bluuebunny/crossref_metadata_2025_split huggingface dataset:
52Fe Studies of the Effects of Treatment on Erythropoiesis in Megaloblastic Anaemia
Authors: Bruce‐Tagoe A. A. , Hoffbrand A. V. , Short M. D. , Szur L. 
Published: March 2008

Summary. 52Fe has been used to study iron turnover and the extent of haemopoietic tissue in five patients (age range 13–74 yr) with severe megaloblastic anaemia, before and after treatment. All the untreated patients showed extension of haemopoietic marrow, and this was most marked in the youngest subjects. One patient (aged 60) showed a considerable degree of extramedullary haemopoiesis in the spleen. The extent of haemopoietic tissue decreased after therapy in all cases and appeared to reach normal by about 4–5 mth.Plasma iron turnover was considerably increased before therapy in three patients and after 48 hr of therapy in the remaining two. Repeat studies after 5 weeks to 8 mth therapy showed a decrease in iron turnover in all five, but this was still raised at 5 weeks and 4 mth in two patients.
Application of Time Series Analysis to Serial Blood Neutrophil Counts in Normal Individuals and Patients Receiving Cyclophosphamide
Authors: Dale David C. , Alling David W. , Wolff Sheldon M. 
Published: March 2008

Summary.For periods of time ranging from 1 to 3 mth white blood cell and differential counts were made daily on eight normal volunteers and two patients receiving cyclophosphamide. The patients tended to have lower mean neutrophil (PMN) counts than the volunteers; moreover, there was a corresponding decrease in the variability of the counts around the means.The PMN counts appear to follow a lognormal distribution which would arise if the mechanism stabilizing the PMN counts adjusts the number of circulating cells in a multiplicative manner.Periodogram analysis of the log PMN counts showed evidence of regular periodic variation in only one of the subjects, a patient undergoing treatment with cyclophosphamide. The sequences of daily log PMN counts appeared to represent first‐order autoregressions with an average serial correlation coefficient of 0.125. These findings indicate that a typical series of daily PMN counts does not show cyclic variation.
Studies of the Procoagulant and Fibrinolytic Activity of Promyelocytes in Acute Promyelocytic Leukaemia
Authors: Gralnick Harvey R. , Abrell Elinor 
Published: March 2008

Summary.Malignant cells from four patients with acute promyelocytic leukaemia (APL) were analysed for coagulant and fibrinolytic activity. For comparison polymorphonuclear leucocytes from normal donors and myeloblasts from patients with acute myelogenous leukaemia (AML) were also tested. The APL cells compared to the other cells consistently shortened the recalcification time and partial thromboplastin time of normal, factor VIII deficient and factor IX deficient plasma, but had no effect on factor X deficient or factor VII‐X deficient plasma. APL cells demonstrated increased (250–1500%) tissue factor activity when tested in a two‐stage assay in which tissue factor is the rate limiting component. The increased tissue factor activity was primarily found in the granular fraction of APL cells. Fibrinolytic and proteolytic activity of the APL cells was variable. Cells from three of the APL patients demonstrated a slight increase (150–400%), while the cells from one APL patient had markedly diminished proteolytic activity. The increased fibrinolytic activity was associated primarily with the granular fraction. Although the fibrinolytic activity is increased, it does not parallel the increase in tissue factor activity. This imbalance of coagulation and fibrinolysis in the cellular composition of promyelocytes could explain the high incidence of clinical thrombo‐haemorrhagic disorders related to intravascular coagulation in APL.
Effects of Ancrod (Arvin) in Mice: Studies of Plasma Fibrinogen and Fibrinolytic Activity
Authors: Silberman Simone , Bernik Maria B. , Potter Elizabeth V. , Kwaan Hau C. 
Published: March 2008

Summary.Plasma fibrinogen and its degradation products (FDP) were examined in mice given single injections of Ancrod, the purified coagulative fraction of Malayan pit viper venom previously called Arvin. Clottable fibrinogen had disappeared completely 5 min after the injection and slowly reappeared 12 hr later. At least 2 FDP appeared in the plasma 30 min after the injections while 5 hr later only one FDP was present which corresponded to human fragment E. This was in contrast to in vitro lysis of mouse fibrinogen which yielded two late FDP corresponding to human fragments D and E.The fibrinolytic system also was examined in the plasma of these animals. 30 min after the injection, plasminogen was decreased in amount but increased in susceptibility to activation by urokinase. Transient, modest increases in fibrinolytic activity were evident in diluted plasma samples 1 hr and again 8 hr after the injections. Inhibitors of fibrinolysis did not differ from those in normal mice.These observations are correlated with our previous immunofluorescent and histochemical studies on these and other animals. The present data support our earlier conclusion that fibrinolysis within small vessels of the tissues is of major importance in the reaction to Ancrod.
Antiplasmin Activity of Rat Serum Slow α‐Globulins
Authors: Horne C. H. W. , Forbes C. D. , Prentice C. R. M. 
Published: March 2008

