bluuebunny/crossref_metadata_2025 huggingface dataset:
Characterization of the Cobalamins Attached to Transcobalamin I and Transcobalamin II in Human Plasma
Authors: Nexø Ebba
Published: August 2010

Insolubilized antibody to transcobalamin I was used to separate transcobalamin I and transcobalamin II. By bioautography of the extracted cobalamins it was shown that transcobalamin II bound more deoxyadenosylcobalamin than did transcobalamin I, and that methylcobalamin accounts for most of the cobalamins attached to transcobalamin I. This finding may indicate that transcobalamin I has a function in the metabolism of methylcobalamin in man.
Immunologic Profile of Patients with Cured Hodgkin's Disease
Authors: Björkholm M. , Holm G. , Mellstedt H.
Published: August 2010

Blood lymphocytes from 9 patients with Hodgkin's disease (HD) were studied. The results were compared with those of 6 seminoma testis patients and 9 healthy unrelated controls. All patients were in complete and unmaintained remission more than 10 years after termination of radiotherapy. The mean T‐lymphocyte count of HD patients was lower than that of controls and seminoma testis patients. Lymphocyte DNA synthesis induced by pokeweed mitogen and phytohaemagglutinin was normal in both patient groups. Concanavalin A‐induced DNA synthesis was low in 4 patients with HD although the mean stimulation of the group did not differ from controls or seminoma testis patients. Lymphocyte activation by PPD was slightly decreased in the 2 patient groups. No increase in spontaneous lymphocyte DNA synthesis was observed. The responding and stimulatory capacity in mixed lymphocyte culture was decreased in 3 and 2 HD patients respectively. 4 out of the 9 patients with HD but none with seminoma testis displayed severe impairment in T‐lymphocyte functions. As 1 of the 4 had been treated solely by surgery, late effects of irradiation can only partly explain the results. The results may favour a hypothesis postulating a constitutional defect contributing to the immunoincompetence in HD.
Discrimination of B, T and Null Lymphocytes by Esterase Cytochemistry
Authors: Higgy K. E. , Burns G. F. , Hayhoe F. G. J.
Published: August 2010

A technique has been devised which optimally demonstrates non‐specific esterase activity in human blood lymphocytes. The reaction is carried out on smears fixed with formalin vapour, using α‐naphthol butyrate as substrate at pH 8 and at a low concentration for a short incubation period. The pattern of esterase activity revealed by this method provides a discriminating marker for mature T‐lymphocytes, which show dense, localised, dot‐like positivity, and a probable marker for ‘null’ cells in the form of scattered granular positivity. B cells appear to be negative. Monocytes show a clearly different pattern of granular positivity.
A Case of T‐cell Lymphoma with Sézary Cells in the Blood and Bone Marrow Accompanied by Peripheral T and B Lymphocytosis
Authors: Manthorpe Rolf , Jønsson Viggo , Christensen Bjarne Egelund , Hesselvik Margaretha , Egeberg Jørn , Videbæk Aage
Published: August 2010

A case of T‐cell lymphoma occurred in a man, aged 66, whose symptoms started as itching and the appearance of 1–2 cm indurated plaques with central pustules on the skin of the trunk and face. A few months later generalized lymphomas and splenomegaly were present. Investigations of the lymphocytic subpopulations in situ in fresh frozen sections of lymphomas and skin showed only T cells. Samples of blood and bone marrow disclosed lymphocytosis and Sézary cells. Both T and B lymphocytes were definitely increased in the blood. The value of tissue studies in analyzing lymphocytic subpopulations is emphasized as a differential diagnostic aid in classifying lymphomas. A relationship between the T‐cell neoplasia and the T and B lymphocytosis to mycosis fungoides and the Sézary syndrome is discussed.
Obesity, Plasma Lipids and Polymorphonuclear (PMN) Granulocyte Functions
Authors: Palmblad Jan , Hallberg Dag , Rössner Stephan
Published: August 2010

20 obese subjects were compared with 20 controls with normal weight regarding their polymorphonuclear (PMN) granulocyte functions, and plasma lipids. The obese subjects showed a significantly decreased PMN bactericidal capacity, and increased PMN adherence. No differences were found in their mean PMN chemotaxis and opsonic capacity of plasma. The values of plasma triglycerides and free fatty acids were higher in the obese, while plasma cholesterol and phospholipids corresponded to the control values. The changes in granulocyte function did not correlate significantly to plasma lipid levels or to body weight and Broca's index in either group. ‐ It is concluded that changes in granulocyte function occur in obesity, but are not related to plasma lipids or degree of overweight.
Microcalorimetric Measurements of Heat Production in Erythrocytes of Patients with Liver Diseases
Authors: Monti M.
Published: August 2010

Erythrocyte metabolic activity has been investigated in 12 patients with severe liver disease. The erythrocyte resting heat effect value has been found to be 25 % higher than normal (P < 0.005). A normal value was obtained when heat effect was measured under maximal stimulation with 10−4M methylene blue (MB). Glucose consumption was found to be normal, both in unstimulated and methylene blue stimulated erythrocytes. The present calorimetric results do not give support to the hypothesis that haemolytic anaemia in patients with liver disease is related to decreased activity of the pentose phosphate pathway.
Unique Eosinophil Granules in a Case of T‐Cell Lymphoma
Authors: Manthorpe R. , Egeberg J. , Hesselvik M. , Videbæk Aa.
Published: August 2010

A 41‐year‐old man developed intense itching without visible cutaneous changes, epigastric pressure pain, and a slight intolerance to alcohol. He was found to have persistent blood eosinophilia.

The eosinophil granulocytes were of abnormal appearance in the light microscope: larger than normal, the nuclei were multilobulated (4–6 lobes), the cytoplasm contained atypical, large granules, ample glycogen, and up to 12 vacuoles.

