bluuebunny/crossref_metadata_2025 huggingface dataset:
Erythroblast Kinetics in Pernicious Anaemia, Erythroleukaemia and Sideroblastic Anaemia
Authors: Suda Tetsuo , Omine Mitsuhiro , Tsuchiya Jun
Published: August 2010

DNA synthesis time (Ts) and 3H‐thymidine labelling index (TLI) of erythroblasts have been determined in 25 patients with various types of haematologic disorders using in vitro double labelling method. No remarkable differences in both Ts and TLI were noted between haematologically normal subjects and patients with increased effective erythropoiesis (haemolytic anaemias), suggesting that the cell cycle time is not principally altered under the augmented erythropoiesis.

In pernicious anaemia, Ts of basophilic erythroblasts was significantly shortened and TLI was elevated above normal. Normalization of erythropoiesis by vitamin B12 was associated with a transient increase of TLI in polychromatic erythroblasts, which was interpreted to reflect prevention of intramedullary premature death of basophilic megaloblasts. Erythroleukaemia showed a markedly prolonged Ts and lowered TLI indicating the presence of cells with prolonged cell cycle time. These findings contrasted to that of pernicious anaemia despite certain morphological as well as functional similarities. In idiopathic sideroblastic anaemia, prolongation of Ts was observed to a similar extent as erythroleukaemia, while TLI remained almost normal. In 2 cases with suspected erythroleukaemia presenting an intermediate clinical picture between erythroleukaemia and sideroblastic anaemia, Ts of basophilic erythroblasts was found to be prolonged along with modestly lowered TLI.
Serum Ferritin in Megaloblastic Anaemia
Authors: Hussein Sonay , Laulicht Martine , Hoffbrand A.V.
Published: August 2010

Serum ferritin concentrations have been estimated in 30 patients with untreated megaloblastic anaemia, 27 with Addisonian pernicious anaemia. A significant difference was found between the mean serum ferritin level of the 27 pernicious anaemia patients (330 μg/l) and of 22 normal control subjects (164 μg/l) (P < 0.05 > 0.02). There was an inverse correlation between serum ferritin and Hb concentration in men with pernicious anaemia but not in women. Serum ferritin levels were lower in 10 of 13 patients studied after 24 h of vitamin B12 therapy and in all 13 studied at 48 h after therapy. The fall continued during the haematological response to therapy. It seems likely that serum ferritin reflects reticuloendothelial iron and the high levels in untreated megaloblastic anaemia are due to the shift in iron from Hb to reticuloendothelial stores. The wide variation in serum ferritin at any given Hb level presumably reflects variation in iron stores of the individual patient.
Prognostic Factors in Hodgkin's Disease
Authors: Björkholm M. , Holm G. , Mellstedt H. , Johansson B. , Killander D. , Sundblad R. , Söderberg G.
Published: August 2010

Purified blood lymphocytes from 81 consecutive and previously untreated patients with Hodgkin's disease were studied in vitro. The patients were evaluated 23 to 52 months after institution of therapy and were divided into two prognostic groups: 1. Complete remission with or without previous relapse/relapses. 2. Uncontrolled relapse or deceased from Hodgkin's disease.

Lymphocyte activation by concanavalin A, pokeweed mitogen or phytohaemagglutinin was impaired and spontaneous DNA synthesis was high in patients with poor prognosis as compared to the good prognosis group. The prognostic information increased if the four lymphocyte tests were combined in a score (range 0–8). All 8 patients with pronounced lymphocyte defects (score 7–8), died, while all patients with score 0 and 1 were in complete remission. In contrast, total lymphocyte and T‐lym‐phocyte counts and lymphocyte stimulation by PPD were of no prognostic value. The ability of certain lymphocyte functions to predict prognosis was equal or better than that of age and better than clinical staging, histopathology and symptoms.
Acute Renal Failure Associated with a Malignant Lymphoproliferative Disorder with Monoclonal Light Chain Immunoglolobulin Production
Authors: Berkel J. , Granillo‐Bodansky C. , Borne A. E. G. Kr. v. d.
Published: August 2010

A case of a 32‐year‐old male negro is reported in whom acute renal failure was the first manifestation of a diffuse lymphoplasmocytic infiltrative disorder. The malignant cells only produced immunoglobulin light chain (Λ‐type).

A description of the pathological findings is given. The case presented can be considered an intermediate form between macroglobulinaemia and multiple myeloma.
Hypercatabolism of the Third Component of Complement (C3) in Angioimmunoblastic Lymphadenopathy
Authors: Teisberg P. , Åkesson I. , Halvorsen S. , Skjørten F. , Brinch L.
Published: August 2010

A metabolic study with radioactively labelled C3 has been performed in a patient with angioimmunoblastic lymphadenopathy (AILD). The labelled protein was metabolized at a faster rate in the patient than in normal individuals. This indicates that the humoral immune system may be of pathogenetic significance in the AILD syndrome.
Characterization of the Normoblast Population in β‐Thalassaemic Blood by Rapid‐Flow Cytofluorometry
Authors: Rachmilewitz Eliezer A. , Thorell Bo
Published: August 2010

Samples of peripheral blood from patients with β‐thalassaemia major which contained significant numbers of nucleated normoblasts were stained with acridine orange and analyzed with rapid‐flow cytofluorometry. The pyknotic normoblast‐nuclei gave less green ‘DNA’ fluorescence than the (diploid) leucocytes and constituted a separate, distinct subpopulation. Mean values of the fluorescence intensities and standard deviations as displayed by multichannel analyses gave a numerical value for normoblasts with regard to their maturation stages. These mean values correlated with the differential counts of ‘early and late’ normoblasts in the light microscope under rigidly standardized conditions. Rapid‐flow cytofluorometry thus provides an objective and quantitative way to monitor and define peripheral blood normoblast populations as a measure of the severity of ‘erythropoietic stress’.
Harvesting of Granulocytes for Transfusion Therapy by Haemonetics 30
Authors: Rosenkvist J. , Behnke O. , Johansen Kirsten Stæhr , Karle H. , Nielsen O. Saxtrup , Sørensen H. , Valerius N. H.
Published: August 2010

