bluuebunny/crossref_metadata_2025 huggingface dataset:
Unusual Pattern of Inheritance of Haemoglobin‐H Disease
Authors: Alessio Lorenzo , Sulis Efisio , Pabis Attilio , Mannucci Mannuccio P.
Published: August 2010

A 35‐year‐old Sardinian woman with haemoglobin‐H disease was found to have normal parents. This case with an unusual inheritance pattern is described, and its possible relevance for the current concepts of haemoglobin‐H disease genetics is discussed.
A New Vitamin B12 Binding Protein in Serum Causing Excessively High Serum Vitamin B12 Values
Authors: Schwartz Michael , Bastrup‐Madsen P.
Published: August 2010

Following treatment with a vitamin B12 preparation with retarded effect, extremely high serum vitamin B12 values were observed in several cases. The explanation for this phenomenon is a very high serum vitamin B12‐binding capacity, caused by a vitamin B12 binding protein located in the γG area – presumably an antibody.
Haemoglobin Sogn (β14 Arginine) A New Haemoglobin Variant
Authors: Monn E. , Gaffney P. J. , Lehmann H.
Published: August 2010

Among 3,000 Norwegian individuals two haemoglobin variants were found. One was a Haemoglobin D Punjab. The other was a new variant, Haemoglobin Sogn, in which leucine is replaced by arginine at the internally sited residue 14 of the β chain. The haemoglobin variant shows a slight heat instability.

Heterozygotes for Haemoglobin Sogn show a small amount of free α chains in their haemolysates, but no Heinz bodies are present in their erythrocytes and they are clinically healthy.
Studies on Two Patients with Concomitant Intrinsic Factor Secretory Defect and Jejunal Diverticulosis
Authors: Murray‐Lyon Iain M. , Finlayson Niall D. C. , Shearman David J. C.
Published: August 2010

Two cases of jejunal diverticulosis associated with intrinsic factor deficiency are reported. One case had megaloblastic anaemia and full correction of 58Co vitamin B12 absorption was achieved by broad spectrum antibiotic therapy alone. The second case had intrinsic factor deficiency and full correction of 58Co vitamin B12 absorption was achieved by the addition of oral intrinsic factor alone. The variable contribution made to the development of vitamin B12 deficiency in jejunal diverticulosis by intrinsic factor deficiency and bacterial colonisation of the small intestine is discussed.
Ring‐Shaped Nuclei of Granulocytes in a Patient with Acute Erythroleukaemia
Authors: Stavem Per , Hjort Peter F. , Vogt Erik , Hagen Carl B. van der
Published: August 2010

A patient with acute erythroleukaemia had a large number of ring‐shaped granulocyte nuclei. Cytogenetic studies showed normal chromosomes. A hereditary anomaly was unlikely, since his 2 children were normal. Therefore, the anomaly was probably acquired and a result of the acute erythroleukaemia.
The Mode of Expressing the Content of Intracellular Components of Human Erythrocytes with Special Reference to Adenine Nucleotides
Authors: Hjelm Magnus
Published: August 2010

No general agreement has been reached so far for the mode of expressing the content of intracellular components in human erythrocytes. The validity of the measures (1) moles per cell, (2) moles per cell volume and (3) moles per intracellular haemoglobin has been evaluated from a theoretical point of view in comparisons of different cell populations and in kinetic considerations. It was found that none of the expressions were valid if the intracellular concentration of water soluble components is concerned. The use of the expression (4) moles per liter of apparent cell water calculated from (2) and MCHC appears to be as a better approximation of the intracellular concentration of the components concerned.

The consequences of these theoretical considerations when evaluating biochemical investigations on human erythrocytes, are exemplified by determinations of adenine nucleotides (ATP, ADP) in erythrocyte populations of differing mean age from patients with non‐spherocytic haemolytic anaemia and thalassaemia minor, and from healthy individuals. It can be shown that the conclusion to be drawn from the determinations are highly dependent on the mode of calculating the result.
Quantitative Studies on the Normal Decay of Lymphocytes in the Thymolymphatic System
Authors: Claesson Mogens Helweg
Published: August 2010

Studies on the number of dying and dead lymphocytes in unfixed lymphocyte suspensions from thymus, mesenterial lymph nodes and different peripheral lymph nodes from mice were done by employing the Dye Exclusion Test. The number of decaying lymphocytes demonstrates a striking variation within the various age‐groups.

During the first months of life the number of dying lymphocytes in the thymus is rather low ‐ from 3 to 5%. It amounts to 15 to 20% between the ages of 3 and 12 months. In suspensions from mesenterial and peripheral lymph nodes the lymphocyte decay during the first two months of life is twice as large as the thymocyte decay. After this the cell decay in the different lymphoid organs becomes gradually similar. About the time of 5 months of age and later the number of decaying lymphocytes and thymocytes are almost identical.

Corresponding studies on thymus suspensions from mouse foetuses showed that a rather striking thymocyte decay takes place during the 16th day of foetal life (8%), while the number for the 18th day of foetal life were as low as 2%.

