a term used to describe a variety of different molecular lesions of the erythrocyte membrane skeleton with similar clinical manifestations.
Transplantation of bone marrow fibroblastoid stromal cells in mice via the intravenous route
Authors: Piersma A. H. , Ploemacher R. E. , Brockbank K. G. M. 
Published: March 2008

Summary. Kinetics of fibroblastic colony‐forming cells (CFU‐F) were studied in mouse bone marrow after lethal total body irradiation and intravenous bone marrow transplantation. After an initial decrease, CFU‐F numbers recovered, and plateaued 5 weeks post‐treatment at 10% of normal values.Using chromosome‐marked donor bone marrow cells we found that 1 day after transplantation 72% of donor CFU‐F had reached the recipient's bone marrow, indicating a highly specific lodgment of CFU‐F. Three months after transplantation donor CFU‐F were still detectable and comprised about half of the femoral CFU‐F population.It is concluded that CFU‐F, a component of the haemopoietic microenvironment, are transplantable via the intravenous route.
The ‘spent’phase of polycythaemia vera: hypersplenism in the absence of myelofibrosis
Authors: Najean Y. , Arrago J. P. , Rain J. D. , Dresch C. 
Published: March 2008

Summary A clinical phase (spent phase) in the course of polycythaemia vera (PV) cases is described as enlargement of the spleen in spite of treatment, frequent cytopenia of one or several lines, persistent red cell hypervolaemia with considerable increase of plasma volume, persistence of myeloid hyperplasia with no collagen myelofibrosis or osteomyelosclerosis, absence of hepatosplenic erythro‐blastic metaplasia, as shown by radio‐iron kinetics and/or 111In‐transferrin scintigraphy.The frequency of this phase was 5% in a study where it was not systematically sought, but it could in fact be greater. Its occurrence is not related to the clinical and biological parameters of PV. On the other hand, it is significantly more frequent and earlier in patients treated by phlebotomies than in those treated by myelosuppression (32P).In four of the 12 cases, this phase was rapidly followed by an acute leukaemia. In eight cases there was a 1–5 year interval before a myelofibrosis with splenic myeloid metaplasia. This evolution could at this stage be delayed by chemotherapy. The efficacy of splenectomy should be studied.
Radioimmunoassays of human high and low molecular weight kininogens in plasmas and platelets
Authors: Kerbiriou‐Nabias Daniegle M. , Garcia Francia O. , Larrieu Marie‐Josea 
Published: March 2008

Summary. Radioimmunoassays of human high molecular weight kininogen (HMWK) and low molecular weight kininogen (LMWK) were developed using antibodies directed against the light and the heavy chains of kallikrein‐cleaved HMWK. With the anti‐light chain antibodies, the radioimmunoassay was specific for HMWK with a detection limit of 0.4 ng. The anti‐heavy chain antibodies were used to quantify the concentration of total kininogen antigens. In four different plasmas with a congenital deficiency in HMWK procoagulant activity, there was no detectable antigen in two cases and trace amounts, less than 1 μg/ml in the other plasmas (normal concentration: 72 ± 6 μg/ml). In the absence of HMWK, the radioimmunoassay performed with the anti‐heavy chain antibodies was specific for LMWK. The amount of LMWK was different in each of these patients’plasmas, ranging from no detectable antigen, i.e. less than 0.15 μg/ml, to a normal content.Antigens immunologically indistinguishable from plasma kininogens were detected in lysates of five times washed platelets. HMWK antigen concentration was 3.17±0.87 μg per 1011 platelets (mean value in 11 donors). LMWK was also present in platelet lysates and the relative concentration versus HMWK was the same as in plasma.
Pyrimidine 5′nucleotidase and several other red cell enzyme activities in β‐thalassaemia trait
Authors: Vives Corrons J. L. , Pujades M. A. , Aguilar i Bascompte J. L. , Jou J. M. , Rozman C. , Ester A. 
Published: March 2008

Summary. The activity of 18 red blood cell (RBC) enzymes and reduced glutathione (GSH) content were measured in 70 normal subjects, in 50 heterozygous β‐thalassaemia carriers and in 50 non‐thalassaemic patients with haemolytic anaemia and high reticulocyte counts. In addition, pyrimidine 5′nucleotidase (P5N) activity was also determined in 34 patients with hypochromic, microcytic, iron deficiency anaemia. β‐Thalassaemia trait was associated with an increase in almost all of the enzyme activities, except for 2,3‐bisphosphoglycerate synthetase (BPGS) and glutathione reductase (GR) which were normal and for acetylcholinesterase (AChE) and P5N which were slightly and markedly decreased respectively. The increases in enzyme activities were similar to those observed in patients with non‐thalassaemic reticulocytosis except for glyceraldehydephosphate dehydrogenase (GAPD), phos‐phoglyceratekinase (PGK), pyruvate kinase (PK), glutathione peroxidase (GPX) and adenylate kinase (AK) which were higher than in non‐thalassaemic group of patients with increased number of reticulocytes. No correlation was found between the severity of P5N deficiency and the intensity of basophilic stippling which was present in 46 of 50 thalassaemic carriers here studied. In addition, GSH content and UV absorption spectra of deproteinized thalassaemic RBC extracts were also found to be normal.The present findings provide further information on the metabolic status of RBC in β‐thalassaemia trait and suggest a possible molecular explanation for the frequently observed basophilic stippling in this disease.
Serous effusions in monocytic leukaemias
Authors: Mufti G. J. , Oscier D. G. , Hamblin T. J. , Nightingale Anne , Darlow S. 
Published: March 2008