In the electron microscope too the eosinophil granules were entirely atypical, having an electron‐dense matrix, often with a light central inclusion body which was inhomogeneous, having longitudinally oriented structures with a periodicity of about 10 nm. These findings are quite contrary to normal eosinophil granules.

Enzymic studies of cytoplasmic enzymes from the granulocytes revealed a greatly reduced content of eosinophil cationic proteins, whereas 5 (7) other enzymes were present in a normal or slightly reduced quantity. The phagocytic capacity of the eosinophils against latex particles was normal.

The patient developed generalized lymphomas, histologically very malignant, of the convoluted, acid phosphatase positive cell type (T‐cell lymphoma). Sub‐population studies of lymphocytes from a lymph node revealed 58 % TEcells, while the remainder were B cells. At death, 31/2 years after the onset of symptoms, severe endomyocardial fibrosis was found. The thymus could not be identified.

It is concluded that lymphomas should be described on the basis of clinical, histological, and histochemical criteria as well as studies of lymphocyte sub‐populations and that the highly unusual eosinophil granulocytes still deserve particular attention. The endocardial fibrosis is assumed to have been due to substances liberated from the eosinophil cells.
The Extracellular Release of Granulocyte Collagenase and Elastase during Phagocytosis and Inflammatory Processes
Authors: Ohlsson Kjell , Olsson Inge
Published: August 2010

Human granulocytes release 25–30 % of the granular neutral proteases, collagenase and elastase, to the exterior of the cell during phagocytosis of yeast cells or immune complexes. Similar amounts of myeloperoxidase and lactoferrin are released. Crossed immunoelectrophoresis demonstrated that collagenase and elastase released extracellularly formed complexes with serum α1‐antitrypsin. The presence of α1‐antitrypsin complexes with granulocyte collagenase and elastase were also demonstrated in inflammatory processes, e.g. in the peritoneal exudate of acute peritonitis. The reactivity of neutrophil proteases with natural plasma protease inhibitors must be considered in assessing the role of these proteases as the etiologic agent of tissue damage and degradation during the inflammatory process.
Serum Erythropoietin in Myelomatosis
Authors: Hansen Ole Paaske , Thorling Eivind B. , Drivsholm Aage
Published: August 2010

Erythropoietin activity in serum was measured using 59Fe incorporation into erythrocytes in protein‐starved, hypoxic mice.

The activity in serum from 20 patients with untreated myelomatosis was not significantly different from that in 31 saline controls. Only three patients had detectable erythropoietin levels in serum: 0.24 IU/ml, 0.27 IU/ml and 0.50 IU/ml (standard B), respectively.

The venous haematocrit was correlated positively with the glomerular filtration rate as measured by 51Cr EDTA‐clearance. No correlation could be established between venous haematocrit and serum albumin or serum transferrin.

The results are in agreement with the assumption of a defective erythropoietin activity due to renal failure in myelomatosis.
Chronic Lymphocytic Leukaemia with Subsequent Development of Multiple Myeloma
Authors: Pedersen‐Bjergaard Jens , Petersen Hans Draminsky , Thomsen Mogens , Wiik Allan , Wolff‐Jensen Jacob
Published: August 2010

In a patient with chronic lymphocytic leukaemia, two M components of the IgGk and IgGλ classes were demonstrated in the serum at the time of diagnosis. The patient was first followed without treatment; after 18 months multiple myeloma developed. At that time, immunofluorescence study of lymphocytes of the peripheral blood showed mainly membrane‐bound immunoglobulins (S‐Ig) of the IgGk class. The bone marrow disclosed a definite predominance of plasma cells with cytoplasmic IgGλ, suggesting that the two B cell‐derived diseases had arisen from two different cell clones.

During the development of multiple myeloma, the serum concentration of the M component IgGλ increased. Concurrently, the M component of IgGk gradually disappeared from the serum, and the concentrations of the normal immunoglobulins IgA and IgM declined. Following cytostatic treatment, the concentration of the myeloma‐derived M component IgGλ was halved and simultaneously the M component IgGk reappeared in the serum.

To our knowledge, this case is the second reported of chronic lymphocytic leukaemia and multiple myeloma indicating development of the two diseases from different cell clones, and the first reported case with myeloma‐induced suppression of M‐com‐ponent secretion from malignant cells.
Erythrocyte Abnormalities Induced by Chemotherapy and Radiotherapy: Induction of Preleukaemic States?
Authors: Renoux Michel , Bernard Jean‐François , Torres Martine , Schlegel Nicole , Amar Michèle , Lopez Manuel , Boivin Pierre
Published: August 2010

Increased incidence of acute leukaemia (AL) following exposure to ionizing radiation is well documented. AL has also been reported with increased frequency after chemotherapy for nonmalignant diseases. The effect of chemotherapy and/or radiotherapy on the following erythrocyte parameters: pyruvate‐kinase (PK) activity, phospho‐fructo‐kinase (PFK) activity, HbF level, red cell antigens production and bone marrow sideroblasts, has been studied in 31 patients with myelomatosis and in 33 patients with nonhaematological malignancies.