The findings have been discussed on the basis of knowledge of two lymphocyte populations, one short‐lived and one long‐lived, as well as the theory concerning thymocyte migration.
The Role of Phagocytic Cells in the Immune Reaction of Rabbit Blood Lymphocytes in Antigen Stimulated Cultures
Authors: Lamvik J. O.
Published: August 2010

A reduction in blastoid transformation, development of plaque‐forming cells and liberation of agglutinins was noticed in blood lymphocyte cultures stimulated with sheep red cells (SRC), when the cultures were prepared with reduced numbers of phagocytic cells. In cultures which gave a high immune response, numerous rosettes composed of a central macrophage surrounded by proliferating lymphocytes, were observed. The findings support the view that phagocytic cells may play an important role in preparation and transfer of antigen to lymphocytes which are able to proliferate and transform into antibody producing cells.
Pyruvate Kinase in Human Erythrocytes
Authors: Hanel H. K.
Published: August 2010

The importance of efficient separation of erythrocytes from leucocytes when studying haemolysates is shown experimentally, pyruvate kinase determination given as an example. The use of the spiral centrifuge is recommended.
The Role of Oxygen Supply in the Regulation of Erythropoiesis in Compensated Anaemia
Authors: Neĉas E. , Neuwirt J.
Published: August 2010

The problem of the regulation of erythropoiesis in compensated anaemia was studied. The complete compensation of increased haemolysis in experimental haemolytic anaemia, induced either by phenylhydrazine or methionine and in chronic post‐haemorrhagic anaemia was not achieved. The exposure of rats with haemolytic anaemia induced by methionine to an air pressure of 4 atmospheres absolute led to a substantial suppression of the stimulated erythropoiesis. Our results do not support the hypothesis of the existence of two or more regulating mechanisms in erythropoiesis.
Cytochemical Variants of Neutrophil Leukocyte Populations in Chronic Myelocytic Leukaemia
Authors: Gahrton G. , Brandt L. , Franzén S. , Nordén Å.
Published: August 2010

A microspectrophotometric method was used for determination of the amount of periodic acid‐Schiff reactive material (PASMa), most likely a measure of the glycogen content, in individual blood and bone marrow neutrophil leucocytes from seven cases of chronic myelocytic leukaemia (CML) and five non‐leukaemic subjects. Neutrophils from untreated CML contained low PASMa amounts as compared to neutrophils from normal subjects, while normal amounts of PASMa were found in neutrophils from CML patients in busulfan‐induced remission. Blood and bone marrow neutrophils in the same individual subject contained similar PASMa amounts whether sampled from a normal person, untreated CML (low PASMa level), polycythaemia vera (high PASMa level), or CML in complete remission (normal PASMa level). Treatment of CML with busulfan initiated earlier normalization of PASMa amounts in bone marrow neutrophils than in blood neutrophils, although similar normal PASMa amounts were found in both blood and bone marrow neutrophils in complete remission. The pattern of normalization showed that the busulfan‐induced normalization of PASMa content in CML‐neutrophils was due to action on precursor‐myeloid cells and not to direct action on mature neutrophils. The PAS‐normal cells in remission might indicate a metabolic change of the leukaemic myeloid cell population or represent a second ‘normal’ population of neutrophil leucocytes.
Catheter Material and Blood Coagulation Studies in vitro
Authors: Jacobsson Bo , Nilsson Inga Marie
Published: August 2010

An in vitro investigation of coagulation factors revealed that contact of blood with the wall of a catheter activates the intrinsic blood‐coagulation system. This implies a risk of the formation of a clot in stagnant blood in the tip of an indwelling vascular catheter. It is also conceivable that such contact activation may have an effect on the circulating blood.
The Activator Time
Authors: Blix Sverre
Published: August 2010

A simple and rapid method for evaluation of activator activity in patients during thrombolytic therapy‐the activator time‐is presented. It is based upon a clot lysis system with an euglobulin solution from normal plasma as substrate, but fibrinogen could also be used. The method may tell whether the patient is sufficiently treated or not, and the possibility of establishing a therapeutical range is suggested.
An Evaluation of Aryl Sulphatase Activity in Leukæmic Cells
Authors: Boysen Gudrun
Published: August 2010

A histochemical method for demonstrating aryl sulphatase activity in haemic cells has been applied to 22 cases of leukæmia of different types. The difference in distribution of activity in myeloid and lymhatic cells, which has been suggested as an aid in the classification of acute leukæmias, has not been confirmed.
The Relationship between Haem and Globin Synthesis by Erythroid Precursors in Refractory Normoblastic Anaemia
Authors: Kramer S. , Viljoen E. , Becker D. , Zail S. S. , Metz J.
Published: August 2010

The specific activity of glycine‐2‐14C into haem and globin has been studied in normal marrow, in cases of thalassaemia and refractory normoblastic anaemia, in patients with iron deficiency anaemia and porphyria of erythropoietic origin. In controls the ratio of haem to globin was less than 1.0. This ratio was reduced in the 2 cases of thalassaemia probably due to the associated reduced activity in ALA synthetase and haem synthetase enzymes. Haem synthesis by iron deficient erythroid precursors can be stimulated by iron with corresponding increase in globin synthesis. There is apparently a mechanism controlling the relationship between haem and globin. Some cases of sideroblastic anaemia resemble patients with thalassaemia in the reduction of the ratio of haem to globin and in the marked reduction of the synthesis of haemoglobin when related to cell counts.

When the ratio of the specific activity of haem to globin is reduced the in vitro addition of pyridoxine to the duplicate may allow one to determine whether it will be effective in therapy.

Reduced globin synthesis in pre‐leukaemic sideroblastic anaemia may carry a poor prognosis but conversely adequate globin production in relationship to cell counts may be associated with a rapidly fatal outcome.