Summary. We report four patients who either presented with or developed serous effusions during the course of monocytic leukaemia. This finding has not been documented previously. All the patients had very high peripheral blood monocyte counts when effusions developed. The effusions resolved after the patients were treated with anti‐leukaemic therapy. It is therefore likely that the effusions were disease related. Two of the patients have been treated with raxoxane, one with eptoposide and the other with cytarabine.Monocytic leukaemias comprise a spectrum of disorders ranging from acute monoblastic leukaemias (AMOL) and acute myelomonocytic leukaemia (AMML) to chronic myelomono‐cytic leukaemias (CMML) (Shaw, 1980). Whereas leukaemic infiltration of the gums, central nervous system and skin is well recognized in AMOL and AMML, it is uncommon in CMML (Shaw, 19 78). In contrast to the lymphoid malignancies, leukaemic infiltration of the serosal surfaces giving rise to effusions is rare in both myeloid and monocytic leukaemias and has not been previously documented in CMML. We report four patients with monocytic leukaemia who developed either pleural and/or pericardial effusions or ascites which responded to anti‐leukaemic therapy.
Bone marrow transplantation for acute lymphoblastic leukaemia: a survey of the European Group for Bone Marrow Transplantation (E.G.B.M.T.)
Authors: Zwaan F. E. , Hermans J. , Barrett A. J. , Speck B. 
Published: March 2008

SUMMARYBetween 1979 and 1982, 192 patients with acute lymphoblastic leukaemia were given allogenic bone marrow transplants from HLA‐identical siblings. Data on 5 7 patients transplanted in first remission were compared with the results in 96 patients transplanted in second remission, and 39 in third or subsequent remission. The majority of these patients were of the non‐B non‐T‐ALL and T‐ALL subtypes. The 2‐year actuarial survival for non‐B non‐T‐ALL was 58% for patients transplanted in first remission, 34% for second remission patients and 33% for patients transplanted in third or subsequent remission (P= O60). For patients with T‐ALL, the survivals at 2 years for first and second remission transplant patients were 61% and 10%, respectively (P=0‐04). Multifactorial analysis demonstrated a significantly higher probability of survival for patients grafted in first remission (compared with more advanced remission states) and with bone marrow from young (age ≤ 20 years) donors. The 2‐year actuarial risk of relapse for patients with non‐B non‐T‐ALL transplanted in first remission was 0%, 32% and 69% for second and third or subsequent remission patients, respectively (P= 0‐04). For T‐ALL the actuarial risk of relapse at 2 years is 10% for first remission grafts and 48% for second remission grafts (P= 0‐07). Only patients (with both non‐B non‐T‐/and T‐ALL) transplanted in first remission have a high and stable probability of remaining in remission.
Fibronectin C in acute leukaemia
Authors: Reilly J. T. , Galloway M. J. , Mackie M. J. , McVerry B. A. 
Published: March 2008

SummaryFibronectin C (FN: C) is thought to represent fibronectin complexed with other plasma proteins and can be detected by a crossed two‐dimensional immuno‐electrophoretic technique. While we have not detected this on examination of normal plasma, FN: C was found in 26 of 33 patients (79%) with acute leukaemia at diagnosis. FN:C persisted throughout the induction phase of chemotherapy and disappeared only when a complete remission was obtained in 16 of 17 FN:C+ patients. FN:C has reappeared in the only patient whose leukaemia has relapsed to date. FN:C was present in both acute non‐lymphoblastic (85%) and acute lymphoblastic leukaemia (50%). Leukaemia remission rates were similar in patients who were FN: C+ or FN: C∼ (69% and 57%). Evidence is presented to suggest that the composition of FN:C is partially related to the binding of fibrinogen/fibrin to fibronectin in plasma. No significant difference in in vivo activation of coagulation was detected between FN: C+ and FN: C∼ patients. There was no correlation between the presence of FN: C and plasma fibronectin levels.
Acute myelogenous leukaemia modulated by B12deficiency: a case with bone marrow blast cell assay corroboration
Authors: Ahmann Frederick R. , Durie Brian G. M. 
Published: March 2008

SummaryA patient is described who appeared to demonstrate modulation of acute myelogenous leukaemia with vitamin B12deprivation. Support for this observation was obtained via anin vitrobone marrow blast cell assay. The addition of B12to this patient's bone marrowin vitrosignificantly stimulated the colony forming ability of the blast cells. Patients with B12deficiency have an increased incidence of haematopoietic malignancies and excessive replacement of B12may in some cases have the potential of accelerating such disorders. Intensive B12replacement in refractory megaloblastic anaemias should be done with caution.
Pure red cell aplasia: relationship between inhibitory activity of T cells to CFU‐E and erythropoiesis
Authors: Hanada Takashi , Abe Tsukasa , Nakamura Haruo , Aoki Yasuko 
Published: March 2008

SUMMARYT cell‐mediated inhibition of autologous late erythroid colony formation was found in two patients with PRCA. Each patient was treated separately with immunosuppressive agents, ALG, bolus methylprednisolone or cyclophosphamide. Cyclophosphamide was the most effective among these immunosuppressive therapies. Peripheral blood T cells, which were taken serially from the patients during the course of the disease, were cryopreserved until use. The inhibitory activity of T cells was assayed after remission using autologous bone marrow. It was found that the decrease of inhibitory activity was closely correlated with clinical improvement and that the effectiveness of the immunosuppressive therapy on inhibitory activities of T cells differed between therapies. These findings suggest that T cell‐mediated inhibition of erythropoiesis may be pathogenetic for PRCA in some patients.