bluuebunny/crossref_metadata_2025 huggingface dataset:
Haematopoiesis in Busulphan‐Treated Mice: A Comparison between Two Different Stem Cell Assays
Authors: Josvasen Nicolai , Bøyum Arne
Published: August 2010

The effect of busulphan on haematopoiesis was investigated in mice. Bone marrow and blood cells were sampled 2 h to 55 d after a single treatment with busulphan. The effect on progenitor cells was examined with the diffusion chamber (DC) technique and the spleen colony assay.

Busulphan had a severe depressive effect on progenitor cells in bone marrow, and their number was reduced to 0.01–0.1 % of the control value on day 5–6. During the first 2 d the CFUs were reduced more than granulocyte progenitors as determined in the DC system. Histological examination of spleen colonies showed that in the same interval erythroid colony number was more depressed than granuloid colony number. This situation reversed after 4–6 d.

Our interpretation is that busulphan affects all types of progenitor cells irrespective of their proliferative state. However, multipotent CFUs in Go‐phase are more depressed than granulocyte progenitor cells (DC), which have a higher proliferative rate. The selective effect on erythroid colonies during the initial period might indicate that busulphan impairs the ability of multipotent CFUs to erythroid differentiation. This damage was repaired within a few days.

An abortive regeneration of bone marrow cells was observed on day 7–10. This can best be explained by an inflow from a cell pool intermediate between stem cells and early precursor cells, with some degree of ‘stem‐cellness' (self‐sustaining capacity).
Comparative Studies on the in vitro Uptake of 3H‐Cytidine and 3H‐Uridine by Normal and Leukaemic Lymphocytes
Authors: Bremer K. , Schreml W. , Harriss E. B.
Published: August 2010

In vitro labelling of lymphocytes by RNA precursors is a useful tool to study the characteristics of normal and leukaemic cell populations. Basic conditions have been studied for in vitro labelling and indicated a desired level of cell concentration (2 × 104 to 2 × 105) and temperature (37° C).

Normal leukocytes showed a fairly constant behaviour with respect to total uptake of radioactivity, incorporation into intracellular fractions and the ratio of 3H‐cytidine to 3H‐uridine incorporation (average ratio 10 ± 1). In contrast, the blood lymphocytes of patients with chronic lymphocytic leukaemia (CLL) exhibited a great variation in the parameters studied, ranging from below normal to several times normal values. This broad spectrum of in vitro characteristics has to be taken into consideration when populations of CLL lymphocytes are compared to normal lymphocytes and to those of other lymphatic disorders.
Electrophoresis of Blood Platelets and Erythrocytes: A Study in Healthy Persons and Patients with Haematological Diseases, Prosthetic Heart Valves, Virus Infections and Hyperlipaemia
Authors: Grøttum Kjell A.
Published: August 2010

The mean electrophoretic mobility of normal human blood platelets in 301 samples from 260 healthy individuals was 1.08 μm/s/V/cm in citrated plasma and 0.84 and 1.05 μm/s/V/cm in 0.145 M saline (pH 7.3) and in sucrose/phosphate/saline buffer (pH 6.4), respectively. Normal red cell mobility in citrated plasma in 229 samples from 195 persons was 1.41 μm/s/V/cm, in 0.145 M saline and 0.0667 M phosphate buffer (pH 7.3) 1.09 and 1.28 μm/s/V/cm, respectively.

The results of blood platelet and red cell electrophoresis in 90 patients with various diseases are reported. Severe abnormalities in platelet mobility were not found in any of the patients. In patients with von Willebrand's disease a slight increase in platelet mobility was observed. In 4 patients with haemolytic‐uraemic syndrome and 2 patients with microangiopathic haemolytic anaemia a reduced red cell mobility in citrated plasma and buffers was found.

In a case of red cell polyagglutinability, two populations of erythrocytes were observed electrophoretically. About half of the red cells had a mobility of about 43 % of normal in citrated plasma.
The Activation of Fetal Lymphocytes
Authors: Weber Theodor H. , Santesson Berta , Skoog Valdemar T.
Published: August 2010

Fetal lymphocytes from the cord blood of normal full‐term fetuses can be stimulated in vitro by PHA to transformation and mitotic division. The reaction kinetics differ from those of adult cells, in that the minimum G1 period is about 4 h shorter. However, the cord blood also contains a small number of lymphocytes, which enter the S period very soon after the addition of PHA, i.e. they have been prestimulated in vivo. These cells are completely lacking in adult blood. In addition, fetal lymphocytes show a significantly higher spontaneous transformation ratio. Possible reasons for these differences are discussed.
A Case of Acute Myelomonocytic Leukaemia Associated with Myelomatosis
Authors: Parker A. C.
Published: August 2010

A 79‐year‐old man with acute myelo‐monocytic leukaemia was found to have an IgG paraprotein on serum electrophoresis and a moderate increase in plasma cells (15 %) in the initial marrow examination. Treatment of the acute leukaemia with cytosine arabinoside and daunorubicin was followed by an increase in plasma cells in the marrow to 40 %. Small osteolytic lesions were found at autopsy in the thoracic and lumbar vertebrae. The relationship between myelomatosis and acute leukaemia is discussed.
Erythrocyte δ‐Aminolaevulinic Acid Dehydratase in Homozygous b‐Thalassaemia
Authors: Lyberatos C. , Mitsiou Ch. , Philippidou Aik. , Papayannis A. G. , Chalevelakis G. , Gardikas C.
Published: August 2010

