bluuebunny/crossref_metadata_2025 huggingface dataset:
Studies on Human Preleukaemia
Authors: Hast Robert
Published: August 2010

The prognostic significance of the bone marrow sideroblasts found in aregenerative anaemia with hypercellular bone marrow was evaluated particularly to see if the sideroblasts might be a preleukaemic sign.

The study includes 32 patients with abnormal sideroblasts; 14 had mainly ringed sideroblasts while 18 had mainly the intermediate or ferritin type. Basic erythro‐kinetic data (59Fe erythrocyte incorporation and erythroblast labelling indices) were similar in the two groups. The colony forming capacity (CFUC) was abnormal in 2 of 5 patients with ringed sideroblasts compared to 12 of 13 in the intermediate sideroblast group. Patients with mainly ringed sideroblasts had significantly longer duration of disease state, higher platelet and leucocyte counts, no increase in the percentage of bone marrow myeloblasts and did less often develop acute leukaemia. Thus aregenerative anaemias can be divided according to the type of abnormal sideroblasts in two groups with different risk of development of acute leukaemia. The ringed sideroblastic anaemia seems not to be a preleukaemic stage in the prospective sense in contrast to aregenerative anaemia with mainly intermediate sideroblasts.
The Mechanism of Endotoxin‐Induced Hypoferraemia
Authors: Torrance J. D. , Charlton R. W. , Simon M. O. , Lynch S. R. , Bothwell T. H.
Published: August 2010

The effect of endotoxin on the processing of erythrocyte iron by reticuloendothelial cells of the liver and spleen was studied in rats using heat damaged erythrocytes labelled with59Fe.

Endotoxin did not alter the uptake of the damaged cells but markedly inhibited the subsequent early phase of iron release from the reticuloendothelial cells. The inhibition seemed to be due to both a decreased rate of labelled haem destruction and an increased incorporation of radioiron into ferritin. Although early iron release was decreased 0–2 h after endotoxin administration, the diversion of iron into ferritin was more marked when endotoxin was given 18 h before. The block in iron release was partially overcome in animals that had been kept on an iron free diet or had been phlebotomised. In these animals the decreased rate of haem catabolism remained unaltered but less iron was diverted into ferritin.
Platelet Volume, Density and 5 HT Organelles (Mepacrine Test) in Acute Leukaemia
Authors: Nouvel C. , Caranobe C. , Sie P. , Capdeville J. , Pris J. , Boneu B.
Published: August 2010

Qualitative platelet parameters (volume, 5 hydroxy‐tryptamine (5 HT) organelles studied by the mepacrine test, and density) were evaluated in 31 patients with acute leukaemia: 11 myelomonocytic (AML), 8 lymphoblastic (ALL), 12 granulocytic (AGL). Macrothrombocytosis was observed in most of the cases of AML, was rare in AGL and was never found in ALL. The 5 HT organelles / volume ratio was normal in AGL and ALL but was significantly decreased in AML. In contrast, platelet density distribution was always abnormal whatever the platelet volume and 5 HT organelle concentration. Thus, using simple new methods, convenient even in cases of thrombocytopenia, we demonstrate that qualitative platelet abnormalities are a constant feature in acue leukaemia and that they are more severe in AML.
Response to Doxorubicin in Hairy Cell Leukaemia
Authors: McCarthy D. , Catovsky D.
Published: August 2010

A case of hairy cell leukaemia (HCL) treated by single‐agent chemotherapy after the failure of splenectomy is reported. The patient was treated with doxorubicin 50 mg on 4 occasions over an 8‐day period. After 2 weeks of profound pancytopenia, substantial blood and bone marrow improvement ensued. This case demonstrates that HCL may in some cases be treated successfully by intensive chemotherapy.
Inhibition of Haemopoietic Stem Cell Proliferation by a Diffusible Product of Bone Marrow Cells
Authors: Benestad H. B. , Testa N. G. , Lajtha L. G.
Published: August 2010

In a double diffusion chamber (DC) culture system bone marrow cells elaborated diffusible factor(s) that prevented spleen colony‐forming cells (CFU‐S), but not PHA stimulated lymphocytes, from entering cell cycle. Mature granulocytes and macrophages did not produce such factor(s).