By means of a Haemonetics 30 blood cell separator white blood cells (WBC) were collected for transfusion purposes from 21 normal unstimulated donors. Citrated Hydroxyethyl Starch (HES) was used as anticoagulant. A mean yield of 1.13 times 1010 WBC was obtained in 2 1/2–3 h. Half of the collected WBC were PMN cells containing a few per cent band forms, the other half lymphocytes and monocytes (12 %). No shift in the distribution of WBC was found during the leucapheresis. Light and electron microscopy did not reveal any damage or changes in the structure of the processed granulocytes. The Trypan blue exclusion test showed 99 % vital cells. In vitro assessment of the chemotactic and bactericidal activity of the collected granulocytes showed normal function. Also the content of myeloperoxidase was within normal range.
BCG Therapy in Acute Non Lymphoid Leukaemias
Authors: Vuvan H. , Fiere D. , Doillon M. , Martin C. , Coiffier B. , Felman P. , Bryon P. A. , Favre‐Gilly J. , Revol L.
Published: August 2010

The survival of patients with acute non‐lymphoid leukaemias (ANLL) receiving chemotherapy (32 patients) or chemotherapy plus BCG (31 patients) has been compared in a randomized trial which started in Nov 1974. Fresh BCG (Institut Pasteur, Paris) was administered by Heaf gun in the interval of chemotherapy cycles. The overall survival was better in the chemo‐immunotherapy group (median duration of survival of 25 months compared to 16 months in the chemotherapy group). However, the first remission duration did not differ (median duration of the 15 and 12 months, respectively). Plateau survival curves were not obtained in either group.

A second complete remission was obtained in 7 out of 12 patients with bone marrow relapses in the chemo‐immunotherapy group, and only in 2 out of 15 in the chemotherapy group (P < 0.05). BCG seemed more efficient in patients older than 40 years; no difference was observed according to sex.
Reversible Severe Anaemia and Granulocytopenia Caused by Procainamide
Authors: van Beek Rudolf J. J. , Bieger Robert , den Ottolander Gerard J.
Published: August 2010

A 64‐year‐old male developed severe anaemia during procainamide therapy. At the same time granulocytopenia was found. Discontinuation of procainamide led to complete recovery. Severe anaemia due to the use of procainamide is not well known. The available data indicate a maturation block of the erythropoiesis as the possible cause of the anaemia, perhaps in combination with an autoimmune haemolysis.
Characteristics of Anaemic Women The Population Study of Women in Göteborg 1968–1969
Authors: Lennartsson Jan , Bengtsson Calle , Hallberg Leif , Tibblin Elisabeth
Published: August 2010

Anaemic women (Hb concentration < 120 g/1) who participated in a population study of women in Göteborg, Sweden were compared to the women in the population sample as a whole. Due to the way of sampling and a high participation rate the sample was representative of women in Göteborg in the ages studied (age strata between 38 and 60). In the same way the anaemic women were representative of anaemic women in the same general population. Iron deficiency was found to be the main reason for the anaemia in the ages of 38–50 years. Except for big menstrual blood losses in anaemic women no reason for iron deficiency could be found. History of infectious diseases was about as common in anaemic women as in women in the total population sample. Sleeping habits, time off work and physical activity at work and during leisure time were similar in anaemic women and in women in the general population. The proportion of women who reported a subjective feeling of tiredness was also similar.
The Relationship between Marrow Iron Stores, Plasma Ferritin Concentrations and Iron Absorption
Authors: Bezwoda W. R. , Bothwell T. H. , Torrance J. D. , MacPhail A. P. , Charlton R. W. , Kay G. , Levin J.
Published: August 2010

The percentage absorption from a 3 mg dose of ferrous iron was measured in 50 subjects with iron stores that varied over a wide range. Iron status was assessed by a number of measurements, including the haemoglobin concentration, the plasma iron concentration, the total iron‐binding capacity, the plasma ferritin concentration and the concentration of non‐haem iron in the bone marrow. There were good inverse correlations between the log percentage iron absorption and both the log marrow non‐haem iron concentration (r —0.94; P < 0.001) and the log plasma ferritin concentration (r —0.78; P < 0.001). In addition, there was a positive correlation between the marrow non‐haem iron concentration and the plasma ferritin concentration (r +0.84; P <0.001). These results suggest that reticuloendothelial iron stores represent an important determinant of iron absorption and that their size can be guaged from the plasma ferritin concentration.
Scanning Electron Microscopy of Exudative Macrophages in Malignant Lymphoma
Authors: Sokol R. J. , Durrant T. E. , Lambourne C. A. , Hudson G.
Published: August 2010

The possible value of scanning electron microscopy (SEM) of skin window preparations in the clinical situation has been investigated with reference to 48‐hour macrophages in 26 patients with malignant lymphoma.