Erythrocyte δ‐ALA dehydratase activity was quantitatively measured in 33 normal subjects and 50 patients with homozygous b‐thalassaemia. It was found that, a) In thalassaemic erythrocytes the activity of this enzyme was usually higher than in the normals, the difference of the mean values being statistically highly significant (P < 0.0005). b) This increment of the enzyme activity was not related only to the increased number of immature erythrocytes in thalassaemic samples since it persists after their elimination.
Trisomy G‐21 in Adult Myelomonocytic Leukaemia An Abnormality Common to Granulocytic and Monocytic Cells
Authors: Brandt L. , Levan G. , Mitelman F. , Olson I. , Sjögren U.
Published: August 2010

Chromosome analysis with fluorescent and Giemsa staining revealed trisomy G‐21 in almost 100 % of the bone marrow cells in a case of adult acute myelomonocytic leukaemia with equal proportions of granulocytic and monocytic cells. The results indicate a common precursor cell for the two cell series.
Pseudotumour of Bone in a Haemophiliac with Circulating Antibodies to Factor VIII Recovery after Rupture of Cyst Wall without Necrosis or Perforation of the Skin
Authors: Stavem P. , Egeberg O. , Kolmannskog F. , Nökleby K.
Published: August 2010

A 15‐year‐old boy with severe haemophilia A and circulating antibodies to factor VIII, developed a pseudotumour with osteolysis of the tibia. He made a complete recovery following what seemed to be a spontaneous inward rupture of the cyst wall.
Red‐Cell Carbonic Anhydrase Isoenzymes in Megaloblastic Anaemia
Authors: Dunbar Ann P. , Tudhope G. R.
Published: August 2010

Erythrocyte carbonic anhydrase isoenzymes B and C have been measured by means of electrophoresis on cellulose acetate membrane strips followed by staining, elution and spectrophotometry. The results from 30 normal subjects showed for carbonic anhydrase B a mean and standard deviation of 12.1 ± 1.4 mg/g Hb, and for carbonic anhydrase C, 3.9 ± 0.9 mg/g Hb.

Both isoenzymes were markedly increased in all 10 cases of untreated megaloblastic anaemia, the mean ± SD for carbonic anhydrase B being 24.8 ± 7.6 mglg Hb and for the isoenzyme C, 7.7±k 1.2 mg/g Hb. These results are both highly significantly greater than the normal means (P < 0.001). The elevated levels of both isoenzymes returned to the normal range within 2–4 weeks of starting vitamin B12 therapy in 3 patients with pernicious anaemia in whom serial enzyme measurements were made. In a group of 13 patients with pernicious anaemia, who had been adequately treated with vitamin BIZ for 1–15 years, the mean levels of isoenzymes B and C were 10.3 ± 0.6 and 2.9 ± 0.7 mg/g Hb respectively; both these mean results are significantly less than the corresponding normal means (P < 0.001).
Acute Leukaemia Associated with an Abnormal Genotype
Authors: Garson O. Margaret , Milligan Wendy J.
Published: August 2010

A young female presenting with lymphoblastic leukaemia was found to have an abnormal genotype due to a reciprocal translocation between chromosomes B and D. The father and twin sisters of the propositus were also found to possess the trans‐location. Neither the propositus nor the other members of the family possessing the abnormal karyotype showed any phenotypic abnormality.

Giemsa banding identified the chromosomes involved in the translocation as B4 and D14.

Additional chromosome abnormalities were present in the leukaemic cells and these disappeared with therapy and reappeared in relapse.

It is suggested that genotypic reciprocal translocation may be yet another cytogenetic abnormality associated with a special tendency to the development of acute leukaemia.
Study on the Fibrillar Formation Surrounding the Nuclear Bridge in Some Types of Leukaemic Cells
Authors: Ito S. , Hatiom A.
Published: August 2010

Rieder cells which had a characteristically lobulated nucleus with a deep cleft were studied by both conventional and ultra‐high voltage electron microscopes in one case of acute granulocytic leukaemia and two cases of acute promyelocytic leukaemia. Light microscopically a few leukaemic cells in these cases possessed two nuclei. It was demonstrated that a bundle of microfilaments tightly surrounded the nuclear bridge of the Rieder cell. This finding seemed to be strikingly similar to that of the amphibian amitotic cell division observed by Pehlemann although origination of a bundle of microfilaments from the centriole and a process of cell division following the amitotic nuclear division could not be confirmed. Considering the possible contractile property of the microfilaments described hitherto in other types of cells, it is postulated that the Rieder cell in these types of leukaemia was produced by a process of abortive amitosis caused by the contraction of the microfilaments.
Haemoglobin M Saskatoon and Haemoglobin M Hyde Park in Two Yugoslavian Families
Authors: Efremov G. D. , Huisman T. H. J. , Stanulovic M. , Zurovec M. , Duma H. , B.Wilson J. , Jeremic V.
Published: August 2010

A methaemoglobin variant, found in a Yugoslavian family and termed previously Hb‐M‐Novi Sad, has been identified as Hb‐M‐Saskatoon (α2β2G3(E7)Tyr). 4 members of a second Yugoslavian family also had Hb‐M disease but chemical analyses of this variant showed it was the same as Hb‐M‐Hyde Park (α2β292(F8)Trr). Subjects with either type of Hb‐M show signs of a mild haernolytic anaemia, and inclusion bodies were found in subjects with Hb‐M‐Saskatoon.

Hb‐M‐Saskatoon cannot be separated from Hb‐A by electrophoresis at alkaline pH whereas Hb‐M‐Hyde Park has a mobility slightly different from that of Hb‐Az. Both variants can be isolated by anion exchange chromatography, and dissociate readily into dimers at pH 10.1.