Various numbers of steady‐state or regenerating mouse bone marrow cells were cultured in single diffusion chambers for periods up to 7 d. After the initial cell loss, the net growth of CFU‐S was inversely related to both total cell number and CFU‐S number in DC. Diffusible factors rather than cell‐to‐cell contact appeared to be involved in the inhibition, even though we were not able to demonstrate inhibition of net growth of CFU‐S with the double chamber approach.
Studies in Chronic Lymphocytic Leukaemia
Authors: Bazerbashi M. B. , Reeve J. , Chanarin I.
Published: August 2010

1. 9 Studies of lymphocyte transport are reported in which the 51Cr labelling technique of Hersey (1971) was employed on 8 patients with chronic lymphocytic leukaemia (CLL) and 1 with lymphosarcoma.

2. A new approach to the analysis of the data was formulated employing the principles of impulse analysis. This allowed the quantitation of cell transport between various locations making the minimum number of assumptions. It was also possible to take account of the principal defect of this label, its tendency to elute from live cells.

3. In spite of marked lymphocytosis, numbers of cells moving between those tissues whose lymphocytes exchange relatively slowly and the bloodstream was found to be normal. The blood and lymph nodes may therefore accumulate lymphocytes in response to a transport defect which hinders the progress of these cells from one pool to another.

4. The finding that CLL lymphocytes fluxes are normal in certain locations casts doubt on the hypothesis that lymphocyte proliferation is uncontrolled in this disease. An alternative possibility is that cell proliferation is regulated so as to maintain these fluxes within the normal range.
Mitotic Activity in Myeloid Leukaemias
Authors: Sjögren U.
Published: August 2010

The proportions of myeloblasts, promyelocytes, myelocytes and promonocytes within the granulopoietic precursor pool of the bone marrow and mitotic indices (MI) of the precursor cells were determined in 277 patients with various forms of myeloid leukaemias. The MI of the leukaemic patients were significantly lower than those of 20 normals. Thus the median MI in 186 cases of acute myeloid leukaemias was 0.68%, compared with 1.0% in 85 cases of chronic leukaemias and 1.2% in the normals. Separate MI of the different leukaemic precursor cells were counted in 21 cases of acute myeloid leukaemias and these indices were lower than those of the corresponding normal cells. There were significant differences between the different groups and subtypes of leukaemias and it is suggested that mitotic countings might be of diagnostic and prognostic significance.
Origin of Intranuclear Inclusions in Myeloma Cells
Authors: Djaldetti M. , Lewinski U. H.
Published: August 2010

The mechanism of inclusion of the cytoplasmic dense bodies into the nucleus of myeloma cells was studied by transmission electron microscopy. The findings in different cells suggest that the inclusion of the electron‐dense bodies is a result of an interaction between the nuclear envelope and the endoplasmic reticulum. The ability of the nuclear membrane to form an extension into the cytoplasm and the connection with the cisternae of endoplasmic reticulum enables the movement of the dense bodies towards the nucleus. The proposed mechanism does not support the concept of a simple invagination of the bodies into the nucleus or that they are produced in the nucleus itself.
Standardization of Romanowsky Stains
Authors: Marshall P. N. , Bentley S. A. , Lewis S. M.
Published: August 2010

A panel of 17 eminent haematologists has assessed the performance of 5 Romanowsky stains prepared from pure component dyes, comparing the suitability and acceptability of these stains for the preparation of routine blood and bone‐marrow films. It was found that the results obtained using the stain described by Marshall et al (1975) were comparable to those obtained using a modification of the stain described by Wittekind et al (1976). The performance of the 3 other stains was less acceptable. Variations in stain formulation have been correlated with stain performance.
Effect of Phenytoin on DNA Synthesis by Human Bone Marrow
Authors: Reid C. , Chanarin I.
Published: August 2010

Diphenylhydantoin (phenytoin) when incubated with human marrow interfered with the synthesis of deoxyribosenucleic acid (DNA) by those cells. This interference was dose related but was present at all concentrations, 25 μg/ml to 100 μg/ml. Cells unable to complete DNA synthesis would die in the marrow, lead to ineffective haemopoiesis and an increased folate requirement.
ATP‐ase Activity of Purified Human Normal T‐ and B‐Lymphocytes
Authors: Kragballe Knud , Ellegaard Jørgen
Published: August 2010