The preparations were processed for SEM using critical point drying and sputter coating. Identification of macrophages was made with reference to light microscopy both of parallel preparations and of specimens already studied by SEM. Distinctive SEM appearances were present in most of the patients. Although many macrophages were similar to those from normal subjects, the majority showed a more variable morphology including the presence of ruffles and coarse ridge‐like profiles. Highly significant differences were found on statistical analysis. It is suggested that immaturity of the 48‐hour macrophages is present in some patients with malignant lymphoma (perhaps reflecting the macrophage dysfunction already described in these diseases). The findings indicate the potential clinical application for this technique.
Concomitance of an Active and an Inactive Mutant of Red Cell Pyruvate Kinase (PK)
Authors: Zanella A. , Rebulla P. , Izzo C. , Zanuso F. , Sirchia G.
Published: August 2010

A new mutant red cell PK associated with mild chronic haemolytic anaemia is described. The propositus, double heterozygous for a maternal gene coding for a structural abnormal enzyme and a paternal gene coding for a catalitically inactive enzyme, was suitable for an accurate functional characterization of the PK variant since his erythrocytes contained only one active mutant form of this enzyme. The active isoenzyme was characterized by low activity, decreased affinity for phospho‐enolpyruvate, incomplete fructose‐1,6‐diphosphate activation, increased ‘zero‐time transition temperature’, increased stability to guanidine‐HCl and storage at + 4° C, increased guanosine‐5′‐diphosphate and cytidine‐5′‐diphosphate utilization, altered elec‐trophoretic pattern with a single slow‐moving component and abnormal isoelectric point. Affinity for ADP, ATP inhibition, optimum pH, molecular weight of the sub‐units, antigen concentration and immunological properties were in the normal range.
Adult Acute Lymphoblastic Leukaemia: Study of 32 Patients and Analysis of Prognostic Factors
Authors: Ruggero Donatella , Baccarani Michele , Gobbi Marco , Tura Sante
Published: August 2010

The clinical and haematological characters and the response to therapy of 32 consecutive patients with acute lymphoblastic leukaemia, aged more than 12 years, were reviewed. All patients were given vincristine (V) and (6‐methyl) prednisolone (P) for 6 weeks; daunomycin (D) was added in 9 cases, and cyclophosphamide (C) + D in another 10 cases. 3 patients died during induction. 4 patients failed to achieve remission. 25 patients (78 %) achieved complete remission (CR). All of them but one received ‘prophylactic’ central nervous system (CNS) therapy with cranial irradiation and i.t. methotrexate (MTX) and arabinosyl cytosine. CR was maintained with daily 6‐mercaptopurine and weekly MTX. Median duration of CR was of 22 months. 2 patients are currently disease‐free and off‐therapy, 78 and 53 months after diagnosis, respectively. Blast cell membrane markers were studied in 21 consecutive cases: 3 patients had T‐cell leukaemia and 2 patients B‐cell leukaemia. Prognostic factors were evaluated basing on the present series of 32 patients, and on further 106 cases reported by others. Age (under and above 40) influenced significantly both the CR rate and the length of survival. In patients aged under 40, a circulating blast cell count higher than 25,000/μl, or a platelet count lower than 50,000/μl, negatively affected the survival.
The Effect of Sonicated Serum on Growth of Normal and Leukaemic Human Cells in Agar Cultures
Authors: Knudtzon S. , Mortensen B. T.
Published: August 2010

The existence of circulating factors in blood of possible importance for granulo‐poiesis was investigated by examining the effect of sonicated serum on agar cultures of human haematopoietic cells. Ultrasound treatment of serum can activate enzymes normally bound to carrier proteins.

In normal bone marrow cultures, growth was inhibited by sonicated serum when the cells were cultured in a single layer without exogenous colony‐stimulating factor (CSF) included in the culture, while an enhancing effect with a 2–5 fold increase in the number of colonies was seen in feeder layer‐stimulated cultures. Morphologically, in contrast to the normal change to eosinophils and monocytes‐macrophages during the culture period, the cultures with sonicated serum added showed continuous growth of neutrophils and no increase of other cell types. Experiments using tritiated thymidine indicated that the enhancing effect of sonicated serum involved marrow cells which were more prone to thymidine S‐phase kill. The effect of sonicated serum was further evaluated in cultures from patients with leukaemia. In AML in relapse, feeder‐layer stimulated cultures of bone marrow cells were inhibited (11/14 cases) by sonicated serum, while reversion to the normal enhancing pattern was seen for patients in remission.
Acquired von Willebrand's Syndrome and Ehlers‐Danlos Syndrome Presenting with Gastro‐Intestinal Bleeding
Authors: Clough Virginia , MacFarlane I. A. , O'Connor J. , Wood J. K.
Published: August 2010

The clinical and laboratory findings in a patient with severe gastrointestinal bleeding and the Ehlers‐Danlos syndrome are described. Coagulation studies and a lack of history of previous haemorrhage were consistent with a diagnosis of acquired von Willebrand's syndrome. His response to treatment with blood transfusion, Factor VIII, cimetidine, tranexamic acid and cyclophosphamide is described. Family studies revealed other members with Ehlers‐Danlos syndrome but normal coagulation.
Effects of Serum and Cations on the Selective Release of Granular Proteins from Human Neutrophils during Phagocytosis
Authors: Venge Per , Hällgren Roger , Stålenheim Gunnemar , Olsson Inge
Published: August 2010

The extracellular release from human neutrophils of the primary (azurophil) granule constituents, myeloperoxidase (MPO), chymotrypsin‐like cationic protein (CCP), col‐lagenase and lysozyme, and the secondary (specific) granule constituents, lactoferrin and lysozyme, was measured during ingestion of staphylococcus protein‐A‐IgG complexes. In buffer, lactoferrin release was consistently higher than that of the other protein. In serum, lactoferrin release increased concomitantly with ingestion, whereas the rate of lysozyme and especially of MPO release were stimulated to a higher degree than ingestion.