The haeme‐globin ratio of both Hb‐M variants is decreased. Isolated Hb‐M‐Saskatoon and Hb‐M‐Hyde Park contained about 30 to 40 % less haeme.
The Effect of Heparin on Plasma and Platelet Lipids During Alimentary Hyperlipaemia in Man
Authors: Nordøy Arne , Strøm Ellen , Berntsen Harald
Published: August 2010

Alimentary hyperlipaemia was induced in 19 healthy male subjects by ingestion of a standardized fatty meal composed mainly of saturated or unsaturated fatty acids. During alimentary hyperlipaemia, heparin was given intravenously. A marked increase of plasma FFA with a concomitant decrease of plasma triglycerides was observed after heparin. The fatty acids of the high plasma FFA fraction reflected the dietary fatty acids. The plasma lysolecithin/lecithin ratio showed a significant increase when heparin was given after the unsaturated fatty meal.

The platelet number was unchanged after heparin. The platelet FFA fraction showed a moderate but significant increase after the saturated fatty meal, whereas no such increase was seen after the unsaturated fatty meal. However, contrary to the condition in the fasting state, no correlation could be established between the plasma and platelet FFA after heparin. It is suggested that heparin or changes induced by heparin, such as a change in the lysolecithidlecithin ratio, may be responsible for the lack of influence of high plasma FFA on platelet number and platelet lipids.
Isolation and Characterization of an in vivo Thrombin‐Induced Anticoagulant Activity
Authors: Hyde Elisabeth , Wetmore Robert , Gurewich Victor
Published: August 2010

An anticlotting activity present in rabbit blood following the administration of small amounts of thrombin is described. The substrate for this activity was found to be related to the vitamin‐K dependent clotting factors. The activity was labile in blood but relatively stable after it was isolated from plasma of thrombin treated animals. Oxy‐genation of blood was found to accelerate its inactivation. A method for the rapid chromatographic isolation of the activity from small amounts of plasma is described. The method is considered sufficiently practical to be utilized clinically. The anti‐clotting activity is considered similar to that which can be elaborated in vitro from thrombin activation of isolated prothrombin complex as previously described by others. A physiologic role for the activity is proposed.
Hypertonic Saline Induced Abortion as Pathophysiologic Model of Low Grade Intravascular Coagulation
Authors: Royen Eric A. van , Treffers Peter E. , Ute Jan W. ten
Published: August 2010

The reported occurrence of low grade DIC after hypertonic saline induced abortion (HSIA) was confirmed by a positive ethanol gelation test, a fall in factor VIII activity, fibrinogen level and platelet count and an increase in fibrinopeptide A and FDP. HSIA proved to be an excellent model for the investigation of low grade DIC on ground of the standardized clinical conditions. Several methods for the measurement of thrombin activated factors V and VIII did not provide more information than the ethanol gelation test, the estimation of factor VIII activity and the radioimmunoassay of fibrinopeptide A.

Contact activation of the maternal blood by an inflammatory reaction of the syncytiotrophoblast is proposed as being implicated in the observed changes of blood coagulation. A single dose of heparin prevented the formation of fibrin monomers, fibrinopeptide A and FDP.
Amoeboid Movement Configuration A Cell Configuration Observed in Tumour Cells from 3 Cases of Bone Marrow Neoplasia
Authors: Norberg B. , Rydgren L. , Stenstam M.
Published: August 2010

In smears from peripheral blood or from bone marrow of healthy probands, the nucleated cells usually lack the cell contour of locomotion, amoboid movement configuration (AMC).

The present study is concerned with two patients with acute myeloblastic leukaemia and one with generalized lymphmarcoma, in whom 40–50 % of the tumour cells seen in bone marrow smears displayed the AMC. The high incidence of tumour cells with AMC produced a characteristic picture in the bone marrow smears. The phenomenon of AMC in the mentioned tumour cells was subjected to morphological, microcinematographic and ultrastructural analysis. Most tumour cells with AMC were found to have an active lymphocyte‐like locomotion. Some tumour cells with AMC, however, displayed spasmodic cytoplasmic contractions for 10–15 min without displacement of the cell body. This picture of locomotory rest in locomotory configuration has not been observed in cells from healthy probands under comparable experimental conditions.

It is suggested that the phenomenon of amoeboid movement configuration might be produced by a direct disorder in the locomotion mechanism of the present tumour cells, or alternatively, in the regulative mechanisms of locomotion.
Hb D Punjab ‐ Alpha Thalassaemia Combination in a Turkish Family
Authors: Cavdar Ayhan O. , Arcasoy Ayten
Published: August 2010

Data are presented of a Turkish family in whom Hb D and alfa‐thalassaemia were present. Hb D was identified as Hb D Punjab by amino acid analysis. The propositus had a combination of Hb D Punjab and alfa‐thalassaemia disease and suffered from a rather severe anaemia, therefore, a possibility of hypersplenism in addition to hereditary haemoglobin defects was considered.
On the Fate of DNA Synthesizing Lymphoid Blood Cells in Hodgkin's Disease
Authors: Schick P. , Trepel F. , Begemann H.
Published: August 2010

Autotransfusions of the DNA synthesizing blood cells from 2‐2.5 liters of blood, labelled in vitro with 3H‐thymidine, were performed in 2 patients with Hodgkin's disease. The fate of the labelled lymphoid cells was followed up for 5 min to 60 h in the circulating blood and occasionally in the lymph node tissue. The data indicate that 1) circulating DNA synthesizing large lymphoid cells leave the blood in less than 2 h; 2) they produce by mitosis large and medium sized lymphocytes, which mainly appear in blood and lymph nodes and 3) their generation time, in agreement with other estimates, is about 25 h.
Needle‐Like Crystals in Plasma Cells in a Patient with a Plasma Cell Proliferative Disorder
Authors: Stavem Per , Vandvik Bodvar , Skrede Sverre , Hovig Torstein
Published: August 2010