In 15 healthy individuals the ATP‐ase activity of circulating B‐lymphocytes was found to be significantly higher than of T‐lymphocytes. Two different methods were used for the purification of B‐ and T‐lymphocytes and the result was independent of which one of these methods was used. The demonstration of different ATP‐ase activities of un‐stimulated B‐ and T‐lymphocytes corresponds well with previously published histo‐chemical findings in lymphatic tissues examined for ATP‐ase activity. It is concluded from the present study that because of overlapping values determination of the lymphocyte ATP‐ase activity cannot serve as a single marker for the B‐ and T‐cell nature of human lymphocytes, but demonstration of an unusual high ATP‐ase activity in unstimulated lymphocytes will serve as additional identification of these as belonging to the B‐lymphocyte population.
Immunological Family Studies in Hodgkin's Disease
Authors: Björkholm M. , Holm G. , Mellstedt H.
Published: August 2010

27 consanguineous and non‐consanguineous relatives to 12 patients with Hodgkin's disease (HD) have been studied. The lymphocyte DNA synthesis induced by concanavalin (ConA) was depressed in 6 patients. 7 out of 15 tested relatives (3 first degree relatives and 4 spouses) showed a profound impairment in lymphocyte response to ConA stimulation. None out of 12 relatives to ConA normal patients was defect in this respect. In relatives to ConA hyporesponsive patients there was a small but statistically significant decrease in lymphocyte response to pokeweed mitogen (PWM) and PPD in vitro. No differences in lymphocyte subpopulations were seen. The findings strongly support an exogenous factor (probably a virus) causing the depressed mitogen response in relatives to immunodeficient patients with HD. A possible linkage between the immunodeficiency and the pathogenesis of HD is discussed.
51Cr Labelling of Normal Human T and B Lymphocytes for Kinetic Studies in Vivo
Authors: Jønsson Viggo , Christensen Bjarne Egelund
Published: August 2010

Lymphocyte traffic between blood and tissues was assessed by 51Cr labelling of lymphocytes and subsequent autologous reinfusion in 10 normal elderly persons. The technique for isolation and platelet depletion of lymphocyte suspensions is described. By the labelling procedure used about 70 μCi 51Cr may be incubated in about 100 million lymphocytes. This permits measurement of lymphocyte‐bound radioactivity on the T and B fractions separately. The blood disappearance curves for labelled lymphocytes indicate the existence of exchangeable pools in the tissues of T as well as of B lymphocytes, that of the T lymphocytes being apparently larger. A characteristic finding in the blood disappearance curves for total lymphocytes is an increase in lymphocyte‐bound radioactivity in the blood 4–6 h after reinfusion, designated reappearance. The disappearance curve of the B lymphocytes shows reappearance 4–10 h after reinfusion, whereas that of the T lymphocytes falls exponentially without any recordable reappearance. On the basis of the disappearance curves and a knowledge of the topographic distribution of T and B lymphocytes in the lymphoid tissues, a model of T and B lymphocyte traffic in the lymph nodes is discussed. This model operates with T and B lymphocyte passage by way of postcapillary venules and describes the migration in and around the germinal centres. The T lymphocytes in the periphery of the germinal centres are assumed to derive mainly from the afferent lymph, whereas the B lymphocytes in the centres are exchanged with lymphocytes in the blood in an exchangeable pool. The functional implications are discussed.
The Exchangeable Splenic Platelet Pool in Response to Intravenous Infusion of Isoprenaline
Authors: Fredén Krister , Olsson Lars‐Bertil , Suurküla Madis , Kutti Jack
Published: August 2010

8 healthy volunteers and 4 asplenic subjects, in whom autologous platelets had been labelled with radioactive sodium chromate, received i.v. infusions of isoprenaline in a dose of 0.03 μg × kg–1 × min–1 over a period of 6 min. In the former group these infusions caused a significant decrease in the concentration of labelled as well as unlabelled platelets in the peripheral blood. Body surface countings showed that a significant increase in the count rate over the spleen occurred concomitantly with the decrease in the circulating platelet‐bound radioactivity (PBR). In the group of asplenic subjects no change in PBR occurred. It is concluded that adrenergic beta‐receptor stimulation causes a transitory trapping of platelets in the exchangeable splenic platelet pool.
Red Cell Membrane Proteins and Lipids in Spherocytosis
Authors: Johnsson Riitta
Published: August 2010

The protein and lipid content of erythrocyte membranes in 13 patients from 9 families with congenital or hereditary spherocytosis (HS) were studied.