We have demonstrated the appearance after chemotherapy or radiotherapy of some abnormalities usually associated with leukaemia or preleukaemic states. In patients treated for myelomatosis, a PK deficiency has been detected in 58 % of the cases, an increased HbF level in 47 %, a modification of blood groups antigens in 68 % and abnormal sideroblasts in 30 %. No PFK deficiency was found. In patients treated for solid tumors, a PK deficiency in 50 % of the cases, a PFK deficiency in 20 %, an increased HbF level in 10 %, a modification of blood group antigens in 40% has been detected. Chemotherapy administered over a period of 3 months has been shown to induce these abnormalities. Localized radiotherapy may have a similar effect. The combination of these two factors seems to cause an increase in the frequency and intensity of these abnormalities.
Biochemical Characteristics of Chronic Lymphocytic Leukaemia
Authors: Simonsson Bengt , Nilsson Kenneth , Terenius Lars , Glimelius Bengt
Published: August 2010

Chronic lymphocytic leukaemia (CLL) has a variable clinical course and there is a great need of new prognostic laboratory parameters in this disease. 24 CLL patients were subjected to routine haematological and clinical investigation. The leukaemic cells were analyzed for surface immunoglobulins, complement and sheep red blood cell receptors, Concanavalin A‐induced agglutination, cytoplasmic glucocorticoid receptor and for proliferative activity by measurement of tritiated thymidine incorporation.

The surface markers studied indicated that all cases were of B‐cell origin. Only 5 of 23 patients studied had normal serum immunoglobulin levels. These cases showed a nonprogressive disease. 8 patients had increased infection tendency, all of whom had subnormal IgG levels; 4 of them also had subnormal IgA and IgM and 2 had subnormal IgA levels. 5 out of 6 patients with progressive disease and 3 of 11 with nonprogressive disease had an increased proliferative index, indicating a correlation between this parameter and disease progression. ConA agglutinability was not correlated to disease activity. Cells from 17 of 22 patients showed measurable amounts of glucocorticoid receptor. The 5 patients lacking this receptor had inactive disease.
Fatal Aplastic Anaemia Complicating Infectious Mononucleosis
Authors: van Doornik M. C. , Veer‐Korthof E. T. van T , Wierenga H.
Published: August 2010

A 7‐year‐old boy developed fatal aplastic anaemia, 2 weeks after the first signs of infectious mononucleosis. Although some drugs were administered, infection with the Epstein‐Barr virus, leading to infectious mononucleosis, is assumed to be the cause of this aplastic anaemia. Some mechanisms possibly leading to the aplasia are discussed.
A New Variant of Glucosephosphate Isomerase Deficiency with Mild Haemolytic Anaemia (GPI‐MYTHO)
Authors: Galand C. , Torres M. , Boivin P. , Bourgeaud J. P.
Published: August 2010

A new case of glucosephosphate isomerase deficiency with mild haemolytic anaemia was observed in a 6‐year‐old girl. Deficient enzyme was characterized by a profoundly decreased activity in the red cells, a normal electrophoretic phenotype, normal isoelectric point, normal optimum pH, a molecular instability and a clearly decreased Michaelis constant for fructose‐6‐phosphate. Propositus was double heterozygote for a ‘silent gene’ inherited from the mother and an abnormal enzyme from the father. Because this abnormal enzyme has undescribed characteristics, it responds to a new variant for which we propose the name GPI‐MYTHO.
Sustained Haematological Remission after Bone Marrow Curettage in a Case of Primary Myelofibrosis
Authors: Matzner Yaacov , Polliack Aaron , Shenar Eilat , Izak Gabriel
Published: August 2010

This brief case report describes a sustained haematological remission obtained in a patient with primary myelofibrosis treated by bone marrow curettage after previous therapy with androgens was unsuccessful. The patient remains in haematological remission 5 years after the procedure. It is suggested that this procedure be attempted in more cases of myelofibrosis.
Transcobalamin I and other Human R‐Binders: Purification, Structural, Spectral and Physiological Studies
Authors: Nexø Ebba
Published: August 2010

Transcobalamin I (TCI), a human R‐binder of unknown function, has been purified in the native state by ‘labile ligand affinity chromatography’. By this method protein denaturing agents are avoided, because desorption takes place by dissociation of the insolubilized ligand (cobalamin) simply by a shift in temp.

The amino terminal sequence of TCI is identical to those of other R‐binders, and the amino acid composition varies only slightly. A common phylogenetic origin for the R‐binders and intrinsic factor is suggested by the similarity in amino acid composition.

Spectral analyses of aquo‐, hydroxo‐, azido‐ and cyanocobalamin, free or attached to TC I suggest that the sixth ligand is donated to the cobalamin from the protein. The observed changes in the spectra are in keeping with the hypothesis that TC I and other R‐binders are enzymes.

Turnover studies with iodine‐labelled TC I saturated with cyanocobalamin revealed that it is metabolized with a fractional catabolic rate of 0.15 d‐1, and that the cobalamin is liberated only on degradation of the protein. From these data the amount of cobalamin transported by TC I can be calculated to be approximately 0.1 nmol per day, thus TC I does not play any significant role in the daily transport of cobalamin.
Traffic of T and B Lymphocytes in the Normal Spleen
Authors: Christensen Bjarne Egelund , Jønsson Viggo , Matre Roald , Tønder Olav
Published: August 2010

The occurrence of C3b and C3d complement receptors and of Fc and E receptors in normal splenic tissue was studied in cryostat sections incubated with specific marker cells. Thereby the topographic distribution of T and B lymphocytes in the splenic tissue was mapped: TE lymphocytes are found especially in the periarteriolar sheaths, B lymphocytes in eccentric zones around the arterioles. The cells in the splenic pulp carry especially Fc, but also C3b receptors, whereas C3d receptors appear to be specific of lymphocytes. The basic lymphocyte traffic behind the mapped distribution of the lymphocyte subpopulations in the spleen was elucidated by lymphocyte kinetic studies using autologous 51Cr‐labelled reinfused lymphocytes. These studies were performed partly on normal persons and partly on splenectomized persons without immune defects. The results indicate the presence of an exchangeable pool of T as well as of B lymphocytes in the spleen, the T cell pool being larger and making up in normals about 30 % of the total exchangeable T lymphocyte mass. The matrix for the T lymphocyte pool in the spleen is the periarteriolar sheaths. The difference in structure between the lymph nodes and the lymphoid tissue of the spleen, including differences in the distribution of lymphocyte subpopulations, are explained by the special vascular arrangement in the spleen.
Prognostic Value of in Vitro Bone Marrow Culture in Refractory Anaemia with Excess of Myeloblasts
Authors: Faille A. , Dresch C. , Poirier O. , Balitrand N. , Najean Y.
Published: August 2010

Bone marrow from 17 patients with refractory anaemia with excess of myeloblasts (RAEM) was cultured in methylcellulose semi‐solid medium.