A patient with a plasma cell proliferative disorder and a monoclonal IgG‐kappa protein in the serum was observed over a period of four years. Two unusual features were seen in this patient: (1) An apparently benign course in spite of relatively large and slowly increasing amounts of Bence Jones protein (monoclonal kappa light chains) in the urine, and (2) crystalline structures located outside the cisternae of the endoplasmic reticulum in a large number of bone marrow plasma cells. The crystals, which ultrastructurally appeared to be composed of protein material, were not stained by immunofluorescent antisera to immunoglobulins. They were, however, observed only in cells staining for gamma heavy and kappa light chains. This suggests an association between the crystalline structures and the synthesis of monoclonal immunoglobulin proteins.
Treatment of Therapy‐Resistant Acute Myeloid Leukaemia with 7 and 8 Cytostatics
Authors: Brincker Hans
Published: August 2010

16 courses of an 8‐drug regimen including daunomycin, vincristine, cytosinearabinoside, thioguanine, methotrexate, cyclophosphamide, prednisolone and hydroxyurea, and 12 courses of a 7‐drug regimen including the same drugs minus hydroxyurea were administered in 16 otherwise therapy‐resistant cases of acute myeloid leukaemia. In spite of a significant and rapid reduction of the leukaemic cell‐mass in all the cases treated, only two brief minimal remissions were obtained. The main toxic effect was myelosuppression. However, the treatment was associated with a high frequency of mucosal ulcerations of the oesophagus and stomach, and it cannot be excluded that the latter complication may be drug‐related.
Folic Acid Deficiency in Sickle Cell Anaemia
Authors: Liu Yong K.
Published: August 2010

Megaloblastic anaemia responding to folic acid has been reported in a number of patients with sickle cell anaemia. The incidence of reduced serum and erythrocyte folate levels in such patients remains unclear, however. Serum and erythrocyte folate contents were measured microbiologically in patients with sickle cell anaemia as well as in two control groups with sickle cell trait or normal haemoglobin pattern. Low serum and erythrocyte folate values were significantly more common in 61 sickle‐cell patients than in each of the two socio‐economically‐matched control groups including 61 subjects with Hb AS and 69 blacks with Hb AA. There was no correlation between serum folate and haematocrit or reticulocyte count. The erythrocyte folate, measured before and after removal of reticulocytes, remains a reliable indicator of deficiency of folic acid in sickle‐cell patients despite variable degrees of reticulocytes. Folate therapy resulted in higher haematocrits in three of four sickle‐cell patients with low serum and low erythrocyte folate values, but in only one of 12 patients with low serum folate and normal erythrocyte folate. These findings are consistent with other observations that folate requirement may be increased in chronic haemolytic disorders and suggest that erythrocyte folate values should be obtained in sickle‐cell patients suspected of having folate deficiency.
The Effect of Vitamin E on Haemolysis in Paroxysmal Nocturnal Haemoglobinuria: In vitro and in vivo Studies
Authors: Gomperts E. D. , Zail S. S. , Christensen Diane , Metz J.
Published: August 2010

The administration of vitamin E, a natural antioxidant, to a patient with paroxysmal nocturnal haemoglobinuria (PNH) failed to diminish the urinary excretion of 59Fe as monitored by 59Fe whole body counting and urinary loss of isotope. However, in vitro vitamin E corrected the increased sensitivity of PNH red cells to haemolysis by hydrogen peroxide. These results support the concept that the susceptibility of PNH red cells to lipid peroxidation is an in vitro phenomenon bearing little relation to the mechanism of in vivo haemolysis.
Vitamin B12‐Binding Proteins of R‐Type, Cobalophilin
Authors: Stenman Ulf‐Håkan
Published: August 2010

A gradient was developed for isoelectric focusing in the pH range 2–5. Cobalophilin (earlier called R proteins or vitamin B12‐binding proteins of R‐type) was isolated from saliva and amniotic fluid in homogeneous form. It was found to be a glycoprotein with a molecular weight of 59,300–69,100. The preparation from amniotic fluid contained 33% carbohydrate.

Cobalophilin variants in plasma, serum, granulocytes, platelets, amniotic fluid, milk, saliva and gastric juice were characterized by isoelectric focusing. Most fluids and cells contained the same isoproteins, with pI values between 2.3 and 5.0. Isoproteins of presumably myelogenic origin (e.g. those in granulocytes and plasma) had pI values below 4.2, whereas those of glandular origin (in milk and saliva) had a pI range of 4.0–5.0.

Serum contained more cobalophilin than plasma, owing to release of this protein from granulocytes during clotting. This phenomenon also changed the isoprotein pattern. Plasma and serum from newborn infants and from patients with leucocytosis, polycythaemia vera and chronic myelogenous leukaemia contained the same isoproteins as were found in plasma from healthy subjects. In addition to these, isoproteins with lower than ‘normal’ pI values were often found in chronic myelogenous leukaemia and occasionally in leucocytosis.