Magnesium (0.5‐2 mM) was more potent than calcium (0.5‐2 mM) in promoting release but these cations worked synergistically. Zinc (0.5‐4 mM) was found to be a potent and selective inhibitor of collagenase release. Manganese (0.25‐4 mM), which inhibited the ingestion of SpA‐IgG complexes, also inhibited release of CCP, collagenase, lysozyme and MPO, but actually stimulated lactoferrin release. The data suggests that lactoferrin and lysozyme may be confined to distinct granule populations or else released in a different fashion from the granules. When the effects on release of primary granule proteins are concerned it is suggested that the dissociation of binding of various agents to an anionic granule matrix may be affected differently by various cations.
Quantitation of Erythropoiesis in Myelomatosis
Authors: Birgens Henrik S. , Hansen Ole Paaske , Henriksen Jens H. , Wantzin Per
Published: August 2010

Quantitation of the erythropoiesis with radio‐iron (59Fe) was applied to 9 patients with untreated myelomatosis. The method included blocking of the 59Fe reutilization by injection of non‐radioactive iron.

There was no uniform pattern in the Fe‐kinetics values. The Plasma Iron Turnover (PIT) and the Red Blood Cell Iron Turnover (RBCIT) varied from subnormal to values markedly increased above upper normal limit. The calculated average Mean Red Cell Life time (MRCL) of erythrocytes was just below normal range. The mean Marrow Transit Time (MTT) was normal in the patients, despite subnormal venous haematocrit, indicating insufficient stimulation of the bone marrow. The renal function, measured as 51Cr‐EDTA clearance, was found positivily correlated to the RBCIT (r = 0.78, P < 0.05). The results suggest that the previously demonstrated relationship between anaemia and renal failure in patients with myelomatosis is caused mainly by an inability of the bone marrow to produce sufficient red blood cells under the stress of anaemia related to the degree of renal impairment.
Development of Human Granulocyte and Monocyte Fc Receptors
Authors: Herborn H. A. , Valdimarsson H. , Wickramasinghe S. N.
Published: August 2010

Bone marrow cells were examined for the presence of IgG‐Fc receptors using a red cell rosette technique. Receptors were demonstrated on some of the neutrophil pro‐myelocytes, almost all of the neutrophil myelocytes, and a few of the eosinophil myelocytes. Cells which appeared on morphological grounds to be promonocytes also expressed Fc receptors.
The Varied Sensitivity of Partial Thromboplastin and Prothrombin Time Reagents in the Demonstration of the Lupus‐Like Anticoagulant
Authors: Mannucci P. M. , Canciani M. T. , Mari D. , Meucci P.
Published: August 2010

An acquired inhibitor of blood coagulation, similar to that described in patients with Systemic Lupus Erythematosus (SLE), was detected during routine coagulation screening in 10 patients who did not meet the criteria for a diagnosis of SLE. The lupus‐like anticoagulant (LLAC) was diagnosed on the basis of prolonged activated partial thromboplastin time (APTT) and/or prothrombin time (PT) which failed to correct when patient plasma was added to normal plasma; an additional criterion was an abnormal tissue thromboplastin inhibition test. No patient had a specific inhibitor directed against factors VIII and IX. Demonstration of LLAC was highly dependent upon the type of reagents adopted in the APTT and PT: the abnormality was detected consistently by one reagent only. One‐stage assays of factors VIII and IX were characteristic of the presence of an inhibitor, showing non‐parellel dose‐response curves or decreased activity at low dilutions which were partially corrected at higher dilutions. Although 7 patients were free of abnormal bleeding, unequivocal signs of haemorrhagic tendency after a surgery were present in the remaining 3 patients. The findings suggest that LLAC is a non‐exceptional cause of prolonged coagulation screening tests, and that it may sometimes be associated with impaired haemostasis.
Isoenzymatic Study of Leucocytic Acid Phosphatase in Haematologic Diagnosis
Authors: Lafuente Rosario , Woessner Soledad , Sans‐Sabrafen J.
Published: August 2010

Isoenzymatic study of leucocytic acid phosphatase under normal conditions identifies 3 isoenzymatic bands, which exhibit a noticeable cell specificity. Band 2 is granulocytic, band 3 lymphocytic and band 4 monocytic in origin. Pathologic deviations in the isoenzymatic pattern are both qualitative and quantitative. For some diseases such as chronic lymphocytic leukaemia there: is a well‐defined, differential pattern according to immunological B‐ or T‐cell origin. The more significant qualitative aspects are related to the appearance of abnormal bands, especially band 3b, indicating blastic cellularity, and 5, corresponding to hairy cells. The isoenzymatic analysis of acid phosphatase activity is a simple haematdogic complementary test, particularly useful in the differential diagnosis of lymphoproliferative disorders with peripheral blood manifestations.
The Diffusion Chamber Technique as an in Vivo Assay in Mice for the Effectiveness of Antitumor Agents
Authors: Huybrechts Michel , Symann Michel , Trouet André
Published: August 2010

The diffusion chamber (DC) technique has been applied to the culture of L1210 cells in view of determining their sensitivity to chemotherapic agents. The surviving fraction of L1210 cells cultivated in DC's 4 d after i.v. injection of the host mice with 10 mg/kg of daunorubicin or daunorubicin‐DNA was 40% and 9%, respectively. The results suggest that the DC technique could be a useful in vivo predictive test for cancer chemotherapy.
Human Erythropoiesis in Diffusion Chambers
Authors: Walther F. , Schopow K.
Published: August 2010

The diffusion chamber technique has been modified in order to stimulate human erythropiesis in addition to granulo and monocytopoiesis.