Compared with normal bone marrow, 3 patterns of growth occurred corresponding with different clinical stages of the condition. Patients whose bone marrow grew normal colonies and those who produced a predominance of microclusters had the longest life expectancy, while those who produced a predominance of macroclusters had the shortest life expectancy with a high rate of acute leukaemic transformation. Colony culture appears to be a useful prognostic tool in this condition.
Subcutaneous Cerebrospinal Fluid Reservoirs in Patients with Acute Leukaemia
Authors: Spiers A. S. D. , Booth A. E. , Firth J. L.
Published: August 2010

Subcutaneous cerebrospinal fluid (Ommaya) reservoirs were inserted in 23 patients with acute leukaemia and 2 patients with malignant lymphoma. In 9 leukaemia patients reservoirs were used for the prophylaxis of central nervous system leukaemia: only 1 patient developed meningeal leukaemia but less invasive therapy has now been shown to achieve effective prophylaxis and prophylactic use of Ommaya reservoirs has been discontinued. In 16 patients, reservoirs were inserted as aids to the treatment of meningeal leukaemia or lymphoma. Intracranial disease was successfully controlled and recurrences were prevented but patients eventually died from relapse of their systemic disease. The principal complications of the subcutaneous reservoirs were obstruction, misplacement, and one case of infection. Leucoencephalo‐pathy in several patients was attributable to irradiation and cytotoxic drugs and not to the reservoirs. Problems due to misplacement of the reservoir occurred in 3 out of 13 reservoirs inserted by the occipital route and in only 1 of 12 reservoirs inserted by a frontal approach. With adequate supervision, the reservoirs are valuable aids to intrathecal therapy in patients with established meningeal leukaemia or lymphoma.
Interrelationships between Blood Volume, Venous Haematocrit and Renal Failure in Myelomatosis
Authors: Hansen Ole Paaske , Drivsholm Aage
Published: August 2010

Plasma volume (PV), venous haematocrit (PCV) and total blood volume (BV) were measured in 24 patients with untreated myelomatosis using Evans Blue dye technique and PCV corrected for ‘trapped’ plasma and body/venous haematocrit ratio. The state of renal insufficiency was expressed as a serum creatinine and a glomerular filtration rate using 51CrEDTA clearance.

The total BV was normal in all but 3 patients, whereas the PCV was subnormal with an average value of 0.267. Consequently, the PV was expanded with an average value of 25 % compared to normal values. The PV was positively corrected to the concentration of gammaglobulin in serum. The 3 patients with increased BV caused by an increased PV had subnormal red blood cell mass and thus a partial haemo‐dilution anaemia only.

A highly significant correlation was found between renal clearance and PCV and between renal clearance and the indirectly measured red blood cell mass, respectively (P < 0.001).

The data demonstrate that renal failure in myelomatosis is associated by an increase in PV and a decrease in PCV without any change in total BV. No correlation was found between the degree of renal insufficiency and body/venous haematocrit ratio. Thus, the major determining factor in prediction of PCV and red blood cell mass was the glomerular filtration rate. The results are in agreement with the assumption of a nephrogenic pathogenesis in anaemia of myelomatosis.
Severe Aplastic Anaemia with Complete Autologous Marrow Reconstitution Following Treatment with Antithymocyte Globulin
Authors: Pedersen‐Bjergaard Jens , Ernst Peter , Nissen Nis I.
Published: August 2010

In a patient with end‐stage acquired aplastic anaemia, unsuccessful treatment with splenectomy, prednisone, pyridoxine and anabolic steroid was followed by administration of antithymocyte globulin (ATG). This therapy soon led to a gradual improvement and after 12 months, complete remission was obtained. The possible mechanism of action of ATG and the general principles of treatment are discussed.
Type I Congenital Dyserythropoietic Anaemia with Myelopoietic Abnormalities and Hand Malformations
Authors: Holmberg L. , Jansson L. , Rausing A. , Henriksson P.
Published: August 2010

Type I dyserythropoietic anaemia was diagnosed in an infant, who presented with respiratory distress and hepatosplenomegaly soon after birth. Anaemia became manifest during the neonatal period. The case clearly proves the congenital nature of the disease. Abnormalities of the myelopoietic series indicate that it might be a stem cell disease and the presence of skeletal anomalies of the hands suggests a genetic relationship to some cases of Fanconi and Diamond anaemia. No serum lipid or vitamin E deficiency was present as in type II congenital dyserythropoietic anaemia. Serial serum ferritin determinations incidated that iron stores are increased early in type I congenital dyserythropoietic anaemia despite no transfusion load.
Haemoglobin Concentration and Peripheral Blood Cell Counts in Women The Population Study of Women in Goteborg 1968–1969
Authors: Tibblin Elisabeth , Bengtsson Calle , Hallberg Leif , Lennartsson Jan
Published: August 2010