It is concluded that cobalophilin from different fluids and cells is a single microheterogeneous protein with a variable carbohydrate composition. The distribution of cobalophilin in different body fluids and cells supports the suggestion that cobalophilin is an antimicrobial protein (Gullberg 1972) like lactoferrin and lysozyme.
A New Case of Heavy μ‐Chain Disease
Authors: Danon F. , Mihaesco C. , Bouvry M. , Clerc M. , Seligmann M.
Published: August 2010

The tenth case of μ chain disease is described. The patient lives in Ivory Coast as in our previously reported case. He was not affected with chronic lymphocytic leukaemia and the main clinical feature was liver cirrhosis of unknown origin. The amount of abnormal protein in the serum was great enough to give an abnormal bond on the routine electrophoresis. The protein was devoid of light chains and was present in the form of disulfide linked polymers of incomplete μ chain. The molecular weight of the monomer was approximately 58,000. The protein comprised the Fc fragment and a part of the Fd segment. Bence Jones protein was not found in the urine.
Lymphocyte ATP‐ase Activity in Treated Cases of Hodgkin's Disease
Authors: Ellegaard Jørgen , Traunberg Henrik , Esmann Viggo
Published: August 2010

The ATP‐ase activity was determined in lymphocytes isolated from peripheral blood in 41 patients with Hodgkin's disease and 50 healthy controls. All patients were previously treated with irradiation or cytostatic drugs and 17 patients were under maintenance therapy at the time of investigation. A significantly increased ATP‐ase activity was found in lymphocytes from patients with Hodgkin's disease. The individual activities were unrelated to the clinical stage of the disease, but correlated to the histological classification of the lymphatic tissue. Significantly lower lymphocyte ATP‐ase activity was found in patients under maintenance treatment with immunosuppressive drugs, especially if the patients had previously been irradiated. It is suggested that the ATP‐ase activity of circulating lymphocytes is related to the immunological activity against the presence of the malignant cells in Hodgkin's disease.
ADP‐Induced Refractory State of Platelets in vitro
Authors: Holme Stein , Holmsen Holm
Published: August 2010

ADP‐induced aggregation was determined at various times of incubation with ADP in unstirred human platelet rich plasma (PRP) to which adenosine deaminase was added. In the early stages of incubation the shape change response was absent, the aggregation response was poor, and not reversible. These three parameters returned slowly towards normal as the incubation proceeded. Pronounced impairment of the aggregation response was present after all added ADP had been degraded in plasma. Log dose‐response (rate of aggregation) curves for the platelets incubated with ADP had shifted to higher ADP concentrations and had also a small decrease in maximal height and in the slope compared to those obtained with control platelets. However, the shift in log dose response to higher ADP concentrations was far more striking, so the increase in log dose (R) necessary to obtain the same rate of aggregation as with control PRP was taken as a measure of refractoriness of aggregation towards ADP. R increased with the time of incubation to an optimal value and then decreased. The magnitude of the optimal value and the time at which optimum was reached increased with the concentration of the ADP incubated with the platelets. The variations in R during incubation did not correlate with the breakdown of added ADP. The platelets began to recover their ability to aggregate with ADP while there was still ADP in the system.
Thymidine Kinase Activity in Human Bone Marrow Cells
Authors: Wickramasinghe S. N. , Olsen I. , Saunders J. E.
Published: August 2010

The thymidine kinase activity per 106 DNA‐synthesising marrow cells and the rate of incorporation of tritiated thymidine into the DNA of 103 DNA‐synthesising marrow cells were estimated in 9 haematologically normal patients and 49 patients suffering from a variety of haematological disorders. Slight increases in thymidine kinase activity were found in 6 of the 31 patients with haematological diseases associated with normoblastic erythropoiesis and greater increases were found in 3 of the 18 patients with megaloblastic haemopoiesis due to vitamin B12 or folate deficiency. In the latter group, there was a statistically significant inverse correlation between haemoglobin levels and thymidine kinase activity. No correlation was found between thymidine kinase activity and the rate of incorporation of tritiated thymidine in either the normoblastic or megaloblastic group, suggesting that the level of thymidine kinase activity does not limit the rate of incorporation of exogenously supplied thymidine into the DNA of human bone marrow cells.
Pseudothrombocytopenia Due to Platelet Aggregation and Degranulation in Blood Collected in EDTA
Authors: Mant M. J. , Doery J. C. G. , Gauldie J. , Sims H.
Published: August 2010

Three patients are described in whom platelet aggregation and/or degranulation occurred in blood collected into EDTA. These changes resulted in spurious thrombocytopenia and morphological changes similar to those observed in some thrombocytopathies. The abnormalities were dependent on the presence of EDTA and did not occur in citrate, oxalate or heparin anticoagulants. In two patients the abnormality was shown to be due to a plasma factor which was not IgG, IgM, fibrinogen or albumin. The most likely explanation is that these patients have an unidentified abnormal plasma component which, on exposure to EDTA, develops ‘anti‐platelet activity’. Although relatively uncommon a prospective study of the incidence of these phenomena indicates that they are probably more common than either platelet cold agglutinins or platelet satellitism. They have practical significance with respect to the diagnosis of thrombocytopenia and also to the interpretation of abnormal platelet morphology.
Karyotypes in Infectious Mononucleosis
Authors: Philip P. , Ernst P. , Wantzin G. Lange
Published: August 2010

Using a trypsin‐Giemsa banding procedure, chromosome analysis was performed on blood from 21 consecutive patients hospitalized for infectious mononucleosis. Mitoses were harvested after 2 and 24 h in vitro incubation without PHA and after 48 h with PHA. No abnormalities were seen.
Cell Production and Cell Function in Human Cyclic Neutropenia
Authors: Brandt L. , Forssman O. , Mitelman F. , Odeberg H. , Olofsson T. , Olsson I. , Svensson B.
Published: August 2010

In vitro studies have been done on haematopoietic cells from a patient with cyclic neutropenia characterized by severe depression of blood neutrophil levels every 21 days. Serial blood counts reveal periodic fluctuations in neutrophils, monocytes and reticulocytes. Agar culture of marrow cells shows normal concentration of colony forming cells. The percentage of colony forming cells in S phase is highly increased during profound neutropenia and normal during the recovery phase relating the granulocyte production to the peripheral neutrophil level. Studies of ingestion rate, bactericidal activity, lactate production and glucose oxidation during phagocytosis in isolated granulocytes show normal results. Also the ingestion rate in isolated monocytes is normal. Serial karyotype analyses of marrow cells during the neutrophil cycle display a normal pattern. Serum myeloperoxidase levels vary inversely with the peripheral neutrophil count indicating increased granulopoietic activity during profound neutropenia, which might be associated with non effective granulopoiesis during profound neutropenia, leading to a lack of granulocyte reserves in the marrow.
Monosomy 7 in Two Adult Patients with Acute Myeloblastic Leukaemia
Authors: Zech Lore , Lindsten Jan , Udén Ann‐Mari , Gahrton Gösta
Published: August 2010