Summary.The antiplasmin activity of serum slow α1‐ and α2‐globulins has been studied in rats injected with streptokinase/human serum and human or rat plasmin. Highly significant falls in serum slow α1 ‐globulin were noted soon after administration of streptokinase and human or rat plasmin; this suggested a reaction between plasmin and slow α1‐globulin with rapid removal of the resultant complexes. Rats with immune complex nephritis (ICN) given streptokinase showed a highly significant increase in slow α2‐globulin, but no change in slow α2‐globulin was noted following injection of human plasmin. Using fibrin agar plates additional evidence was obtained that rat slow α1‐globulin inhibited digestion of fibrin by plasmin. We conclude that of the two globulins in the rat only slow α1‐globulin shows antiplasmin activity and we believe that this represents the functional analogue of α1‐macroglobulin in the human.
Nonspherocytic Haemolytic Anaemia with Increased Red Cell Adenine Nucleotides, Glutathione and Basophilic Stippling and Ribosephosphate Pyrophosphokinase (RPK) Deficiency: Studies on Two New Kindreds
Authors: Valentine William N. , Bennett John M. , Krivit William , Konrad Patricia N. , Lowman James T. , Paglia Donald E. , Wakem Christopher J. 
Published: March 2008

Summary.The second and third kindreds with hereditary haemolytic anaemia characterized by marked basophilic stippling of the erythrocytes, increased red‐cell GSH and adenine nucleotides, and ribosephosphate pyrophosphokinase (RPK, PRPP synthetase) deficiency are reported. In each affected subject increased auto‐haemolysis was not corrected by glucose additives in the autohaemolysis test. Despite the clearly hereditary nature of the syndrome, a heterozygous ‘carrier’ state could not be demonstrated in any family members. While autosomal recessive transmission is likely, the genetics remain obscure. The roles of substantial RPK deficiency as either a causative factor or an epiphenomenon is likewise not firmly established.
Pregnancy and Idiopathic Autoimmune Haemolytic Anaemia: A Prospective Study during 6 Months Gestation and 3 Months Post‐Partum
Authors: Jr Chaplin, Hugh , Cohen Robert , Bloomberg Gordon , Kaplan Harold J. , Moore Joy A. , Dorner Irene 
Published: March 2008

Summary.A 31‐yr‐old woman with a 12 yr history of relapsing idiopathic autoimmune haemolytic anaemia was studied prospectively during her first pregnancy. Her serum contained a warm incomplete autoantibody as well as an elevated cold agglutinin; her red blood cells were strongly coated with IgG and complement (chiefly α2D). Haemolysis was active throughout pregnancy, accelerating from the 34th to 40th week, with developing thrombocytopenia. Amniocentesis in the 8th and 9th months suggested minimal foetal haemolysis. The maternal haemolytic process went into complete clinical remission following delivery of a healthy appearing infant whose red cells were coated with IgG. The infant developed mild hyperbilirubinaemia within 48 hr and experienced a fall in haemoglobin to 50% of the cord level by the 8th week. Abnormalities of maternal and infant C4 levels were observed. Review of 19 reported instances of presumed autoimmune haemolysis during pregnancy revealed life‐threatening anaemia in nearly 50% of mothers, with four still‐births, one neonatal death, and three seriously affected infants. A programme for prospective management of this serious clinical problem is discussed.
Production of Paroxysmal Nocturnal Haemoglobinuria‐like Red Cells by Reducing and Oxidizing Agents
Authors: Goldstein Bernard D. 
Published: March 2008