Only in the patient with erythropoietic porphyria was the specific activity of glycine‐2‐14C into haem synthesis greater than into globin. This finding was in contrast to that of erythropoietic protoporphyria where the ratio was normal.
Mathematical Correlation Between ADP‐Induced Platelet Aggregation and Subsequent Disaggregation
Authors: Holdrinet A. , Ewals M. , Reichert W. J. , Haanen C.
Published: August 2010

The turbidimetrically obtained curves of ADP‐induced platelet aggregation in plateletrich plasma at 37° C. can be mathematically characterized by a logarithmically normal frequency distribution suggesting that the phase of aggregation and the subsequent disaggregation are governed by the same mechanism. Every curve can be described with reference to three parameters, viz.: the maximum change in optical density (ODmax), the time at which this occurs (tmax and the standard deviation of the logarithms of the time variable (s̀).

Aggregation curves of 21 normal subjects, 17 uraemic patients and 11 patients with Von Willebrand̂s disease were studied.

Parameter ODmax although dependent on the platelet concentration was significantly diminished in a proportion of the uraemic patients.

Neither group of patients differed significantly from the normals in parameter tmax. A significant number of uraemic patients and Von Willebrand patients showed lower s̀ values than those found in normal subjects. The abnormality thus indicated entails abnormally rapid disaggregation.

The new parameter (s̀) proved to be a better reproducible and more sensitive measure of the aggregation process than the hitherto usual parameters ODmax and tmax.
Thymus and Node Labelling by Intramediastinal Injection of Tritiated Tracers and Its Bearing on Present Concepts of Lymphocyte Cytokinetics
Authors: Sainte‐Marie Guy , Messier Bernard
Published: August 2010

Since current intravenous or subcutaneous injection of 3H‐thymidine produces a relatively weak labelling of the thymus, an attempt was made to improve this labelling. The newly devised procedure, which consists in injecting the marker intramediastinally, labels the thymus much more heavily than the aforementioned techniques. The observations revealed that the inadequate labelling resulting from intravenous or subcutaneous injections is due to the non‐uniform availability of 3H‐thymidine and that this shortcoming can be rectified by using the intramediastinal route.
Thrombopoietin Activity in Children with Cyanotic Congenital Heart Disease
Authors: Goldschmidt Béla
Published: August 2010

The thrombopoietic activity of the plasma of children suffering from cyanotic congenital heart disease in recipient mice has been studied. Considerable activity was found in each thrombopenic patient and in more than half of the cases with normal thrombocyte count as well. There was no direct correlation between the platelet count and haemoglobin and plasma thrombopoietic activity. Increased activity was found in every patient in whom the arterial blood O2 saturation was below 80%.
Influence of Splenectomy on Thymic Release of Lymphocytes into the Blood
Authors: Ernström U. , Sandberg G.
Published: August 2010

Migration of thymic cells into the blood was studied 3, 7, 14 and 28 days after splenectomy or sham‐operation of young guinea‐pigs. This was done by determination of the content of different white cells in thymic afferent and efferent blood, the lymphocytes being divided into subclasses with different mitochondrial content. Also, the weights of the remaining lymphoid organs were registered. The following effects of splenectomy were obtained.

Early after splenectomy, the experiments indicated increased release of lymphocytes from the thymus. Thus, at 3 days the thymic veno‐arterial difference in number of lymphocytes with 0–5 mitochondria (smallest cells) was increased above that in the sham‐operated animals. At 7 days after splenectomy the increase included both lymphocytes with low and also with medium mitochondrial content (small and medium‐sized lymphocytes).

Later after splenectomy, the thymic veno‐arterial lymphocyte difference was no longer increased. At 28 days, lymphocytosis occurred in the blood, concurrent with increased weight of cervical lymph nodes.

It is proposed that these effects of splenectomy are caused by the loss of normal inhibition of lymphocyte production exerted by the spleen.
Cytoplasmic Microtubules and Radial‐Segmented Nuclei (Rieder Cells)
Authors: Norberg Bo
Published: August 2010

This investigation is concerned with the basic mechanisms of the radial segmentation (RS) of nuclei in human lymphocytes and monocytes. Some experimental conditions fundamental to RS‐studies were also investigated. Osmotic variations were found to influence unspecifically the recording of the RS of nuclei in the experimental system used. The RS was inhibited by pH depression and by low concentrations of ethanol and hexylene glycol, i.e. agents that might be expected to stabilize cytoplasmic microtubules under the conditions prevailing. Through an effect analogous to that of other spindle poisons, the RS was inhibited by podophyllic acid ethylhydrazide and griseofulvin, i.e. agents that cause a rapid decomposition of microtubules. These findings are interpreted to indicate that the RS of nuclei is due to a contraction of intermitotic residues of the mitotic spindle, parallel to a slow dissociation of the molecular units of its constituent microtubules. The recording of the RS of nuclei may form a convenient screening test for metaphase‐blocking cytostatics.
The Identity of the Metachromatic Substance of Basophilic Leucocytes
Authors: Olsson I. , Berg B. , Fransson L. Å. , Nordén A.
Published: August 2010

The metachromatic anionic polysaccharides of basophilic leucocytes from a patient with chronic myeloid leukaemia displaying a very high basophilic leucocyte count has been examined. A polysaccharide containing glucosamine was isolated. It was resistent to testicular hyaluronidase but sensitive to bacterial heparinase and incorporated (35S)‐sulphate and (14C)‐glucosamine indicating its identy with heparin. Density gradient centrifugation of the cells showed that the heparin‐like substance was present in the basophilic leucocytes. Gel chromatography demonstrated that only the largest molecules of the polysaccharide population incorporated (14C)‐glucosamine while a pool of smaller molecules remained unlabelled.
Inhibition of Thrombin Induced Aggregation of Human Platelets by Antithrombin III
Authors: Eika C. , Abildgaard U.
Published: August 2010