Two patients with acute myeloblastic leukaemia were found to have a population of bone marrow cells with C‐group monosomy. Using the Quinacrine Mustard fluorescence technique the lacking chromosome was in both patients identified as a number 7. Both patients entered a complete remission. Monosomy 7 may indicate a separate entity of acute myeloblastic leukaemia associated with a comparatively favourable prognosis.
Congenital Stomatocytosis and Chronic Haemolytic Anaemia
Authors: Bienzle U. , Niethammer D. , Kleeberg U. , Ungefehr K. , Kohne E. , Kleihauer E.
Published: August 2010

A new case of congenital stomatocytosis associated with haemolytic anaemia, increased autohaemolysis, abnormalities in the erythrocyte metabolism, increased osmotic fragility and shortened erythrocyte survival is described. Intracellular cation concentrations are abnormal: Red cell sodium is high, and potassium is low. The pump rate for monovalent cations is increased.
A New Variant of Erythrocyte Pyruvate Kinase – PK ‘Maebashi’
Authors: Kubota Kazuo , Moteki Masaki , Omine Mitsuhiro , Tsuchiya Jun , Maekawa Tadashi , Miwa Shiro
Published: August 2010

A new case of erythrocyte pyruvate kinase deficiency was described. – A 9‐year‐old male patient was hospitalized because of anaemia, jaundice and splenomegaly. Diagnosis was made primarily on the basis of the erythrocyte enzyme studies. Because the pyruvate kinase of this patient demonstrated certain different characteristics from the other variants described previously, it was tentatively designated PK ‘Maebashi’.
G Banding Analysis of Complex Aneuploidy in a Case of Erythroleukaemia
Authors: Philip Preben
Published: August 2010

The chromosomal abnormalities in bone marrow cells from a patient with erythroleukaemia are reported. The complicated pattern of aneuploidy includes 5 marker chromosomes originating from rcp (1; 3), t (8; 11), t (X; X) and del (7).
A Case of Gamma Heavy Chain Disease Associated with Autoimmune Haemolytic Anaemia: Clinical, Haematological, Immunological and Pathological Details
Authors: Shirakura T. , Kobayashi Y. , Murai Y. , Inoue T. , Imamura Y.
Published: August 2010

A 76‐year‐old Japanese man, presented with a case of gamma heavy chain disease associated with autoimmune haemolytic anaemia. The clinical course progressed rapidly, accompanied by fever, lymphadenopathy, erythematous palate and hepatosplenomegaly. As the disease progressed, haemoglobin level gradually reduced, and jaundice occurred. A complication of autoimmune haemolytic anaemia was deduced from the laboratory data obtained at this time. The patient's serum and urine contained an M‐component with electrophoretic and antigenic properties resembling those of the Fc‐fragment of G1 globulin. Microscopic and electron microscopic findings on the tumor cells infiltrating in lymphnodes indicated all types of transitional forms from small lymphocytes to large lymphoplasmoblastic cells.
Circulating Micromegakaryocytes Signaling Blast Transformation of Chronic Myeloid Leukaemia
Authors: Udomratn T. , Steinberg M. H. , Dreiling B. J. , Lockhard V.
Published: August 2010

A patient with chronic myeloid leukaemia had numerous micromegakaryocytes in the peripheral blood and bone marrow appearing coincidentally with the onset of blast crisis. These cells were initially confused with lymphocytes because of their size, configuration and scanty cytoplasm. The true identification of these cells can be suspected by careful scrutiny of well prepared Wrights stained preparations and proven electronmicroscopically. Such marked dysplasia of the megakaryocyte series appears to be a poor prognostic sign in chronic myeloid leukaemia.
Non‐Random and Random Chromosomal Abnormalities in Transformed Chronic Granulocytic Leukaemia
Authors: Sharp J. C. , Potter A. M. , Wood J. Keith
Published: August 2010

Chromosome abnormalities, identified using a banding technique and additional to the Ph, are reported in 10 consecutive cases of transformed chronic granulocytic leukaemia. In most of the cases the abnormalities were non random. In 2 cases serial studies were performed and additional abnormalities found, antedating transformation by one week and two months respectively. In 2 others the Ph status had been established during the chronic phase of the disease. In the remaining cases the first chromosome analysis was performed at the time of transformation.
Granulopoiesis in Infantile Genetic Agranulocytosis In vitro Cloning of Marrow Cells in Agar Culture
Authors: Olofsson Tor , Olsson Inge , Kostmann Rolf , Malmström Stig , Thilen Astrid
Published: August 2010

A 3‐year‐old boy and a 2‐year‐old girl with infantile genetic agranulocytosis have been studied by in vitro cloning of bone marrow cells in agar culture. The patients display a normal concentration of colony forming cells and the morphological maturation is identical with that of control marrow cultured in vitro. The marrow cells of the patients show some degree of auto‐stimulation indicating that endogenous production of colony stimulating factor is operating. As an inverse relationship is expected between the peripheral neutrophil count and the percentage of marrow colony forming cells in S‐phase a high percentage was expected. On the contrary, we find that the percentage of colony forming cells in S‐phase is extremely low indicating a genetic unresponsiveness of granulopoietic precursor cells to feed back regulation in infantile genetic agranulocytosis.
Effect of Azathioprine on the Cell Cycle of Haemopoietic Cells
Authors: Wickramasinghe S. N.
Published: August 2010