Summary. Normal red cells incubated with high concentrations of compounds containing sulphydryl groups are known to develop several characteristics of red cells obtained from patients with paroxysmal nocturnal haemoglobinuria (PNH). These include positive acid and sucrose haemolysis tests and a decline in acetylcholinesterase (AChE) activity. The present studies demonstrate that incubation of normal red cells with the non‐sulphydryl reducing agent sodium borohydride also results in the production of PNH‐like characteristics. Evaluation of this phenomenon utilizing the sulphydryl agents glutathione (GSH) and dithiothreitol (DTT) reveals that the transformation is not dependent upon the presence of oxygen, is not associated with a significant decline in red cell vitamin E levels, and is not observed when GSH is rapidly oxidized by azoester. It appears that the most likely mechanism for the formation of PNH‐like red cells under these conditions is through the reduction of cell membrane disulphide groups.Production of a positive sucrose and acid haemolysis test as well as a decline in AChE activity was also observed in two experimental systems which produce oxidative free radical damage to the red cell membrane: gassing of normal red cells with ozone, and irradiation of red cells from patients with erythropoietic protoporphyria with 400 nm light.
A Vitamin B12 Binder with Transcobalamin I Characteristics Synthesized and Released by Human Granulocytes in Vitro
Authors: Rachmilewitz B. , Rachmilewitz M. , Gross J. 
Published: March 2008

Summary. In‐vitro synthesis and release of a vitamin‐B12 binding protein by peripheral leucocytes derived from healthy subjects and patients with chronic myeloid leukaemia (CML), acute promyelocytic leukaemia (APL) and acute myeloblastic leukaemia (AML) were investigated.Myeloid cells, incubated in a nutrient medium containing [3H]leucine, incorporated labelled amino‐acid into the vitamin‐B12 binding protein with an increase of the unsaturated vitamin‐B12 binding capacity (UBBC) of the cells.The increase in the UBBC of the medium was due to the presence of the newly produced leucocyte binder which was released into the medium by the cells during incubation. On chromatography the newly synthesized binder was similar to transcobalamin I.The incorporation of [3H]leucine into the leucocyte binder by mature granulo‐cytes and undifferentiated myeloblasts was very limited and reached a maximum after 60 min of incubation. Cells from CML and APL patients showed a higher rate of incorporation of the labelled amino‐acid which reached a plateau after 40 hr. The increase in the UBBC of the leucocyte binder was paralleled by the incorporation of [3H]leucine.The percentage of immature myeloid cells in the incubation mixture (promyelocytes to stab forms) was correlated with both the incorporation of [3H]leucine and with the increase in UBBC.
Ethanol Inhibition of Haemoglobin Synthesis: In Vitro Evidence for a Haem Correctable Defect in Normal Subjects and in Alcoholics
Authors: Ali M. A. M. , Brain M. C. 
Published: March 2008

Summary.The effect of ethanol in different concentrations ranging from 0.10 to 0.66 m on globin chain synthesis in vitro was studied by measuring 3H‐leucine incorporation into human bone marrow suspensions and reticulocyte‐enriched blood. With increasing concentration of ethanol there was a decreasing 3H‐leucine incorporation. This inhibition was reversed by haem and pyridoxine hydrochloride (PHC) in i mM concentration. 5‐Aminolaevulinic acid (5‐ALA) at similar concentration had no significant effect. The addition of haem to reticulocyte‐enriched blood obtained from acute alcoholics increased globin‐chain synthesis by 20% whereas PHC and ALA were without effect and none of the additions increased globin synthesis by normal reticulocytes. These data provide direct evidence for the inhibitory action of ethanol on haemoglobin synthesis and suggest that the effect is mediated through inhibition of haem synthesis at a site influenced by pyridoxine.
Abnormal Cells in the Peripheral Blood of Patients with Hodgkin's Disease
Authors: Halie M. R. , Splett‐Romascano M. , Molenaar I. , Nieweg H. O. 
Published: March 2008

Summary.The investigation of cells larger than normal in the peripheral blood of patients with Hodgkin's disease by electron microscopy revealed, besides cells of the granulocytic series and parts of megakaryocytes, four other categories. The cells of the mononuclear phagocytic system could easily be recognized. The so‐called immunoblasts seen in light microscopy as dark basophilic lymphoid cells apparently consist of cells from B‐ as well as T‐cell origin, according to their ultrastructural characteristics. All these cells could also be found in nine healthy control persons, two patients with cytomegalovirus disease and two with reticulosarcoma.The fourth category was formed by a rare cell, only encountered in patients with disseminated Hodgkin's disease. They probably represent the moderately basophilic blast cell seen in light microscopy, considered to indicate dissemination of Hodgkin's disease. Submicroscopic details of these cells are given. It is very improbable that they belong to the monocytes or to the immunoglobulin producing cells. A relation to the thymus derived immunoblasts could not be excluded, while on the other hand the morphological features closely resembled those of cells found in cases of type I and type III of reticulosarcoma (Mori & Lennert, 1969). Thus, in this investigation the number of possibilities for the origin of the typical cells indicating splenic involvement of Hodgkin's disease was limited, but a definite conclusion could not be reached on the basis of the presently available morphological data.
Blood Catecholamine Levels, Factor VIII and Fibrinolysis after Therapeutic Electroshock
Authors: Pina‐Cabral J. M. , Rodrigues Custoadio 
Published: March 2008