The influence of antithrombin III (At‐III) on the thrombin induced aggregation of human platelets was studied by the aggregometer technique according to Born. When At‐III was mixed with platelet suspension, subsequent addition of thrombin resulted in decreased aggregation as compared to the control. The inhibition was dependent on the At‐III: thrombin ratio. Above 0.2 NIH U/ml of thrombin, no inhibitory effect was observed with physiological At‐III concentrations. The effect was more pronounced at 37° C than at 20° C.
Frequency of the Australia‐SH Antigen and Antibody among Norwegian Blood Donors
Authors: Solaas Marit Hornberg
Published: August 2010

The frequency of the Australia‐SH antigen among 3,162 Norwegian blood donors was found to be 0.16% whereas the frequency of the corresponding antibody was 0.03%.
Thrombotest and the Diagnosis of Disseminated Intravascular Coagulation (DIC)
Authors: Godal H. C. , Kierulf P.
Published: August 2010

In 6 patients with diffuse intravascular coagulation (DIC) (3 of them on long term anticoagulant treatment with warfarin sodium), low Thrombotest values and a discrepancy between Thrombotest and Normotest was found. During heparin treatment, the Thrombotest values rose and the Thrombotest‐Normotest discrepancy decreased. These observations indicate that Thrombotest may be a sensitive indicator of consumption coagulopathy. A rise in Thrombotest during heparin treatment probably substantiates this concept.
Plasmin Digestion of Stabilized and Non‐Stabilized Fibrin Illustrated by Immunoelectrophoresis and Haemagglutination Inhibiton Immuno Assays
Authors: Feddersen C. , Gormsen Johs.
Published: August 2010

Digestion of stabilized and non‐stabilized fibrin in clots made from isolated fibrinogen, contaminated by fibrin stabilizing factor (FSF), and plasma is evaluated qualitatively by Immunoelectrophoresis and quantitatively by haemagglutination inhibition immuno assays (HIIA). Non‐stabilized systems are obtained by means of SH‐ and transamidase inhibitors and, in plasma systems, also by genuine FSF‐decient plasma. Stabilized and non‐stabilized fibrin in fibrin clots is easily degradated by plasmin (porcine) to the final D and E antigenic fragments, which are non‐reactive in HIIA. Stabilized fibrin in plasma clots is rather resistant to activator‐induced lysis, as antigenic intermediates, reactive in HIIA, remain even after sustained activator (+ plas‐minogen)‐induced lysis. These intermediates are degradated by high concentrations of plasmin to the final breakdown products, non‐reactive in HIIA.
DISCUSSION
Authors: Christensen B. Egelund
Published: August 2010

Erythrocyte sequestration was studied in 24 patients with chronic lymphocytic leukaemia (CLL) by infusion of autologous 51Cr‐labelled erythrocytes followed by surface measurements at regular intervals over the spleen, liver, and heart.

To obtain a comparable expression of the accumulation of activity in spleens of different sizes, the splenic surface activities were corrected. The correction factors were determined using fluid‐filled balloons (simulating spleens) with the same amount of activity.

In 19 patients with CLL the splenic erythrocyte pool increased with increasing spleen weight (500–4, 300 g). Moreover, the erythrocyte sequestration in the spleen increased with increasing pool. The maintenance of a large erythrocyte pool makes increased demands upon the erythropoiesis, which is perhaps already insufficient, and an increased splenic erythrocyte sequestration contributes to reducing the number of circulating erythrocytes. Splenectomy is invariably followed by increasing haemoglobin level and an average 30% increase of erythrocyte survival (22–48%).

In cases of CLL plus splenomegaly the indications for splenectomy appear to be fairly simple, but may be supported by the demonstration of a large splenic erythrocyte pool and a large splenic erythrocyte destruction.

The studies support the likelihood of lymphocytic infiltration in the liver (increased accumulation of activity), a finding which seems of prognostic significance.
Thymus‐Derived Lymphocytes in Blood, Lymph and Lymphoid Organs after Intrathymic Labelling with 3H‐Thymidine
Authors: Ernström Ulf , Larsson Bengt , Linna Juhani
Published: August 2010

Guinea‐pigs were investigated 2, 4 and 8 days after intrathymic or intraperitoneal labelling with tritiated thymidine. By the comparison between the content of lymphocytes in afferent and efferent thymic blood, an export of heavily labelled lymphocytes from the thymus was demonstrated in the intrathymically labelled animals after 2 and 4 days but not after 8 days. By use of liquid scintillation tritiated thymidine was localized to the spleen in a significantly higher amount 2 days after intrathymic than after intraperitoneal labelling.

By autoradiography heavily labelled lymphocytes derived from the thymus were found in the spleen after 2 days and also to a lesser extent after 4 and 8 days, and in the mesenteric lymph nodes at all times after labelling. In the thoracic duct lymph the frequency of heavily labelled lymphocytes was the same as in the blood after intrathymic labelling, but less than in the blood after intraperitoneal labelling. The result indicates that thymus‐derived lymphocytes are recirculating between blood and lymph.
Iron Absorption and Haemoglobin Regeneration Studies on a New Sustained Release Iron Preparation
Authors: Norrby Anders , Sölvell Lennart
Published: August 2010

The effectiveness of a new sustained release oral iron preparation containing 100 mg of ferrous iron (Duroferon Duretter®) has been evaluated in. three separate studies.