Hb concentration, PCV, erythrocyte and leucocyte counts in the peripheral blood, MCV, MCH and MCHC in a population sample of 1462 women in ages between 38 and 60 years are presented. In addition, a description is given of thrombocyte counts in 38‐year‐old women. Significant differences with age were found for Hb concentration and PCV and for leucocyte counts. High correlations were found between Hb and PCV in the various age groups studied, while lower correlations were found between erythrocyte counts on one hand and Hb or PCV on the other. There was an overrepresentation of smokers among women with high Hb values and among those with high leucocyte counts. The higher leucocyte counts in the youngest age groups could be explained by the larger number of smokers in these ages. Thrombocyte counts were lower in smoking than in non‐smoking women.
Beneficial Effect of Granulocyte Transfusions in Patients with Defects in Granulocyte Function and Severe Infections
Authors: Pflieger H. , Arnold R. , Bhaduri S. , Bultmann B. , Goldmann S. F. , Haghou F. , Kubanek B. , Kurrle E. , Niethammer D.
Published: August 2010

A 3‐year‐old boy (patient A) with a congenital and a 24‐year‐old man (patient B) with an acquired granulocyte function defect received supportive granulocyte transfusions for the management of severe infections. The boy had suffered from recurrent infections since birth. His granulocytes showed in vitro almost no chemotactic responsiveness, an impaired phagocytosis and reduced intracellular killing of Candida albicans. Family studies suggested that it was an inherited autosomal recessive defect. The child developed a Pseudomonas pneumonia at the age of 3 years, which did not respond to antibiotic therapy. Granulocyte transfusions were then started and soon after the fever and pneumonia disappeared.

Patient B showed the haematological signs of a preleukaemic state. He had 3 recurrent episodes of furunculosis which led each time to cellulitis and septic temperatures accompanied by symptoms of an enterocolitis. Tests of granulocyte function in vitro showed reduced intracellular killing of Staphylococcus aureus. Granulocyte transfusions were started, since no clinical improvement could be attained by antibiotics. With transfusion therapy, fever, cellulitis and enterocolitis disappeared each time.
Factor VIII Concentrate Prepared from DDAVP Stimulated Blood Donor Plasma
Authors: Nilsson I. M. , Walter H. , Mikaelsson M. , Vilhardt H.
Published: August 2010

Human fraction 1‐0 (AHF‐Kabi) was prepared from plasma from blood donors who had received an i.v. injection of DDAVP (0.2 μg per kg b.w.) and tranexamic acid (0.01 g per kg b.w.) 15 min before collection of the blood.

The factor VIII preparation from such plasma contained twice as much VIII:C, VIIIR:Ag, and VIIIR:RFC as normal fraction 1‐0.

Normal fraction 1‐0 and DDAVP fraction 1‐0 were given to 2 patients with severe haemophilia A. The in vivo response of the DDAVP fraction 1‐0 corresponded to the in vitro values. No differences in survival time were seen.

Hence, it is possible to produce factor VIII concentrates with at least double the yield by increasing the factor VIII level in blood donors by i.v. injection of DDAVP.
‘Available Oxygen’, a Realistic Expression of the Ability of the Blood to Supply Oxygen to Tissues
Authors: Jones J. G. , Holland B. M. , Veale K. E. A. , Wardrop C. A. J.
Published: August 2010

An expression of clinical applicability is sought for quantitating the availability of oxygen to the tissues from the blood. An ‘available oxygen’ is defined as the arteriovenous oxygen content difference when the mixed venous oxygen tension is assumed to be 20 mmHg. The acceptance of this latter assumption is argued on mathematical and functional grounds.
Nonsecretory IgD‐Kappa Multiple Myeloma in a Patient with Gaucher's Disease
Authors: Benjamin D. , Joshua H. , Djaldetti M. , Hazaz B. , Pinkhas J.
Published: August 2010

A patient with Gaucher's disease and non‐secretory multiple myeloma is described. The presence of typical Gaucher and myeloma cells was confirmed by transmission and scanning electron microscopy. The immunological studies established the diagnosis of ‘non‐secretory’ type of multiple myeloma.
Cell Proliferation and DNA Dependent DNA Polymerase Estimation in Acute Lymphoblastic Leukaemia during Treatment with Prednisone and Vincristine
Authors: Wantzin Gunhild Lange
Published: August 2010

The presence of DNA polymerase and primer‐template DNA in lymphoblast nuclei by measuring the in vitro incorporation of3H‐thymidine‐5′‐triphosphate (3H‐TTP) was studied in 10 patients with acute lymphoblastic leukaemia. Protein synthesis and various other cytokinetic parameters were also studied. After prednisone (P) administration a marked decrease in3H‐TTP labelling index (3H‐TTP LI) was apparent together with an inhibition of3H‐leucine incorporation (3H‐LEU LI) into lymphoblasts. A moderate decrease in3H‐TDR labelling index (3H‐TDR LI) and a later decrease in mitotic index (MI) were seen. Single cell DNA measurements showed a depletion of3H‐TDR labelled lymphoblasts in early part of S‐phase apparent at 24 h lasting up to 54 h after P administration.

Vincristine given as a flash injection later in the study period caused an immediate rise of the MI, at the same time the P induced decline in3H‐TTP LI,3H‐TDR LI and3H‐LEU LI were continued in most patients.