The chamber recipient mice were subjected to a combination of 750 rad irradiation and long‐term non‐interrupted hypoxia (0.5 atm) without chamber retransplantation. 7 d after implantation erythroid cells started to increase, reaching a maximum of up to 70 % of the nucleated cells on day 12.
Phase II Trial of Prednimustine®, Leo‐1031 (NSC‐134087) in Acute Non‐Lymphocytic Leukaemia
Authors: Juul K. , Sørensen P. , Pedersen‐Bjergaard J. , Jensen M. Krogh
Published: August 2010

18 patients with acute non‐lymphocytic leukaemia were treated with Prednimustine, a chlorambucil ester of prednisolone, as a single drug. 5 patients responded with falling blast counts in peripheral blood, but only 1 obtained a complete remission of 3 months' duration. Since the activity of Prednimustine is lower than that of other commonly used drugs in acute non‐lymphocytic leukaemia, future studies should concentrate on other aspects, such as treatment of patients with steroid receptor‐positive blast cells or other types of leukaemia.
Glucose‐6‐Phosphate Dehydrogenase Deficiency in a Native Danish Family A New Variant
Authors: Cohn Jørgen , Carter Nick , Warburg Mitte
Published: August 2010

Deficiency of red cell glucose‐6‐phosphate dehydrogenase was found in a native Danish family, in which 2 boys suffered from severe haemolytic anaemia. The mother and 3 sisters of the boys were heterozygotes for G‐6‐PD deficiency. The biochemical investigations indicate that this deficient G‐6‐PD is very similar to the Mediterranean variant; however, this variant gene may represent another example of G‐6‐PD ‘Helsinki’ or an unique variant with properties similar to G‐6‐PD B(—).
Transient Acquired Resistance to the Coumarin Anticoagulants Phenprocoumon and Acenocoumarol
Authors: Wolff Frederik A. de , Cate Jan W. ten
Published: August 2010

A 62‐year‐old man developed resistance to coumarin congeners during treatment with phenprocoumon (Marcoumar®) and acenocoumarol (Sintrom®), requiring at least triple the usual therapeutic dose and plasma concentration of phenprocoumon for satisfactory anticoagulation. After 8 months on this high dosage, the patient suddenly developed haemorrhagic diathesis. The medication was therefore discontinued. The resistance could not be ascribed to impaired absorption or enhanced metabolism.
B‐Cell Acute Lymphatic Leukaemia: Immunoglobulin Synthesis, Morphology and Clinical Features
Authors: Smith J. , Newell D. G. , Gordon J. , Anson A. , Roath S.
Published: August 2010

A detailed case study of a B cell acute lymphocytic leukaemia (ALL) is described. Ultrastructurally the neoplastic cells resembled other cases of ALL studied. The majority of the neoplastic cells had detectable surface IgGλ and receptors for Feγ while a minority of the cells had receptors for the C3 component of complement. Neoplastic cell preparations were able to synthesize IgGλ with a surplus of free λ chains. This case did not respond to treatment, and death ensued within 36 h of presentation. The clinical and laboratory findings are discussed with respect to other cases of ALL.
Erythrocyte and Leucocyte Enzymes in a Case of Paroxysmal Nocturnal Haemoglobinuria
Authors: Hanel H. K. , Mejer J. , Mørck H. I.
Published: August 2010

In a patient with paroxysmal nocturnal haemoglobinuria (PNH) enzymatic activities of erythrocytes and leucocytes were studied. Studies of autohaemolysis were also performed.

The following erythrocytary enzymes were measured: Glucose‐6‐phosphate dehydro‐genase (G‐6‐PD), pyruvate kinase (PK), glutathione reductase (GR), and acetyl‐cholinesterase (AcChE).

The following enzymes were measured in leucocytes: Adenosine deaminase, purine nucleoside phosphorylase, adenine phosphoribosyltransferase, hypoxanthine phosphoribosyltransferase and adenosine kinase.

Normal activity of G‐6‐PD, GR and PK in erythrocytes was found. In leucocytes and lymphocytes activity of purine nucleoside phosphorylase was reduced. Autohaemolysis in vitro was increased, which could not be compensated by addition of glucose or ATP.
Red Cell Inclusion Bodies in a Case of Preleukaemia
Authors: Streichman S. , Tatarski I. , Manaster J.
Published: August 2010

A 26‐year‐old female patient is reported in whom red cell inclusion bodies (Hb‐H) and thrombocytopenia appeared more than 5 years before the emergence of atypical chr. myeloid leukaemia.

Attention is drawn to the fact that the recently uncovered mechanism of acquisition of Hb‐H (absence of transcription of specific genes) has previously been implicated in other biochemical changes typical of leukaemia.
Mucosal Uptake, Mucosal Transfer and Retention of Iron, Measured by Whole‐Body Counting
Authors: Marx J. J. M.
Published: August 2010

A method is described which enables simple and accurate measurement of the separate steps of iron absorption. A double isotope technique was used with 59Fe as a tracer and 51Cr (as CrCl3) as an inert, not absorbable indicator. With a whole‐body counter it was possible not only to measure initial mucosal uptake and retention but also to calculate mucosal transfer of iron. Thus faeces collection for determination of mucosal uptake of iron was no longer necessary.

Absorption of a physiological dose of inorganic iron was studied in 25 normal subjects (15 males and 10 females), in 21 patients with uncomplicated iron deficiency and in 8 patients with idiopathic haemosiderosis. A significant difference was demonstrated between normals and patients with iron deficiency and in idiopathic haemosiderosis with respect to the various steps of iron absorption. The method provides a simple tool for the study of the regulation of iron absorption at the luminal and intracellular site of the intestinal mucosal cell.
Acute Non Myeloid Leukaemia in Adults: Prognostic Factors in 92 Patients
Authors: Brun B. , Vernant J. P. , Tulliez M. , Kuentz M. , Deregnaucourt J. , Shultze L. , Reyes F. , Rochant H. , Dreyfus B.
Published: August 2010

An analysis of prognostic factors was performed on a series of 92 adult patients with acute non‐myeloid leukaemia (ANML). Four factors were shown to be correlated with a poor short‐term prognosis: (1) Neuropathy involving a single cranial nerve (numbness of the chin) as a presenting feature. (2) A low percentage blast cell infiltration of bone marrow (> 55%). (3) Cytological L2 type or L3 type of cells according to FAB classification proposals. (4) Increased age.