Study I. The absorption of iron was compared to the absorption from rapidly disintegrating ferrous sulphate tablets using a double radioiron technique. Dosage: 200 mg of ferrous iron daily.

Study II. The haemoglobin regeneration rate was compared to the regeneration rate obtained with ordinary ferrous sulphate tablets in a cross‐over study in volunteers twice made anaemic and iron deficient by repeated phlebotomies. Dosage: 200 mg of ferrous iron daily.

Study III. The haemoglobin regeneration rate was studied in a carefully selected homogeneous group of patients with iron deficiency anaemia. Dosage: 300 mg of ferrous iron daily.

The absorption of iron from the sustained release tablets were significantly higher (mean 29 %) than from ordinary ferrous sulphate tablets. In the cross‐over study the haemoglobin regeneration rates obtained with the sustained release preparation were equivalent to that of ordinary ferrous sulphate tablets. All patients with iron deficiency anaemia reached normal haemoglobin values within. 7–10 weeks of treatment. The haemoglobin regeneration rates were the same as those found with optimal doses of oral and parenteral iron.
Factor VIII Inhibitors and the Use of Blood Products in Patients with Haemophilia A
Authors: Ikkala E. , Simonen O.
Published: August 2010

The occurrence of factor VIII inhibitors was investigated in 90 patients with haemophilia A. Inhibitors were found in 11 (12%) patients, all having severe haemophilia A. In 8 of these circulating anticoagulant activity was revealed by a screening method, which ten years earlier had disclosed anticoagulant activity in only one of 82 patients studied.

The amount of blood products transfused to 62 patients with severe haemophilia A in the first study varied from 0 to 7, with a median of 0.9 plasma units per year of age. The values for 69 patients with severe haemophilia A in the present series were: range 0–87 and median 4.5 plasma units per year of age. The increased use of blood products was thus accompanied by an increased occurrence of factor VIII inhibitors. The patients with inhibitors did not differ from other patients with severe haemophilia A with respect to the amount of blood products given.
Decrease in Platelet Surface Charge During Phagocytosis of Polystyrene Latex Particles or Thorium Dioxide
Authors: GRØTTUM K. A. , JØRGENSEN L. , JEREMIC M.
Published: August 2010

Incubation of platelet‐rich citrated plasma with polystyrene particles induced surface contact between platelets and particles, uptake of particles, and changes in platelet morphology. EDTA inhibited both the surface contact between platelets and particles and the uptake of particles. Polystyrene particles and thorium dioxide reduced the electrophoretic mobility of human platelets in vitro. Injected into rabbits both types of particles induced reduction in platelet electrophoretic mobility, platelet aggregation and thrombocytopenia. The reduction in platelet surface charge is suggested to render the platelets more susceptible to aggregation and phagocyosis in the reticuloendothelial system.
Intracellular Porphyrin Concentration and Erythrocyte Life‐Span
Authors: KRAMER S. , SIVE JANE , BECKER D. , VILJOEN D. , METZ J.
Published: August 2010

Red cells from 6 convalescent hospital patients without evidence of porphyria and from 2 patients with erythropoietic porphyria (5 studies) were incubated with delta amino laevulinic acid (ALA) thereby increasing the intracellular porphyrin concentration. These cells were then labelled with 3H DFP or DF32P and re‐injected, and their life‐span compared to a control population of cells labelled with the alternate isotope. Increasing the cellular porphyrin concentration did not alter red cell life‐span. Similarly, increasing the level of porphyrins in rabbit whole blood did not diminish their life‐span, when measured with radiochromium. These data indicate that increased intracellular porphyrin concentration does not prejudice red cell life‐span, and that the haemolytic anaemia in patients with erythropoietic porphyria is not due to the increased intracellular porphyrin concentration.
Structural Identification of Thrombosthenin in Rat Megakaryocytes
Authors: BEHNKE O. , EMMERSEN J.
Published: August 2010

An abundance of filamentous material was seen in the cytoplasm of megakaryocytes after treatment of rat red bone marrow with a glycerol/standard salt solution. The filaments measured 50–120 Å in thickness and were especially abundant in the cells' periphery and in cytoplasmic extensions of thrombocytogenic megakaryocytes, and the filaments were morphologically identical to the contractile proteins (thrombosthenin) seen in platelets. Heavy meromyosin binding was used as a criterion to identify the actinoid filaments.
Cytokinetic Studies in Chronic Myeloid Leukaemia: Evidence for Early Presence of Abnormal Myeloblasts
Authors: BACCARANI MICHELE , KILLMANN SVEN‐AAGE
Published: August 2010

Some cytokinetic parameters (mitotic index, stathmokinetic index after vincristine in vivo, and 3H‐Thymidine flash‐labelling index) of blood and bone marrow cells have been studied in 5 patients with chronic myeloid leukaemia (CML); 3 cases were studied at the time of diagnosis. The labelling index of promyelocytes + myelocytes was very similar to those reported for the corresponding cell types in normal individuals; moreover, the labelling index was identical in marrow and in blood. In contrast, the myeloblasts of CML had a much lower labelling index than normal myeloblasts; also, the labelling index of blood myeloblasts was definitely lower than in marrow myeloblasts. These characteristics of CML myeloblasts are similar to the pattern observed in myeloblasts in acute myeloid leukaemia. As compared to normal granulocytopoiesis, the present study also suggests an overproduction of myeloblasts relative to the production of promyelocytes and myelocytes. From these observations it is suggested that already at the clinical onset of CML, an abnormal subpopulation of myeloblasts has been established.
The Demonstration of a ‘Plasmatic Factor’ in a Case of Coombs' Negative Haemolytic Anaemia
Authors: PARKER A. C. , HABESHAW J. , CLELAND J. F.
Published: August 2010