P is thought to damage the cells both in and outside the cell cycle. In the cell cycle the effect of P is an arresting effect in G1.
B‐Prolymphocytic Leukaemia ‐ A Mantle Zone Lymphoma?
Authors: Pallesen G. , Madsen M. , Pedersen B. B.
Published: August 2010

A case of prolymphocytic lymphoma/leukaemia (PL) sensu Galton in a 32‐year‐old man is presented. The leucocyte count was 19.0 times 109/1 at presentation and tartrate resistent acid phosphatase was present in most prolymphocytes. Immunological investigation of prolymphocytes from lymph nodes, spleen and peripheral blood revealed the surface marker phenotype: Smlg+ (μ, (δ), λ), IgG‐Fc‐receptor +, C3‐receptor +. The prolymphocytes from lymph nodes and spleen were C3‐receptor+ in a high percentage, while only a few were IgG‐Fc‐receptor+. This proportion was reversed in the blood prolymphocytes. The histology of lymph nodes was unique and strongly suggested a preferential involvement (homing phenomenon) of the mantle zone of the lymphatic follicle. These results may indicate that emission of prolymphocytes from lymph nodes to circulation involves a change of surface receptors. It is finally suggested to consider the diagnosis of not only hairy‐cell leukaemia but also PL in the case of tartrate resistent acid phosphatase‐positive lymphoma/ leukaemia.
Biochemical Studies in Hermansky‐Pudlak Syndrome
Authors: Gerritsen S. M. , Akkerman J. W. N. , Staal G. , Roelofsen B. , Koster J. F. , Sixma J. J.
Published: August 2010

In order to find the basic defect in the Hermansky‐Pudlak Syndrome (HPS), biochemical studies of platelets and leucocytes were undertaken.

Glutathione levels of platelets were normal and regeneration of GSH similar to controls occurred after incubation with diamide (a specific agent for GSH oxidation). Phospholipid and fatty acid composition of HPS platelets was normal. The amount of peroxides found in platelet membranes was not elevated. A subnormal aggregation with arachidonic acid could be obtained in PRP using a high concentration of arachidonic acid (2 mM), but normal malondialdehyde levels were measured, suggesting a normal prostaglandin synthesis in HPS platelets.

Glutathion peroxidase and p‐phenylenediamide‐mediated peroxidase (PPD‐peroxidase) were normal in leucocytes of 1 HPS patient. Lysosomal enzymes as far as investigated were normal.
Evaluation of the Activated Whole Blood Clotting Time (ACT) in Vitro
Authors: Stenbjerg S. , Berg E. , Albrechtsen O. K.
Published: August 2010

The activated whole blood clotting time (Am was analyzed in vitro using blood samples from normal individuals and from patients with heart disease, scheduled for open heart surgery. The ACT was found to be normally distributed in a group of normal individuals with a mean d 135 sec. In patients with heart diseases, the average ACT was slightly shorter than in normal individuals. A linear relationship between ACT and heparin concentrations was established. Haemodilution and depletion of platelets did not significantly influence the ACT. When a clean venipuncture with disposable needles is used, ACT is a reliable, uncomplicated bedside technique by which heparinisation can be monitored.
Beta Thromboglobulin and Glycosylated Haemoglobin in Diabetes Mellitus
Authors: Matthews J. H. , O'Connor J. F. , Hearnshaw J. R. , Wood J. K.
Published: August 2010

No correlation has been demonstrated between the elevated levels of plasma beta‐thromboglobulin and glycosylated haemoglobin found in a group of 97 diabetic patients. The abnormality in diabetic platelets which is reflected by beta‐thromboglobulin levels may not therefore be a direct result of poor metabolic control. The highest levels of beta‐thromboglobulin were found in those patients with a raised blood urea. Glycosylated haemoglobin levels correlated with prevailing blood glucose and with the type of therapy employed, and were highest in the insulin dependent diabetics, intermediate in those on tablets and lowest in those on diet alone.
Hereditary α2‐Macroglobulin Deficiency
Authors: Bergqvist David , Nilsson Inga Marie
Published: August 2010

On screening of a normal material 1 man was found (age 37) who on repeated determinations had a low α2M, namely 25% with electroimmuno assay according to Laurell. Investigation of the family revealed that the mother (age 69) and one daughter (age 5) had low values too. All other coagulation and fibrinolytic components were normal. They had no signs of increased fibrinolysis and normal levels of α2‐antiplasmin, α1‐antitrypsin and antithrombin III. Liver function tests were normal. It seems to be an inherited deficiency. The transmission is apparently autosomal dominant and the affected members heterozygotes. The defect has not caused any clinical symptoms. This family appears to be the first reported with an α2M deficiency.
Acute Renal Insufficiency Occurring during Intravenous Desferrioxamine Therapy
Authors: Batey R. , Scott J. , Jain S. , Sherlock Sheila
Published: August 2010

A patient of 14 years suffering from secondary haemosiderosis and thalassaemia major was treated with i.v. desferrioxamine in doses up to 3 g/d. Urine iron loss ranged from 46–158 mg/d. Despite improvement in cardiac and hepatic status the patient developed acute renal insufficiency of the pre renal type. The possible role of desferrioxamine in this complication is discussed.

Desferrioxamine given s.c. or i.v. effects a marked urinary iron loss but its use may not be without significant complications.
An Intestinal Receptor for Heme
Authors: Gräsbeck Ralph , Kouvonen Ilkka , Lundberg Magaretha , Tenhunen Raimo
Published: August 2010

Porcine upper small intestine took up radioactive heme in vitro. The binder was localised to the brush borders, was solubilized with Triton X‐100 and bound heme with high affinity. The binding was inhibited with non‐radioactive heme but not with FeSO4, and control experiments indicated that it was not any of the known heme binders or aggregated heme. The binding factor could also be solubilized from human duodenum.
Measurement of Antihaemophilic Factor A Antigen (VIII:CAg) with a Solid Phase Immunoradiometric Method Based on Homologous Non‐Haemophilic Antibodies
Authors: Holmberg Lars , Borge Lise , Ljung Rolf , Nilsson Inga Marie
Published: August 2010