Lyophilized erythrocyte membrane proteins were solubilized using two different methods and analysed in two electrophoretic systems. On polyacrylamide gel electro‐phoresis with sodium dodecyl sulphate as solubilizing agent, the number of major protein bands detected was equal in both the HS patients and the controls. Likewise, no significant differences in the protein fractions from the two groups were seen using an electrophoretic system that gave better resolution of low‐molecular wt. proteins, although minor differences in distribution between the protein fractions were observed.

The amount of lipid phosphorus and cholesterol in red cell ghosts from unsplenectomized patients with HS was lower than in normal erythrocyte membranes. The decrease in cholesterol content exceeded that of lipid phosphorus. A tendency to reattain control levels was observed after splenectomy. In patients splenectomized several years prior to the study membrane lipid concentrations were normal. A decrease in membrane lipids was also detected in a patient with spherocytosis due to autoimmune haemolytic anaemia and in out‐dated bank blood containing sphero‐cytic red cells; the ratio of cholesterol to lipid phosphorus was abnormally low in both these instances.

Thus the alterations in red cell lipid components in spherocytes of varying origin may represent a phenomenon secondary to the abnormal red cell shape rather than being related to a specific etiological factor.
Preleukaemia and Reticulocytosis: A Case Report with in Vitro Evidence for Abnormal Reticulocyte Maturation
Authors: Lofters W. S. , Ali M. A. M. , Pineo G. F.
Published: August 2010

A case of preleukaemia with marked reticulocytosis and a slightly reduced red cell 51Cr survival is presented. An in vitro ‘reticulocyte survival’ test suggested that the apparent reticulocytosis was due to delayed maturation of reticulocytes. The globin synthesis ratio suggested an abnormal ribonucleic acid.

At autopsy, in addition to the findings of acute leukaemia, mesenteric lipodystrophy was discovered. This abnormality has been seen in patients with malignant diseases, especially lymphomas, but heretofore has not been reported in acute leukaemia.
Coexistence of Haemophilia A and Von Willebrand's Disease in the Same Kindred
Authors: Gastaldi G. , Rasore‐Quartino A. , Galletti A. , Campanella A. , Barone E. , Mannucci P. M.
Published: August 2010

A clinical and laboratory investigation of a kindred in which haemophilia A and autosomal recessive von Willebrand's disease (VWD) were concomitantly present is described. 3 male patients were shown to be hemizygotes for moderate haemophilia A, one female appeared to be haemophilia A carrier and 3 males showed laboratory findings consistent with heterozygosity for autosomal recessive VWD. In one woman, the pedigree and laboratory findings suggest the possibility of double heterozygosity.
Congenital Factor V Deficiency
Authors: Mitterstieler G. , Müller W. , Geir W.
Published: August 2010

A 14‐month‐old girl suffering from a heavy bleeding tendency, caused by a severe isolated congenital factor V deficiency is described. In this study 56 family members were examined. 10 of them had a factor V level ranging 26–60% of the normal – these were classified as heterozygotes. The case histories of the heterozygotes did not reveal a bleeding tendency. The inheritance of this factor V deficiency is autosomal recessive, with varying expressivity in the heterozygotes.
In Vitro Medullary Granulocytic Progenitor (CFUC) Cultures from 6 Cases of Granulocytopenias
Authors: Parmentier C. , Tchernia G. , Subtil E. , Diakhate L. , Morardet N.
Published: August 2010

Medullary granulocyte progenitor (CFUC) cultures were grown in vitro from samples taken from 6 patients with toxic granulocytopenia caused by either thiamphenicol, cephalothin or amidopyrine and who are now apparently cured. A decrease in the medullary concentration of CFUC has been observed and a calculated estimate shows that there was a decrease in their absolute number. A decrease in the number of CFUC per 105 metamyelocytes suggests a possible compensation by mitotic amplification between the stem cell and the differentiated cells. Two successive cultures have shown that the course of such medullary cultures is variable. The existence of medullary anomalies before drug toxicity as well as the practical consequences of the contrast between the apparent cure and the decrease in CFUC are discussed.
Iron Kinetics and Erythropoiesis in Fanconi's Anaemia
Authors: Barosi G. , Cazzola M. , Marchi A. , Morandi S. , Perani V. , Stefanelli M. , Perugini S.
Published: August 2010