Two factors were shown to be correlated with a poor long‐term prognosis: 1) A high level of circulating blast cells; 2) A high tumour burden, as in childhood ANML. These two long‐term prognostic factors were interrelated. In addition, our results suggested that myelofibrosis could be a poor long‐term prognosis factor as well.
Serum Selenium Levels in Malignant Lymphoproliferative Diseases
Authors: Calautti Paolo , Moschini Giuliano , Stievano Bianca Maria , Tomio Luigi , Calzavara Fulvio , Perona Giuseppe
Published: August 2010

Serum selenium levels were measured in 38 patients with malignant lymphoproliferative diseases (MLD) and in 34 non‐hospitalized healthy individuals. Selenium was determined by proton induced x‐ray emission.

In Hodgkin's disease and non‐Hodgkin malignant lymphoma the mean serum levels of selenium were not different from those of the control group. On the contrary lowered mean serum selenium concentrations were observed in the group with chronic lymphocytic leukaemia (5.2 + 0.7 μg/100 ml) as compared to normal individuals (7.9 + 0.3 μg/100 ml). The difference is highly significant (P < 0.005).

A second selenium test was made in 11 out of the 38 patients within 8 weeks from the beginning of radiotherapy or chemotherapy; unchanged levels were found.
Extravascular Phagocytosis of Proerythrocytes following 5‐Fluorouracil
Authors: Radley J. M.
Published: August 2010

Amongst the perturbations induced in mouse bone marrow by 5‐fiuorouracil are the retention and phagocytosis of proerythrocytes within the extravascular compartment. Reasons for the latter response have been sought using electron microscopy. Proerythrocytes undergo normal maturation after 5‐fluorouracil, judged by loss of mitochondria and vesicles, but fail generally to attain a biconcave shape. Most proerythrocytes have lost their organelles before phagocytosis occurs. Quantitative light microscopy shows a rise in the number of proerythrocytes 1 d after 5‐fluorouracil which is attributable to maturation of orthochromatic erythroblasts. Thereafter the population size declines concomitantly with increased engulfment and phagocytosis by macrophages, activity which is maximal on days 4–5. Extravascular loss of proerythrocytes is also observed when marrow is rendered hypocellular by x‐irradiation, or hydroxyurea. It is suggested that excessive maturation of proerythrocytes in the extravascular compartment can lead to their phagocytosis.
THE ICTH/WFH STUDY OF THE PARTIAL THROMBOPLASTIN TIME IN MILD HAEMOPHILIA
Authors: Ingram G. I. C. , O'Brien P. F. , North W. R. S.
Published: August 2010

A study has been carried out in two stages to compare the sensitivity of different Partial Thromboplastin Time (P.T.T.) methods to partial deficiencies of factor VIII. In Stage 1, 63 laboratories throughout the world compared their own methods with a reference method, all using samples of the same three mild haemophilic plasmas. Wide variation was found between laboratories using the same method, especially for those methods which were not well standardised. There was fairly good agreement for the reference method. All methods correctly diagnosed the three mild haemophilic plasmas, but their factor VIII levels were rather low.

Stage 2 was restricted to seven laboratories in Great Britain, comparing seven well‐standardised commercial methods and the same reference method. A much wider range of abnormal plasmas was tested, and a variety of normal ones. P.T.T.'s for all plasmas (factor VIII levels from 5 to 58 iu/dL) fell outside the normal range by all methods, with a few exceptions. It was not possible to rank the methods in any order.
STANDARDIZATION OF THE BLEEDING TIME
Authors: Bowie E. J. W. , Owen C. A.
Published: August 2010

The Duke, Ivy and immersion methods for performing the bleeding time are reviewed and modifications of these methods are discussed. Certain of the automated devices are described. It is concluded that the bleeding time, when properly standardized, is an important test in the evaluation of a hemostatic disorder.
The Contributions of Erythropoietic and Nonerythropoietic Haem Turnover to the Early Labelled Peak of Endogenous CO Formation in Man
Authors: Lindahl J.
Published: August 2010

The endogenous production of14CO during the first week after administration of glycine‐2‐14C (early labelled peak, ELP) was determined in 13 healthy subjects. The subjects were studied during normal, suppressed or stimulated erythropoiesis induced by hypertransfusion and phlebotomy, respectively. The production of14CO was calculated from determinations of the specific activity of the body CO store and the endogenous production of CO. The incorporation of labelled glycine into circulating red cell haemoglobin haem was calculated from the maximal specific activity of haemoglobin haem and the total amount of Hb. The average ELP was 73.0 × 103disintegrations per min (dpm) and the incorporation of glycine into circulating haemoglobin haem 491 × 103dpm in controls. The ELP and the incorporation of glycine increased by 86% and 92%, respectively, in phlebotomized subjects and decreased by 37% and 62%, respectively, in hypertransfused subjects. A linear regression equation was calculated for the relationship between ELP and erythropoiesis, expressed as the incorporation of glycine into circulating haemoglobin haem. The residual ELP remaining in the absence of erythropoiesis was 24.9 × 103dpm calculated from data of all the subjects, and 32.8 × 103dpm calculated from data of controls and hypertransfused subjects only. In conclusion, erythropoietic haem turnover was found to contribute about 60% and nonerythropoietic (mainly hepatic) haem turnover about 40% of the ELP in man.
Erythrocyte Glucose‐6‐Phosphate Dehydrogenase in Chronic Renal Failure and after Renal Transplantation
Authors: Milman Nils
Published: August 2010