Summary.Two groups of patients were submitted to electric convulsive therapy, one without pre‐treatment (ECT) and the other after administration of thiopentone and succinylcholine (modified ECT). After ECT, there was a large increase in the blood level of catecholamines (noradrenaline+adrenaline), of factor VIII and of plasminogen activator. After modified ECT, the release of catecholamines and of factor VIII was significantly reduced, but hyperfibrinolysis took place as in ECT. These results suggest that while catecholamines are probably not responsible for plasminogen activator release during stress, adrenergic mediation of the concomitant increase in factor VIII is highly probable.
The Role of Complement in Endotoxin‐Induced Disseminated Intravascular Coagulation: Studies in Congenitally C6‐Deficient Rabbits
Authors: Müller‐Berghaus Gert , Lohmann Elke 
Published: March 2008

Summary.The effect of intravenous infusion of endotoxin on the activation of intravascular coagulation and on the generation of glomerular microclots was studied in rabbits congenitally deficient in the sixth component of complement (C6). Endotoxin infusion induced the formation of circulating soluble fibrin and caused a drop in leucocyte and platelet counts, a decrease in factor‐V and factor‐VII activities and a prolongation of the thromboplastin time as well as the partial thromboplastin time. These haematological changes are indicative of consumption coagulopathy and were similarly distinctive in C6‐deficient as in normal rabbits infused with endotoxin. Furthermore, renal glomerular microclots formed in 90% of the C6‐deficient and in 80% of the normal rabbits after endotoxin administration. The haematological as well as the histological results of these experiments demonstrate that the sixth component of complement and the later complement components C7–9 are not essential to the initiation of intravascular coagulation by endotoxin and the manifestation of the generalized Shwartzman reaction. The data make it likely that the release of platelet factor 3 does not represent the trigger mechanism of intravascular coagulation since disseminated intravascular coagulation could be induced in C6‐deficient rabbits although the platelets of these animals are incapable of releasing platelet factor 3 activity after platelet‐endotoxin interaction.
Megakaryocyte Polyploidization in Acute Leukaemia and Preleukaemia
Authors: Queisser W. , Queisser U. , Ansmann M. , Brunner G. , Hoelzer D. , Heimpel H. 
Published: March 2008

Summary. Megakaryocyte polyploidy was studied in six cases of preleukaemic acute leukaemia (PL), and in acute leukaemia (AL), five cases in the overt phase and three during complete remission. The megakaryocyte polyploidization was determined cytophotometrically and in some cases also by in vitro labelling with tritiated thymidine (3H‐TdR). The results obtained in PL and untreated AL. demonstrate an impairment of the 3H‐TdR labelling index and altered megakaryocyte polyploidy such as decrease of the maximum polyploidization capacity (five cases), evidence of diploid megakaryocytes (two cases) and of hyperploid megakaryocytes (six cases). These disturbances reflected the cytological abnormalities (microkaryo‐cytes, polykaryocytes) observed in most of the cases studied.In contrast, in cases studied during complete remission of AL. a normal labelling index and polyploidy composition was observed, suggesting that the polyploidization disturbances are reversible. A strict correlation of the polyploidization defect and the platelet production could not be established from the patients investigated.
Serum Colony Stimulating and Inhibiting Factors in Irradiated Germfree and Conventional Mice
Authors: Quesenberry P. J. , Bealmear P. , Ryan M. , Stohlman F. 
Published: March 2008