Ferrokinetic studies were carried out in 4 patients with Fanconi's anaemia (FA). Experimental data were analysed by means of a mathematical model of iron kinetics in order to obtain a quantitative assessment of effective and ineffective erythropoietic activity, mean red cell lifespan, and non‐erythroid iron turnover. The major pathogenetic mechanism of the anaemia appeared to be relative marrow failure, i.e. a reduction in the proliferative capacity of the erythroid marrow. The role of ineffective erythropoiesis was of minor importance. On the basis of the results obtained both the pathogenesis and the natural course of the disease are discussed.
Presence of α ‐Naphthyl Acetate Esterase Activity in Human Haematopoietic Cell Lines and in Fresh Biopsy Specimens of Lymphoma and Myeloma
Authors: Sundström C. , Nilsson K. , Ranki Annamari , Häyry P.
Published: August 2010

We have previously shown that non‐proliferating human T‐ but not B‐lymphocytes contain demonstrable amounts of acid α‐naphthyl acetate esterase (ANAE). The usefulness of this histochemical marker for the diagnosis and classification of malignant lymphoid tumors was investigated by use of a panel of established normal and malignant human haematopoietic cell lines and fresh biopsy cells from malignant lymphomas and myelomas. The results showed that not only the T‐cell derived acute leukaemia lines, but also histiocytic lymphoma and myeloma lines and some of the lymphoma (Burkitt and lymphocytic) and non‐neoplastic lymphoblastoid cell lines with B‐cell surface markers expressed strong ANAE reactivity. Some but not all of the immunoglobulin producing myeloma and lymphocytic lymphoma biopsies were ANAE‐positive. Inhibition experiments with sodium fluoride and E‐600 demonstrated that although the T‐lymphocyte specific esterase is predominantly of ‘A’‐type, the malignant lines contain also non‐specific ‘B’ esterase and pseudocholinesterase. As the presence of the various esterases did not demonstrate any specific distribution pattern among he haematopoietic cell lines of different origin, we concluded that the ANAE marker is no longer T‐specific when malignant lymphoid cells are considered, and that the usefulness of this marker in routine diagnostic work therefore is limited.
A Near Haploid Clone: 24, XY, t (9; 22) (q34; q11) from a Patient in Blast Crisis of Chronic Myeloid Leukaemia
Authors: Daniel A. , Francis S. E. , Stewart L. A. , Barber S.
Published: August 2010

Chromosome studies on bone marrow in a patient with chronic myeloid leukaemia (CML) revealed a mosaic picture with a dominant clone of 24, XY, t (9; 22) (q34; q11). This corresponded to a preponderance of minute blasts in smears of bone marrow aspirate. It is suggested that a haploidy event rather than progressive hypodiploid loss, is responsible for the genesis of the miniature blast cells.
Adenosine Deaminase Activity in Acute Lymphoblastic Leukaemia: Cytochemical, Immunological and Clinical Correlations
Authors: Liso V. , Tursi A. , Specchia G. , Troccoli G. , Loria M. P. , Bonomo L.
Published: August 2010

Adenosine deaminase (ADA) activity has been assessed in lymphoid cells of 23 patients with acute lymphoblastic leukaemia (ALL) in order to attempt a further characterization of ALL cells in addition to the well known cytochemical and immunological T and B lymphoid cell markers. ADA activity did not show any correlation with the immunological characterization of the patients investigated; in fact a wide range of ADA activity was observed with levels ranging from 0 to 32 U in T‐ALL patients and nearly similar values (from 1.8 to 36 U) in the group of non T — non B ALL cases. Normal values ranged from 2 to 5 U (mean 2.9 U; s.d. ± 0.8). Some cytochemical patterns (acid phosphatase and PAS) appeared well correlated with T markers of lymphoid cells, whereas they showed no significant relationship with ADA activity.
Acute Promyelocytic Leukaemia: Further Evidence of the Differentiation‐Linked Expression of la‐Like (p28,33) Antigens on Leukaemic Cells
Authors: McVerry B. A. , Goldstone A. H. , Janossy G.
Published: August 2010