The clinical history of a patient with acute auto‐immune haemolytic disease with a persistently negative antiglobulin test (Coombs' test) is given. The erythrophagocytosis test (ET) and its use in demonstrating the presence of a ‘plasmatic factor’ is described. That a ‘plasmatic factor’ was responsible for the haemolytic disease was substantiated by showing reduced survival of compatible donor cells in the patient's circulation. The diagnosis of the case as acute auto‐immune haemolytic disease is supported by the exclusion of intracorpuscular and other known extracorpuscular causes of haemolysis, by the good response on two occasions to steroid therapy and finally by the good result of splenectomy.
Electronmicroscopy of Leukaemic Myeloblasts with Numerous Lipid‐Containing Vacuoles
Authors: POLLIACK AARON , TIMBERG RINA
Published: August 2010

Numerous sudanophilic myeloblasts were found in the bone marrow and peripheral blood of a patient with chronic myeloid leukaemia who developed acute blastic transformation.

The majority of blasts contained numerous large lipid‐containing vacuoles in their cytoplasm which stained positively with Sudan Black and were obvious on electronmicroscopy. The rarity of this unusual finding is stressed.
Development of Circulating Inhibitor Directed against Factor VIII in Patients with Haemophilia‐A
Authors: COHN JØRGEN , NIELSEN VAGN GERHARD
Published: August 2010

Of 20 patients suffering from severe haemophilia‐A, 4 were complicated by the development of inhibitor directed against factor VIII. Two of the 4 patients were siblings. Before detection of the inhibitor the patients were treated with cryoprecipitate, AHG (Cohn fraction I‐O) and blood transfusions.
Folic Acid and the Pill
Authors: WOOD J. K. , GOLDSTONE A. H. , ALLAN N. C.
Published: August 2010

Three cases of folic acid deficient megaloblastic anaemia are described in which a combined oral contraceptive has been aetiologically implicated. The first patient proved to have coeliac disease precipitated clinically by administration of the oral contraceptive. Case 2 had a long standing marginally folate deficient diet and became anaemic only after taking the preparation for a year. She also had minimal evidence of malabsorption but folic acid absorption is normal. The third patient, who shows no evidence of malabsorption, also had a chronically poor diet and became anaemic whilst on the oral contraceptive. Following cessation of the preparation this last patient remitted without haematinics.
The Pattern of Erythrocyte Sequestration in Immunohaemolysis: Effects of Prednisone Treatment and Splenectomy
Authors: Christensen B. Egelund
Published: August 2010

The erythrocyte sequestration in the spleen and liver was investigated in 25 cases of immunohaemolysis by surface counting after infusion of 51Cr‐labelled autologous erythrocytes. The erythrocyte sequestration pattern was found to depend upon the treatment with prednisone given at the time of the investigation and furthermore upon the total haemolytic intensity.

Increased sequestration of erythrocytes in the liver is always present in cases of severe hyperhaemolysis. After prednisone treatment, hypersequestration of erythrocytes in the spleen predominates. In the present study increased sequestration in the liver was even demonstrated in about 50 % of the cases in which incomplete non‐complement‐fixing antibodies were found.

In the majority of cases prednisone therapy gave only short‐lasting remission. Frequently, it is difficult to reduce the dose of prednisone because of increasing haemolytic intensity.

Complete, long‐lasting remission seems to follow splenectomy in cases characterized by a splenic sequestration pattern during periods without prednisone therapy, but if hepatic hypersequestration has been demonstrated the remissions are not complete. The need for steroids decreased after splenectomy in practically all cases. A combination of prednisone therapy and early splenectomy is advisable in most cases of immunohaemolysis.
Autoimmune Thrombocytopenia Occurring in a Young Woman with Idiopathic Haemochromatosis
Authors: Habib Michael A. , Burningham Richard A.
Published: August 2010

A 25‐year‐old female underwent evaluation for amenorrhea. The findings of profound hyperferraemia, without obvious cause, hepatic haemosiderosis, and pituitary gonadotropin deficiency led to the diagnosis of idiopathic haemochromatosis. Persistent autoimmune thrombocytopenia was found. Autoimmune thrombocytopenia has not previously been described in this disorder.
Leucopoietic Effect of Calusterone (7 β, 17 α‐Dimethyltestosterone) in Women with Advanced Breast Cancer
Authors: Horn Y. , Halden A. , Gordan G. S.
Published: August 2010

Calusterone, a new anabolic, weakly androgenic testosterone derivative, found highly effective in women with advanced breast cancer, was studied for its peroral effect on white blood cell count (WBC). A significant rise in WBC occurred during treatment of 80 women, as compared both with a contol group of 21 untreated breast cancer patients and a similar group of 27 patients receiving Δ1‐estololactone 1, 000 mg daily. Δ1‐estololactone produced no significant change in WBC. The combination of 5‐fluorouracil 500 mg i.v. once a week plus Δ1‐estololactone 1, 000 mg a day by mouth in 28 patients produced significant mild leucopenia. The effect of androgens on leucopoiesis and the potential value of calusterone in leucopenia, in addition to its efficacy in breast cancer, is discussed.
Circulating Anticoagulant Against Factors XI and XII Together with Massive Spontaneous Platelet Aggregation
Authors: Cronberg S. , Nilsson I. M.
Published: August 2010