Antihaemophilic‐factor‐A‐antibodies, which had spontaneously arisen in 2 patients, were used to develop an immunoradiometric method for measurement of antihaemophilic factor A antigen (VIII:CAg). 13 patients with severe haemophilia A had VIII:CAg below the limit of detection (0.01 U/ml). Patients with moderate and mild haemophilia A either had VIII:CAg roughly equal to factor VIII clotting activity (VIII:C) or a not detectable VIII:CAg, suggesting 2 different molecular mechanisms in moderate and mild haemophilia A. VIII:CAg could be detected in serum but in lower amounts than in plasma. In 2 patients with von Willebrand's disease VIII:CAg equalled VIII:C. The post‐transfusional retarded increase of VIII:C in 1 patient with von Willebrand's disease was accompanied by a slight increase in VIII:CAg. Fetal plasma contained measurable amounts of VIII:CAg.
Phagocytic Potential of Hairy Cells
Authors: Jansen J. , Meijer C. J. L. M. , Van der Valk P. , de Bruyn W. C. , Leijh P. C. J. , den Ottolander G. J. , van Furth R.
Published: August 2010

The hairy‐cells (HC) of 10 patients with hairy‐cell leukaemia were studied with several techniques to evaluate their phagocytic potential. Mononuclear cells from normal donors and from patients with acute monocytic leukaemia served as controls.

Light microscopically HC seemed to have ingested bacteria or latex particles. Treatment of the cells with lysostaphin, an enzyme that kills extracellular Staphylococcus aureus, showed that almost all ‘ingested’ bacteria were extracellular. Lanthanum nitrate, added during the fixation procedure for electron microscopy, stained both the outer cell membrane and the membranes of the ‘phagosomes’ of the HC, also indicating that the ‘ingested’ particles were extracellular. HC showed no increased oxygen consumption on exposure to bacteria in the presence of serum. Furthermore, HC showed no lysozyme or peroxidase activity, whereas non‐specific esterase activity was much weaker than in monocytes.

These findings, which show that HC are essentially non‐phagocytic, constitute strong evidence against a monocytic origin of the malignant cells of hairy‐cell leukaemia.
Familial Monocytic Leukaemia A Report of Two Families
Authors: Kjellström T. , Barkenius G. , Mlmquist J. , Rausing A.
Published: August 2010

2 families with a total of 5 cases of acute monocytic leukaemia are described. Possible implications for the pathogenesis of acute leukaemia are discussed.
Growth of the Reh Cell Line in Diffusion Chambers: Evidence for Differentiation Along the T‐ and B‐Cell Pathway
Authors: Lau B. , Jäger G. , Thiel E. , Rodt H. , Huhn D. , Pachmann K. , Netzel B. , Böning L. , Thierfelder S. , Dörmer P.
Published: August 2010

Cells of the Reh line, originally derived from an ALL of the ‘Non‐T, Non‐B’ type were cultured in diffusion chambers implanted intraperitoneally into preirradiated CBA mice. At different intervals over a period of 20 d changes in surface characteristics were examined by labelling the cells with AcALLG, ATCG as well as with polyvalent AIg. The evaluation was performed by using direct immunofluorescence. In addition, the ability to form rosettes with SRBC, AET‐treated sheep erythrocytes, mouse red blood cells and EoxAC was tested. On day 0 of the diffusion chamber culture the cells only carried cALLA, and no rosette formation was observed. In the course of the diffusion chamber culture the cells unequivocally developed T‐cell antigen, and in 1 of 2 experiments they further acquired a receptor for forming AET‐and E‐rosettes. Conversely, in the other experiment a receptor for mouse red blood cells was detected in a considerable portion of the cells. Our data show that the rather undifferentiated Reh line cells in vitro are able to develop features of mature T‐cells and attributes of early B‐cells during the diffusion chamber culture. In vitro they apparently retain a bivalent potentiality of lymphatic maturation. The diffusion chamber system proves to be a suitable tool for promoting differentiation in these cells.
The Leucocyte Alkaline Phosphatase Activity in Mature Neutrophils of Different Ages
Authors: Bondue Henri , Machtn David , Stryckmans Pierre A.
Published: August 2010

The marrow myeloid precursor cells of a haematologically normal patient were labelled by intravenous injection of tritiated thymidine. Young labelled segmented neutrophils were then released from the marrow into the blood by an injection of Cortisol. These PMN had a significantly lower LAP activity than the older blood neutrophils. It is shown that within the morphologic boundaries of the segmented PMN, the cells are still in different stages of cytoplasmic maturation. In addition, labelled and unlabelled neutrophils showed a linear and parallel increase of LAP activity during the 24 h following Cortisol injection. This observation suggests that the neutrophil prematurely released in the blood can mature into normal LAP‐PMN and more generally that LAP activity of a blood neutrophil increases with time.
The Internal Viscosity of the Human Erythrocyte May Determine Its Lifespan in Vivo
Authors: Williams A. R. , Morris D. R.
Published: August 2010

Young red cells obtained by high‐speed centrifugation of a normal blood sample had an MCHC of 31.7 g/dl which corresponds to an internal viscocity of about 9 cP. Old red cells from the same blood sample had an average MCHC of 37.5 g/dl which is equivalent to a mean internal viscosity of about 54 cP. This increased internal viscocity of old cells must result in a prolonged transit time through the narrow channels of the splenic microvasculature and consequently an increased probability of contact with a phagocytic macrophage. This probability of contact increases as the internal viscosity of the cell is further increased and may represent one of the major factors associated with the detection and subsequent elimination of senescent cells by the spleen.
Suppressor T‐Lymphocytes and Auto‐Anti‐B‐Cell Cytotoxic T‐Lymphocytes in a Case of Acquired Hypogammaglobulinaemia
Authors: Kristensen Tom , Nielsen Johan Lanng , Ellegaard Jørgen
Published: August 2010

A case of acquired hypogammaglobulinaemia has been examined. Total and differential leucocyte counts and distribution were normal as were the lymphocyte sub‐populations when defined by surface membrane immunoglobulin, E‐, EA‐ and EAC‐rosette techniques.