The megaloblastic erythropoiesis encountered in 3 azathioprine‐treated patients with renal transplants was found to be associated with a marked increase in the proportion of early polychromatic erythroblasts in the Gz phase of the cell cycle. By contrast, there was only a slight increase in the proportion of Gz cells in the neutrophil promyelocyte‐myelocyte pool of these patients.
Evaluation of Prophylactic Replacement Therapy in Haemophilia B
Authors: Morfini M. , Mannucci P. M. , Mariani G. , Pandelli F. , Petrucci F. , Baicchi U. , Capitanio Anna , Ferrini P. L. Rossi , Mandelli F.
Published: August 2010

Prophylaxis replacement therapy has been assessed for a period of 12 months in 10 patients with severe haemophilia B showing a high incidence of spontaneous bleeding episodes. Two different schedules of administration of a freeze‐dried factor IX concentrate were randomly evaluated: according to scheme A, 7.5 U/kg were administrated biweekly, whereas scheme B was based on the weekly infusion of 15 U/kg. On prophylaxis the frequency of bleeding episodes was significantly reduced (P < 0.005) when compared with that observed in the one‐year period preceding the trial.

Biweekly infusions were superior to weekly infusion (P < 0.01), and the benefit appeared to be related to the higher number of days in which measurable levels of factor IX were attained in plasma.

Range of motion, which was reduced at the start of the trial in 26 joints, was found to have improved in 23. Favourable changes of the joint radiological picture were observed in 6 cases. Hepatitis and factor IX inhibitors did not develop during the trial period. Side effects were rare and mild.
Hormonal Control of Erythropoiesis in Canine Cyclic Haematopoiesis
Authors: Lange R. D. , Jones J. B.
Published: August 2010

The results of erythropoietin (ESF) studies in dogs with cyclic haematopoiesis are presented. Even though the dogs were exposed to a constant stimulus of hypoxia, cycles in the plasma ESF levels occurred at 11 to 12 day intervals. In some dogs, minor midcycle peaks were observed and the amount of ESF produced varied with the different animals. The peaks of ESF characteristically appeared approximately five days after the onset of neutropenia. A hypothesis is presented to explain the known facts concerning canine cyclic haematopoiesis. It suggests that a poietin controlling factor is produced and that this assumed factor then stimulates the production of specific factors leading to increases of reticulocytes, platelets, and monocytes. The monocytes in turn produce more colony stimulating factor (CSF) leading to the formation of granulocytes. Such a sequence of events would explain the apogee of reticulocyte, platelet, and monocyte counts at a time when the nadir of granulocyte counts is reached.
Diffuse Lymphosarcomatous Infiltration in the Central Nervous System
Authors: Hansen Mogens Mørk , Baadsgaard Bjarne , Videbæk Aage
Published: August 2010

Massive lymphosarcomatous infiltration, confirmed at autopsy, occurred in a 46‐year‐old man with neurological signs rapidly progressing to total paralysis of peripheral as well as cranial nerves. These signs set in at a time when the malignant systemic disease seemed to be otherwise in remission, and indeed they gave rise to great differential diagnostic difficulties.
An Individual with Hb‐Lepore‐Baltimore‐ δβ‐Thalassaemia in a Yugoslavian Family
Authors: Efremov G. D. , Rudivić R. , Niazi G. A. , Hunter E. , Huisman T. H. J. , Schroeder W. A.
Published: August 2010

The clinical, haematological and biochemical findings in a person with δβ‐thalassaemia and Hb‐Lepore are described. The patient was a 24‐year‐old student who suffered from anaemia of intermediate seventy with late onset of the clinical manifestations, had minor bone and facial deformities, but had no necessity for regular transfusions. Haemoglobins A and A2 were absent in this individual, and the Hb‐Lepore has been identified as Lepore‐Baltimore. Heterogeneity of γ chain of the Hb‐F follows the expected pattern. The study provides further evidence that neither β nor δ chains are synthesized in cis to δβ‐thalassaemia or Hb‐Lepore.
Correlations between the Deoxyuridine‐Suppressed Value and Some Conventional Haematological Parameters in Patients with Folate or Vitamin B12 Deficiency
Authors: Wickramasinghe S. N. , Saunders J.
Published: August 2010

The correlations between the deoxyuridine‐suppressed value and some conventional haematological parameters (Hb concentration, red cell count, MCV, total white cell count, red cell folate level and serum vitamin B12 level) have been investigated in 40 patients with megaloblastic haemopoiesis due to folate or vitamin B12 deficiency. The only statistically significant correlations found were (a) an inverse correlation between the deoxyuridine‐suppressed value and the Hb level or red cell count in both the folate‐ and vitamin B12‐deficient groups and (b) a direct correlation between the deoxyuridine‐suppressed value and MCV in the vitamin B12‐deficient group. The results indicated that the deoxyuridine‐suppressed value was a better index of the magnitude of the disturbance in red cell production induced by folate or vitamin B12 deficiency than either the red cell folate or serum vitamin B12 levels.
Relapsing Haemolytic Anaemia of Pregnancy with Negative Antiglobulin Reaction
Authors: Hershko C. , Berrebi A. , Resnitzky P. , Eldor A.
Published: August 2010

Four patients are described in whom seven episodes of haemolytic anaemia associated with pregnancy were observed during a follow‐up period of 2 to 16 years. Normal haemoglobin levels were found both before and after pregnancy. Despite consistently negative antiglobulin tests, an autoimmune haemolytic mechanism is suggested by the rapid destruction of transfused blood, favourable response to corticosteroids and the development of transient haemolytic anaemia in three of the infants. In view of its unique clinical and laboratory features and relatively mild course, recognition of this condition as a distinct clinical entitity seems to be justified.
Rarity of Anaemia of Pregnancy in Ethiopia
Authors: Gebre‐Medhin M. , Killander A. , Vahlquist B. , Wuhib E.
Published: August 2010

Hb and PCV values were studied in three groups of Addis Ababa women during their last month of pregnancy and compared with those in a control group of non‐pregnant Addis Ababa women. Irrespective of socio‐economic group and parity, the Hb and PCV values were almost identical in all four groups.