Erythrocyte glucose‐6‐phosphate dehydrogenase (G‐6‐PD) activity was measured in 16 non‐dialysed patients with chronic uraemia, 17 patients on regular peritoneal dialysis, 18 patients on regular haemodialysis, 10 renal transplanted patients with normal renal function, and in 41 healthy control subjects. In non‐dialysed uraemic patients G‐6‐PD values were not significantly different from those in the control group. Peritoneal dialysed patients had slightly, but significantly higher G‐6‐PD values than controls (P < 0.01). Haemodialysed patients demonstrated the highest G‐6‐PD values of all groups, being significantly higher than in both controls (P < 0.001), non‐dialysed (P < 0.01), peritoneal dialysed (P < 0.01), and renal transplanted patients (P < 0.01). Renal transplanted patients had G‐6‐PD values which not differed significantly from controls. In all uraemic patients G‐6‐PD activity was positively correlated to serum creatinine (rs = 0.64, P < 0.001) and negatively correlated to haemoglobin (rs = ‐0.61, P < 0.001). In the peritoneal and haemodialysed groups Gd‐PD activity was correlated to the reticulocyte counts (rs = 0.54, P < 0.001). The results indicate that a younger mean red cell age is responsible for the increased G‐6‐PD activity in peritoneal dialysed and haemodialysed patients.
Characteristics of the Factor VIII Protein and Factor XIII in Various Factor VIII Concentrates
Authors: Nilsson Inga Marie , Holmberg Lars , Stenberg Pål , Henriksson Per
Published: August 2010

The in vitro properties of 5 factor VIII preparations (AHF‐Kabi, Hemofil Hyland, AHF‐Profilate Abbott, Kryobulin Immuno and Factorate High Purity Armour) and an ordinary cryoprecipitate were studied with reference to factor VIII clotting activity (VIII:C), factor VIII clotting antigen (VIILCAg), factor VIII related antigen (VIIIR:Ag) (EI, IRMA, CIE), ristocetin cofactor activity (VIIIR:RCF), fibrinogen and factor XIII. All the preparations with the exception of Factorate had higher levels of VIII:CAg than VIII:C indicating inactivation of the biological activity of VIII:C during the procedure. AHF‐Kabi (fraction I‐0) and the cryoprecipitate, the only preparations capable of normalising the defect in patients with von Willebrand's disease, showed the same level of VIIIR:Ag determined by EI and by IRMA, while all the other preparations (i.e. cryoprecipitates purified further in different ways) had considerably lower levels of VIIIR:Ag determined by IRMA than by EL Based on these in vitro techniques it seems to be possible to predict which preparations can be used successfully in patients with von Willebrand's disease, while no such conclusions can be made from VIIIR:RCF determinations.

EI yielded similar concentrations of factor XIIIasubunit in all the preparations tested. 3 functional assays showed high factor XIII activities in AHF‐Kabi but low or no activities in the others.

Thus, considerable differences were found of the in vitro properties of the proteins in 5 factor VIII concentrates and a cryoprecipitate. The action of proteases and the techniques used in the purification procedure are probably of crucial importance for the properties of the various factors.
Chromosome Aberrations and Prognosis in Preleukaemia
Authors: Panani Anna , Papayannis A. G. , Sioula E.
Published: August 2010

24 patients with preleukaemia were cytogenetically studied by the G‐banding staining technique. All patients developed later frank acute non‐lymphocytic leukaemia and died. 13 patients had a completely normal karyotype (NN‐patients) with a median survival time of 8 months. 6 patients had both normal and abnormal metaphases (AN‐patients) with a median survival time of 18.5 months, while 5 patients had abnormal metaphases only (AA‐patients) with a median survival time of 3 months.
Study of Erythrocytes in a Hereditary Hemolytic Syndrome (HHS): Comparison with Erythrocytes in Lecithin:Cholesterol Acyltransferase (LCAT) Deficiency
Authors: GODIN DAVID V. , GRAY GEORGE R. , Frohlich Jiři
Published: August 2010

Erythrocyte membrane abnormalities in 3 members of a family with a hereditary haemolytic syndrome (HHS) were compared to those previously described in a family with lecithin: cholesterol acyltransferase (LCAT) deficiency. Despite similarities including an increase in membrane phosphatidylcholine, a decrease in phosphatidyl‐ethanolamine, stomatocytosis, and a marked decrease in erythrocyte osmotic fragility a number of differences were observed. These included membrane cholesterol content (increased in homozygotes with LCAT deficiency), changes in sodium and potassium content and Na+,K+‐ATPase activity (the latter being increased in HHS), changes in acetylcholinesterase and sulfhydryl group latency (present in LCAT deficiency, but not in HHS) and 2,3 DPG content (decreased in HHS, normal in LCAT deficiency). Full compensation of the erythrocyte defect occurred in HHS but the homozygotes for LCAT deficiency were slightly anaemic. It is concluded that, although similar abnormalities in phospholipid composition, osmotic fragility, and erythrocyte morphology exist in these two disorders, the molecular nature of the erythrocyte membrane structural and functional changes in HHS and LCAT deficiency is clearly different.
Presence of a Non‐Splenic Platelet Pool in Man
Authors: Vilén Lars , Fredén Krister , Kutti Jack
Published: August 2010

3 asplenic but otherwise healthy men, in whom autologous platelets had been labelled with radioactive sodium chromate, received i.v. infusions of epinephrine in a dose of 0.35 μg per kg per min over a period of 20 min. In response to these infusions an increase in the concentration of labelled platelets (average 8% over the basal value) was seen in all 3 subjects. In no case was the maximal increase in the venous haematocrit higher than 1%. Identical experiments were also carried out on 3 intact male volunteers. As compared to the asplenics, the mean maximal increase in platelet‐bound radioactivity was considerably higher (41%). It is concluded that a small but significant epinephrine sensitive non‐splenic platelet pool is present in humans.
Haemoglobin Concentrations and Erythropoietin Levels in Appropriate and Small for Gestational Age Infants
Authors: Meberg Alf
Published: August 2010