In a patient with SLE‐syndrome anticoagulant activity directed against both factor XI and XII was found together with an intense spontaneous aggregation of platelets.
Paroxysmal Nocturnal Haemoglobinuria and Chronic Myeloid Leukaemia in the Same Patient
Authors: Tso S. C. , Chan T. K.
Published: August 2010

A 63‐year‐old Chinese male presented with paroxysmal nocturnal haemoglobinuria (PNH) associated with certain unusual features. A year later the clinical picture became one of chronic myeloid leukaemia (CML) without demonstrable Philadelphia‐chromosome. The PNH defect disappeared with the onset of frank leukaemia. It is postulated that an unknown damage to the haemopoietic stem cell resulted in a myeloproliferative disorder which manifested as PNH and CML at different stages of his illness.
Selective Malabsorption of Vitamin B12 in a Negro Boy
Authors: Buchanan N. , Geefhuysen J. , Cassel R. , Green R.
Published: August 2010

A case of selective vitamin B12 malabsorption is described in an 8‐year‐old Negro boy. The importance of considering vitamin B12 as a possible cause of megaloblastic anaemia amongst malnourished children is stressed.
Effect of Anticonvulsive Drugs on Folate Absorption and the Cerebrospinal Folate Pump
Authors: Reizenstein P. , Lund L.
Published: August 2010

Effects of phenytoin on folate metabolism were studied in 94 subjects. Even healthy volunteers on diphenylhydantoin showed decreasing serum folates, but neurological disease other then epilepsy did not cause folate deficiency. In conjunction with earlier studies, this indicates that folate deficiency in epilepsy is caused by the drugs, rather than by the disease. Diphenylhydantoin as well as carbamazepine (Tegretolr̀) appear to be involved.

No antimetabolite effect of diphenylhydantoin could be demonstrated in a microbiologic system, nor could any correlation be found between the serum concentrations of diphenylhydantoin and folates. Such a correlation would be expected in case of an antimetabolite action. Therefore, intestinal absorption was studied. No effect could be shown in man on the intestinal absorption of medicinal folates. The absorption of food folates, on the other hand, seemed to be inhibited. A cerobrospinal folate pump exists, which keeps the cerebrospinal folate concentration almost five times higher than that in the serum. This ratio was numerically but not significantly lower in the phenytoin‐treated patients.
Normal Red Cell Survival in the Rabbit
Authors: Smith G. N. , Mollison P. L.
Published: August 2010

Red cell survival in the rabbit was estimated using both 51Cr to label cells in vitro and DF32P to label cells in vivo. The results suggest that, after about the first 7 days, there was no elution of DF32P from the red cells and that, accordingly, the rate of 51Cr elution could be obtained by plotting the 51Cr/DF32P ratio from day 7 onwards. If the assumption is made that the rate of Cr elution was the same between days 1 and 7 as it was after day 7, then observed Cr values from day 1 onwards can be corrected for elution and an estimate of true red cell survival obtained. Analysis of the resulting curves indicates that the initial slope is linear on an arithmetical scale for about the first 17 days, becoming curvilinear thereafter. It is concluded that the curvilinear slope of red cell survival in rabbits, hitherto ascribed to random red cell destruction, is more likely to be due to a rather wide scatter of red cell life‐span combined with superimposed elution of the red cell label. In the present series, mean red cell life‐span in the rabbit was estimated to be 50.4 days; as in previous series, the survival of the longest‐lived cells was found to be about 65–70 days.
Controlled Trial of Oral Contraceptives in Haemophilia
Authors: Brandt N. J. , Cohn J. , Hilden M.
Published: August 2010

A controlled trial of an oral contraceptive in the treatment of 18 boys with haemophilia is reported. During the treatment with combined oestrogens and progesterone we found a reduction in bleedings of the skin and joints, associated with a minor shortening of coagulation time in glass and plastic as well as a decrease in kaolin‐cephalin time. Correspondingly, there was a significant increase in fibrinogen and factor II + VII levels. The treatment did not affect the number of days in bed, haematuria, epistaxis or muscular bleeding. ‐ Because of this rather negligible effect and since all patients developed severe side effects (gynaecomastia and pubic hair), we do not recommend this treatment in the general management of haemophilia.
Nucleotide Leakage from Platelets in Artificial Media: Prevention by Albumin and Other Macromolecules and Relation to ADP‐Induced Platelet Aggregation
Authors: Tangen O. , Andrae M.‐L. , Nilsson B. E.
Published: August 2010

Leakage of ATP and ADP from gel filtered rabbit platelets has been investigated. It was found that upon suspending the platelets in a non‐protein medium, accumulation of ADP and ATP externally to the platelets took place. Addition of albumin to the suspending medium resulted in rapid disappearance of extra‐platelet nucleotides. It could also be demonstrated that the leakage of adenine nucleotides from the platelets was due specifically to the change of media and not to mechanical damage of the platelets during the isolation procedure. Several other macromolecules like fibrinogen, β‐ and γ‐globulins, and dextran sulphate had a moderately protective effect on the platelets, but not to the same extent as albumin. The raised extra‐platelet concentrations of ATP and ADP found in non‐protein media was shown to be related to the decrease of the reactivity of the platelets to ADP. Fibrinogen potentiated ADP‐induced aggregation of gel filtered platelets, but only when the platelets were suspended in a medium containing albumin.
Clasmatocytosis in Rat Bone Marrow Lymphocytes
Authors: Ben‐Ishay Zina
Published: August 2010

Detachment of parts of cytoplasm (clasmatocytosis) from lymphocytes was observed in the bone marrow of normal and sublethally irradiated rats. Though the function of the detached parts remains to be established, it is postulated that clasmatocytosis may represent a secretory function of the lymphocyte. The phenomenon is compared to clasmatocytosis in plasma cells thought to represent one way of secretion of immunoglobulins.
Pattern of the Activator Inhibitor in Carcinoma and Renal Diseases
Authors: Hedner U. , Nilsson I. M.
Published: August 2010

The pattern, after gel filtration of serum on Sephadex G‐200, of the inhibitors of the urokinase activation of plasminogen was studied in 20 patients with carcinoma, 12 with chronic renal insufficiency and 10 with acute renal insufficiency. The urokinase inhibitors were determined with the clot lysis method of Paraskevas et al (1962) and in the patients with carcinoma and those with chronic uraemia also with the caseinolytic method of Lauritsen (1968).