In one of the three test groups, a group of ‘non‐privileged’ multiparae, S‐iron, S‐transferrin (TIBC), S‐folate and S‐vitamin B12 were also examined and showed normal values.

These findings indicate a rarity not only of true anaemia but also of ‘physiological anaemia of pregnancy’. This latter may not, therefore, be an adaptive phenomenon of regular occurrence as has usually been assumed hitherto. The explanation for the aberrant haematological behaviour in pregnant women living in highland Ethiopia is probably the lifelong exposure to a very high iron intake combined with hypoxia due to the high altitude.
Acquired Thrombophilia: A Hitherto Unrecognized Procoagulant‐Antithrombin Imbalance
Authors: Innerfield Irving
Published: August 2010

Inherited, acquired and iatrogenic ‘diseases of low antithrombin activity’ share a common denominator: each is associated with an increased tendency toward, or incidence of, thrombosis. This suggested the possibility that these diseases constituted a distinct pathogenetic entity.

Molecular models and clinical studies suggested 3 interrelated phenomena: increased serum procoagulants; decreased antithrombin; a tendency to thrombosis. The term ‘Acquired Thrombophilia’ is proposed to designate pre‐thrombotic and thrombotic individuals presenting this non‐inherited combination of laboratory and clinical findings.

The antithrombin clotting time is shown to be inversely related to the level of circulating serine procoagulants, directly related to the level of antithrombin, and results from a procoagulant‐antithrombin inter‐molecular reaction. Patients with Acquired Thrombophilia have ‘turned‐on’ or excessive procoagulants resulting in ‘turned‐off’ or tightly bound antithrombin molecules.
μ‐Chain Disease in a Case of Chronic Lymphocytic Leukaemia and Malignant Histiocytoma
Authors: Jønsson Viggo , Videbæk Aage , Axelsen Nils H. , Harboe Morten
Published: August 2010

A 27‐year‐old woman with proteinuria and normal serum creatinine for 10 years developed chronic lymphocytic leukaemia (CLL) and after further 2 years during treatment with prednisone and chlorambucil a malignant histiocytoma appeared. Free μ‐chains were demonstrated in serum, but it is possible that μ‐chain disease (μ‐CD) and Bence‐Jones proteinuria had persisted unrecognized for 12 years, since vacuolated plasma cells were found when the primary bone marrow smear was re‐evaluated and no glomerular disorders were found. The clinical findings from the now 11 known cases of μ‐CD are reveiwed and the immunochemical findings are reported in part II (Axelsen et al 1976).

The patient's mother died of acute leukaemia at the age of 24. The mother's sister has mammary carcinoma and several members of the mother's line apparently have increased tendency to infections. Of 27 family members, none exhibited immunoglobulin defects, but 10 members of the mother's line had B‐ as well as T‐lymphocytosis and 5 members had leukocytosis.
Thrombocytopenia in Childhood: An Evaluation of 433 Patients
Authors: Cohn Jørgen
Published: August 2010

An evaluation of 433 children with thrombocytopenia is presented. The material comprises all cases diagnosed in Denmark during the period 1959–1969. The incidence was found to be 31.9 cases per 1,000,000 inhabitants 15 years of age per annum with a peak at the age of 3 to 4 years; the majority of the cases was diagnosed in the spring. Preceding infection, usually associated with fever and localized in the upper respiratory tract, was the most common cause. The majority of the congenital cases was hereditary and associated with other malformations involving in particular the immune system. In about half of the cases the course was spontaneous and no treatment was necessary; the remaining patients were treated with glucocorticoids, including 46 patients, who had undergone splenectomy. 22 patients died, including 16 cases due to serious underlying disorders; in 6 cases the cause of death was haemorrhage (mortality rate = 1.4%). At the follow up the platelet count was below 50 mia/1 in 14 patients (4%). No difference was found concerning the platelet count at the follow up between the patients with spontaneous recovery, the patients treated with glucocorticoids and the patients, in whom splenectomy had been performed. During the course the disease autoimmune haemolytic anaemia occurred in 2 patients, whereas no patient developed systemic lupus erythematosus.
Chronic Haemolytic Anaemia in Two Patients Heterozygous for Erythrocyte Pyruvate Kinase Deficiency
Authors: KAHN AXEL , MARIE JOELLE , GALAND COLETTE , BOIVIN PIERRE
Published: August 2010

Two patients with mild chronic haemolytic anaemia, a mother and her son, were found to be heterozygous for erythrocyte pyruvate kinase deficiency.

In the red blood cells the enzymatic activity was reduced by about 50 % and the residual PK had normal kinetic properties, stability and electrofocusing pattern. The PK antigen concentration was also decreased by half, so that the ratio of the enzymatic activity to the immunological reactivity (i.e. the molecular specific activity) was normal.

In the son's liver PK enzymatic activity was slightly reduced and, above all, an abnormal active form, more anodic than normal PK, was detected by electrofocusing. The propositus's liver PK was also slightly thermo‐unstable.

It is suggested that the patients were heterozygous for an unstable PK variant which is found in liver, nucleated tissue actively synthesizing proteins, but which disappeared from the erythrocytes because of its unstability.