Summary. Colony‐stimulating factor (CSF) elevations were seen at varying times after lethal irradiation of conventional CF, and CFW mice but no elevations were noted in germfree CFW mice. Changes in serum inhibitor activity after irradiation were variable and did not explain the CSF increases. Both conventional and germfree mice responded to endotoxin with similar increases in serum CSF before and after irradiation. Serum CSF levels in mice with irradiation‐induced myeloid aplasia may be normal and yet these myeloid depleted mice retain the capacity to generate increased serum CSF levels after endotoxin injection.These data suggest that in the irradiated mouse serum CSF levels are not determined by the peripheral blood or marrow neutrophil numbers, but are more likely related to a functional demand on the neutrophil such as bacterial killing.
Platelet Survival and Platelet Production in Idiopathic Thrombocytopenic Purpura (ITP)
Authors: Branehög Ingemar , Kutti Jack , Weinfeld Aleksander 
Published: March 2008

Summary. Platelet mean life span (MLS) and platelet production were measured in 3–5 patients with idiopathic thrombocytopenic purpura (ITP) and in 21 healthy subjects.The mean platelet production in ITP was 2.3 times normal: the highest values were 3–5 times normal. There was a highly significant negative correlation between platelet production and peripheral platelet count. With platelet counts above 50000μl, platelet turnover was within the upper part of the normal range, but with lower platelet counts, turnover progressively increased. It is concluded that the bone marrow in ITP increases platelet production in response to a low platelet count and that this response does not differ from that hitherto known to occur in man and animals rendered thrombocytopenic by thrombopheresis.The disappearance curve of 51Cr labelled platelets in ITP consists of two components, a rapid initial one covering the first 15 min after infusion and then a slower one. The pattern was the same whether autologous or homologous platelets were used for labelling. It is suggested that the initial part of the curve does not represent in vivo survival but is due to slight damage to the platelets during the labelling procedure. These slightly damaged cells can resume normal viability when infused into a normal recipient but are rendered less viable when further damaged by platelet antibodies in patients with ITP. This explains the low recovery of infused labelled platelets in ITP recipients, despite the fact that the size of their splenic platelet pool is normal.
Hereditary Spherocytosis: The Metabolism of Erythrocytes in the Peripheral Blood and in the Splenic Pulp
Authors: Mayman David , Zipursky Alvin 
Published: March 2008

Summary. Na+ transport was studied in intact red cells and membrane preparations (‘ghosts’) of patients with hereditary spherocytosis (h. s.). Ouabain‐insensitive efflux was greater than normal in ghost preparations as well as in intact cells, findings which arc consistent with a membrane defect in h.s. red cells. Ouabain‐sensitive (‘pump’) sodium efflux was normal in h.s. membrane preparations, but was increased in intact cells and the latter probably reflects changes secondary to the increased Na+ influx in these cells.In h.s., red cells are trapped and destroyed in the spleen and we studied splenic pulp red cells in a group of h.s. patients at splenectomy. These cells showed greater osmotic fragility, higher Na+ and lower K+ concentrations than did peripheral red cells. Membrane permeability to Na+ was not greater than in peripheral red cells but the ouabain‐sensitive (‘pump’) Na+ efflux was reduced. Despite this, ATP concentration and ATPase activity were found to be normal. Low levels of 2,3‐diphosphoglyceric acid were noted in splenic red cells but ATP turnover was normal. Since failure of the Na+ pump occurs in h.s. splenic red cells despite adequate substrate (ATP) and an intact ATPase system, it is suggested that the abnormality results from a change in the red cell membrane 4 which reduces the function of the pump. These characteristics of splenic red cells are similar to those found in normal red cells following in vitro incubation at 37°C. This supports the concept that cell death in h.s. results from a deterioration in membrane function secondary to substrate deprivation in the stagnant circulation of the splenic pulp.
An Association between Aplastic Anaemia and Sideroblastic Anaemia
Authors: Geary C. G. , Dawson D. W. , Sitlani P. K. , Allison H. A. , Leyland M. J. 
Published: March 2008

Summary. Four cases of marrow aplasia are described in which sideroblastic anaemia eventually developed. In two cases, the diagnosis of sideroblastic anaemia was established during a phase of partial recovery from the original aplastic episode. In the other two, periods of respectively 6 and 20 yr separated the two diagnoses though, in the latter, persistent thrombocytopenia had suggested that marrow recovery was incomplete.It is suggested that an abnormal erythroid clone, characterized by the presence of ring sideroblasts, may arise during recovery from an aplastic episode, a situation somewhat analogous to the development of PNH in a patient with aplastic anaemia. Apparent clinical remission might mask this underlying haematological abnormality, in some cases, for years.