The amount of inhibitors obtained in the high molecular weight fraction after gel filtration of sera from patients with carcinoma did not differ from normal, while that obtained in the low melecular fraction was significantly raised when assayed with both methods. After gel filtratinon of sera from the patients with chronic or acute renal failure the amount of inhibitors in each fraction was significantly increased when determined with the clot lysis method. But in the patiens with chronic renal failure no increase was found when measured with the caseinolytic method. In these patients the inhibitory effect on the activation of plasminogen is at least partly unspecific owing to accumulation of amino acids in the plasma in uraemia.
The Influence of Technical Factors on the NBT Test
Authors: Björksténm Bengt
Published: August 2010

The results of NBT tests were found to vary depending on how the tests were performed. Heparin increased the proportion of NBT positive neutrophils and the lowest possible concentration should be used. Blood samples for the NBT test could be stored for 8 h at 4° C with only a minor effect on the test results. The incubation time was crucial and 25 min in a 37° C water bath was adopted as standard procedure.
Ultrastructural Features of the Granulocytes in Down's Syndrome
Authors: Djaldetti M. , Bessler H. , Fishman P. , van der Lÿn E. , Joshua H.
Published: August 2010

The granulocytes of 9 patients with Down's syndrome (DS) were examined for morphological abnormalities. Electron microscope examination showed abnormalities in the nucleus, such as pockets, bridges, appendices and fibrillar bodies in 25–57 % of the cells as compared to similar, but less conspicuous findings in 5–10 % of the granulocytes of 5 controls. The similarity of these ultrastructural malformations to those described in different forms of leukaemia is stressed. Since chromosomal abnormalities exist in both DS and leukaemia, the possibility that they are the cause for the ultrastructural alterations is discussed.
Serum Immunoglobulin Levels in Chronic Lymphatic Leukaemia
Authors: Slungaard Arne , Smith Martin J.
Published: August 2010

Serum immunoglobulin levels were measured in 44 patients with chronic lymphatic leukaemia and in a control group of comparable age, race, and sex. Application of two‐sided rank sum tests revealed highly significant reductions of IgG, IgA and IgM in male patients and of IgA in female patients. Slight but insignificant decreases in IgG and IgM were found in female patients. Therefore, these reductions in serum immunoglobulin levels in chronic lymphatic leukaemia are probably attributable to the disease state per se and not to the known variables of age, race, and sex. The potential usefulness of multivariate linear discriminant functions derived from the immunoglobulin levels of the control and patient populations is discussed.
Myeloperoxidase‐Mediated Extracellular Iodination during Phagocytosis in Granulocytes
Authors: Odeberg H. , Olofsson T. , Olsson I.
Published: August 2010

Myeloperoxidase mediated iodination was studied during phagocytosis of killed yeast cells and immune complexes and it was found that iodinated plasma proteins appeared in the extracellular fluid. The incubation medium obtained after removal of granulocytes and particles also catalyzed the iodination of plasma proteins when a hydrogen peroxide generating system consisting of glucose oxidase was included. The extracellular iodination seems to be due to the action of myeloperoxidase released during phagocytosis. Large particles such as killed yeast cells induced a larger release of iodinating capacity than the smaller E. coli.
Platelet Responses to Ristocetin in Von Willebrand's Disease
Authors: Sanderson J. H. , Burn Aileen M. , Cooke Sheila
Published: August 2010

Studies of the aggregations of platelets in response to ristocetin have been carried out in normal subjects and in 21 patients with von Willebrand's disease. These demonstrate a wide range of patterns of response to ristocetin in von Willebrand's disease, the responses depending on a plasma factor labile at room temperature. This factor is probably identical to ‘von Willebrand factor’ or ‘factor VIII‐related antigen’ and the level of this factor appears to be specific to each patient.
Study on the in vitro Rieder Cell
Authors: Ito Suiko
Published: August 2010

Light and electron microscopic studies were performed on the in vitro Rieder cells from patients with chronic lymphocytic leukaemia (CLL) and acute prornyelocytic leukaemia (APL).

Light microscopically, it was found that Rieder cells were increased at essentially the same rate as in the EDTA and sodium citrate treated blood, when the control samples (coagulated blood and defibrinated blood) were simply left at room temperature as well as at 37° C. Vincristine had a suppressive effect on the Rieder cell formation in CLL but did not reduce the number of APL‐cells that had lobulated and separated nuclei.

Electron microscopically, a bundle of many microtubules was found to surround the nuclear constriction of the in vitro Rieder cells in CLL, whereas the corresponding bundle consisted of only microfilaments in the in vivo Rieder cells of APL. Vincristine induced disappearance of the microtubules of Rieder cells and formation of many crystals in the cytoplasm of leukaemic cells of CLL and APL but did not affect the microfilaments in the cells of APL.