The proliferative response of unseparated lymphocytes to PHA and Con‐A was normal while the response to PWM was clearly impaired. In contrast, testing of purified B‐lymphocytes by PWM was within the normal range. The response to PWM was found to be depressed by adding the patient's T‐lymphocytes to autologous B‐lymphocytes as well as to B‐lymphocytes from 10 normal, healthy controls. The patient's T‐lymphocytes were further tested for cytotoxicity against autologous B‐lymphocytes and B‐lymphocytes from the 10 control individuals. Cytotoxicity was observed against autologous B‐lymphocytes and against the B‐lymphocytes of 3 controls.

These findings indicate the presence of peripherally circulating unspecific T‐suppressor lymphocytes and cytotoxic T‐lymphocytes of some immunogenetic specificity. The suppressor and cytotoxic T‐lymphocytes may separately or in combination be involved in the pathogenesis of hypogammaglobulinaemia. Thus, these findings are equivocal but underline the immunologic aspects of the pathogenesis.
Acute Myeloblastic Leukaemia in Sarcoidosis Treated with Methotrexate
Authors: Hermann Christian , Andersen Erik , Videbæk Aage
Published: August 2010

A 60‐year‐old man with sarcoidosis of skin and knee joints was treated with prednisone and methotrexate (MTX).

A total of 590 mg MTX was given over two periods each of 3 months duration.

4 1/2 years after the first MTX dose he developed acute myeloid leukaemia.

Only one more case of acute myeloid leukaemia following MTX treatment for a benign disease has been described, and until further cases have been reported the correlation might be considered a matter of simple coincidence.
THE CONTROL OF HEPARIN THERAPY BY THE ACTIVATED PARTIAL THROMBOPLASTIN TIME: RESULTS OF COLLABORATIVE STUDIES
Authors: Thomson Jean M
Published: August 2010

Collaborative studies to measure the effect of heparin on the activated partial thromboplastin time (APTT) have been conducted by the National (UK) Reference Laboratory for Anticoagulant Reagents and Control (NRLARC) in Great Britain and overseas and by the College of American Pathologists (CAP) in the United States. The value of multicentre trials to assess the various APTT methods, as opposed to single centre investigations, is discussed. Results from the NRLARC studies indicate that APTT methods from commercial manufacturers are relatively insensitive to heparin at low levels, compared with the standardised APTT reagent and technique produced by the NRLARC. The latter shows good sensitivity over a wide range of heparin concentrations. Variables encountered with the different commercial APTT methods are outlined. An APTT reagent should show good sensitivity at low levels and a linear response to a wide range of heparin concentrations. The in vivo response of the NRLARC standardised method in detecting circulating heparin during a clinical study of low‐dose heparin prophylaxis during surgery has also been evaluated.
STANDARDS FOR FACTOR VIII
Authors: Barrowcliffe T. W.
Published: August 2010

Because of the need to relate haemophilic samples to the normal population, the unit of Factor VIII clotting activity (VIII:C) was first defined as the amount in 1 ml. of normal plasma. However, because of wide variation in the normal population, and poor stability of VIII:C in plasma, pooled normal plasma is unsuitable as a reference standard for Factor VIII. The 1st International Standard for Factor VIII was established in 1970 to provide a stable reference material. It consisted of a freeze‐dried intermediate‐purity concentrate, and its potency was established by comparison with fresh normal plasma in a large number of laboratories. This standard was replaced in 1976 by the 2nd International Standard, also a stable intermediate purity concentrate. These long‐term reference standards are intended for calibration of working standards, and for manufacturers of concentrates it is preferable to use concentrates as working standards, because of the desirability of assaying «like against like». For assay of patients' plasmas, a plasma standard is necessary, and in the U.K. successive batches of British plasma standards have been calibrated against the International Standard (concentrate) by several laboratories. However, calibration of these plasmas against the concentrate standard has been shown to give substantial discrepancies between laboratories, and an overall discrepancy of 20% between one‐stage and two stage assays. Although standardisation of reagents and technique has improved agreement between laboratories, discrepancies still remain, and these could be markedly diminished by establishment of a plasma reference standard, to exist alongside the concentrate standard. Improvements in methods of handling have led to better stability of Factor VIII in freeze‐dried plasma and it is now planned to establish a stable reference plasma for VIII:C. Such a plasma could also act as a reference for the other Factor VIII related activities, i.e. VIII related antigen, VIII clotting antigen, and VIII ristocetin co‐factor.
Lymphocyte Size and Survival of Patients with Chronic Lymphocytic Leukaemia (B‐Type)
Authors: Rozman Ciril , Miontserrat Emili , Feliu Evarist , Woessner Soledat
Published: August 2010

In 57 cases of B‐type chronic lymphocytic leukaemia (B‐CLL), the size of peripheral blood lymphocytes was estimated by means of transmission electron microscopy. The mean lymphocyte diameter (MLD) of 50 cells was correlated with the clinical staging as well as the survival. 30 out of 38 patients found in stages 0, I, and II displayed a normal or increased MLD. Conversely, this value was decreased in 12 out of 17 cases in stages III and IV. MLD of patients in clinical stages III and IV was significantly lower as compared with MLD of patients in stages 0, I, and II (P < 0.001). The actuarial curve of 57 patients showed a roughly estimated median survival probability of 43 months. This was of more than 54 months in patients with normal or increased MLD, but only of 22 months in those with a decreased MLD. The difference between these 2 survival curves was statistically significant (P < 0.01). A reduced peripheral blood lymphocyte size, as estimated in suspension by means of transmission electron microscopy, appears to have a bad prognostic significance.