Capillary Hb concentration on the first day of life was registered in 201 infants with weight appropriate for gestational age (AGA) and in 99 infants small for gestational age (SGA). In both groups Hb increased towards term (P < 0.01), while the SGA infants reached higher Hb concentration at term (21.5 ± 1.9 g/dl; mean ± SD) than the AGA infants (17.9 ± 1.5 g/dl) (P < 0.001). The cord serum erythro‐poietin (EP) level increased towards term equally in both groups. Delivery did not seem to influence the EP level as same values were obtained in cord serum from infants delivered by elective cesarean section as after uncomplicated vaginal delivery. No correlation was found between cord serum EP and first day capillary PCV. Postnatally a rapid fall in the EP level occurred. In healthy adults no serum EP activity was detected. It is suggested that increasing hypoxia, rapid growth, and shift from hepatic to myeloid erythropoiesis may be related to the increasing Hb concentration and serum EP level towards term. Improvement of oxygenation during air breathing may cause the decreasing serum EP level after birth.
The Release of Plasminogen Activator and Factor VIII after Injection of DDAVP in Healthy Volunteers and in Patients with von Willebrand's Disease
Authors: Nilsson Inga Marie , Holmberg Lars , ÅBerg Magnus , Vilhardt Hans
Published: August 2010

Increases in plasma concentrations of VIII:C, VIII:CAg, VIIIR:Ag and plasminogen activator (PA) were observed in 50 healthy volunteers given i.v. injections of DDAVP (desaminocys1‐8‐D‐arg‐vasopressin). The PA activity reached its maximum immediately after the injection, VIII:C, VIII:CAg and VIIIR:Ag after 3040 min. However, a positive correlation was found when the PA and VIII:C responses in each of the normals were analysed.

DDAVP was also administered to 3 patients with severe von Willebrand's disease. 2 of the patients displayed no changes in VIII:C, VIII:CAg, VIIIR:Ag or VIIIR:RCF and there was no increase in PA. The third patient responded with an increase in VIII:C and to a minor degree in VIII:CAg. This patient developed fibrinolytic activity, but in the lower normal range.

In 3 other patients with mild von Willebrand's disease DDAVP caused increases in VIII:C, VIII:CAg, VIIIR:Ag and PA.

We feel that the combined data may support the concept that one and the same target cell is involved in the DDAVP mediated release of factor VIII related activities and PA.
Proliferation and Differentiation in Diffusion Chambers of Marrow, Blood and Spleen Cells from Patients with Chronic Myeloid Leukaemia during Chronic Phase and Blastic Transformation
Authors: Nilsson Bo , Olofsson Eva , Olofsson Tor , Olsson Inge
Published: August 2010

The proliferative and differentiating capacity was compared between immature normal marrow cells and marrow, blood or spleen cells from patients with chronic myeloid leukaemia (CML) in chronic and blastic phases. Low density cells highly enriched in myeloblasts and promyelocytes were cultured in diffusion chambers (DC) and implanted into the peritoneal cavity of mice pretreasted with cyclophosphamide. In CML of chronic phase cell production was higher than normal with the exception of spleen cells. Total cell production was lower in blastic phase than in CML chronic phase. The [3H]‐TdR labelling index of myeloblasts of blastic phase CML increased considerably upon implantation in DC consistent with re‐entry into active proliferation. Immature normal cells give rise mostly to macrophages while the corresponding CML cells of chronic phase give a much higher PMN production with peaks after 12–18 d. Also in CML of blastic crisis the blasts differentiate into mature PMNs, but the maturation time is shortened with peaks of PMNs after 5–10 d. DC cultures of spleen cells from patients with CML showed a less differentiating capacity with a very low PMN production compared to cultures of marrow or blood cells. Our results indicate intrinsic differences in growth and maturation capacity between normal immature granulopoietic cells and cells from patients with CML of chronic phase or blastic crisis. The results may also indicate intrinsic differences between spleen, blood or marrow cells in CML.
Granulocytic Sarcoma Preceding Acute Myeloid Leukaemia
Authors: Ersbøll Jens , Petri Jens , Jensen Klaus Hou , Hansen Mogens Mørk
Published: August 2010

The clinical and histopathological features of 3 cases of colourless granulocytic sarcoma preceding acute myelogenous leukaemia by 3, 12, and 18 months are presented. One patient had involvement of the jejunum, another patient had involvement of the vulva and cervix, the third patient had a bone tumour. In 2 patients a misdiagnosis of non‐Hodgkin's lymphoma of the histiocytic type was made, in 1 patient the initial diagnosis was eosinophilic granuloma. The diagnosis of granulocytic sarcoma depends upon the demonstration of granulocytic differentiation in the tumour. This should imply the use of naphthol AS‐D chloroacetate stain;touch imprints and electron microscopy. The differential diagnosis of granulocytic sarcoma and histiocytic lymphoma is discussed. The pertinent literature is reviewed.
Clones with 5q—and Inversion of Chromosome 7 in PHA Stimulated Peripheral Blood
Authors: Leeksma C. H. W. , Nijs J. Den , Havik F. , Kerkhofs H. , Geraedts J. P. M.
Published: August 2010

In a patient with autoimmune thrombocytopenic purpura, 2 distinct clonal abnormalities, deletion 5q and inversion 7, could be demonstrated in phytohaemagglutin stimulated peripheral blood. A large number of bone marrow mitosis was normal while unstimulated peripheral blood revealed no metaphases. The mutational events leading to the aberrations might have taken place in lymphoid precursor cells or in a pluripotential stem cell that lost the capacity to develop in the myeloid direction.