Patients with acute non‐lymphoblastic leukaemia were studied to determine if the presence of la‐like antigens on leukaemic cells was related to the maturity of a leukaemic cell line. Ia‐like antigens were present in the majority of acute myeloblasts and myelomonoblastic leukaemias. 4 patients with acute promyelocytic leukaemia were la‐negative. This finding suggests that la‐like antigens reflect normal differentiation linked events in haemopoiesis. The presence of Ia‐like antigens on leukaemic cells did not alter the patient's response to chemotherapy.
Morphologic Studies of the Erythropoietic Part of the Bone Marrow in Myeloid Leukaemias
Authors: Sjögren U.
Published: August 2010

The erythropoietic part of the bone marrow has been morphologically analyzed in 147 patients with various forms of myeloid leukaemias and in 20 healthy controls. In all the patients the percentage of basophilic erythroblasts was abnormally high and correlated to elevated mitotic indices. Megaloblastic changes were found to be relatively common in the patients. All these findings are compatible with an ineffective erythropoiesis where the normal differentiation towards more mature erythroblasts becomes increasingly blocked during the course of the disease regardless of the type of myeloid leukaemia.
Some Immunological and Haematological Aspects of Human Cyclic Neutropenia
Authors: Andrews R. B. , Dunn C. D. R. , Jolly J. , Jones J. B. , Lange R. D.
Published: August 2010

In addition to standard peripheral blood cell counts, sequential studies have been made of changes in the T‐lymphocyte population and in the serum titres of the presumptive humoral regulators of haematopoiesis, Colony Stimulating Activity (CSA) and Erythroid Stimulating Activity (ESA), in a young woman with cyclic neutropenia (CN). In addition, serum immunoglobulins, C3 and total complement levels and serum protein concentrations were determined on several occasions during the study. Similar tests were done concomitantly on a haematologically normal, age and sex‐matched control. Cell counts on peripheral blood from the subject with CN demonstrated a clearly denned periodicity in neutrophil and monocyte concentrations and equivocal fluctuations in reticulocyte numbers. There was no evidence of periodicity in the lymphocyte concentrations and the T‐lymphocyte population appeared functionally normal. Spontaneous incorporation of tritiated thymidine into peripheral blood cells showed a highly significant correlation with the monocyte count, suggesting that these cells were responsible for the radioisotope uptake. CSA titres were elevated on all occasions tested and showed no evidence of periodicity. ESA showed some evidence of cycling with elevated levels being observed during the periods of neutropenia. Serum complement levels were within the normal range but all classes of immunoglobulins were elevated and albumin levels were depressed.
Autonomous Erythropoietin Induced Erythrocytosis
Authors: Davies S. A. , Goolden A. W. G. , Lewis S. M. , Zaafran A.
Published: August 2010

Erythrocytosis in a young Sinhalese man is described. The patient was known to have had a raised Hb and PCV for at least 10 years. Subsequent investigations failed to support the diagnosis of polycythaemia vera or to reveal a cause for secondary polycythaemia. Blood erythropoietin values were raised, but no cause for inappropriate secretion could be identified. Although there was no evidence of erythrocytosis in the family, the findings in this patient appear to be those of a condition which has been called familial polycythaemia. The spleen was unusually large and was associated with hypersplenism and thrombocytopenia. Problems of diagnosis and management are described. Phlebotomy appears to be the treatment of choice, with a regimen of regular venesection for the control of symptoms due to hyperviscosity and vascular occlusion.
Elevated Plasma Lysozyme in Hodgkin's Disease An Indicator of Increased Macrophage Activity?
Authors: Hansen Niels Ebbe , Karle Hans
Published: August 2010

Plasma lysozyme levels were studied in 42 patients with Hodgkin's disease and were found significantly increased. Plasma lysozyme varied with the stage of the disease and with symptoms, but did not correlate with the histological subtype or with blood neutrophil and monocyte counts. Serial measurements in four patients on MOPP treatment showed a rapid decrease following treatment. The increased plasma lysozyme in Hodgkin's disease stems most likely from the macrophage system, either because the macrophages are hyperactive and/or because the malignant cell in Hodgkin's disease is of macrophage origin.
Haemolytic Anaemia of Pregnancy
Authors: Goodall H. B. , Ho‐Yen D. O. , Clark D. M. , Thomson Margaret A. R. , Browning Margaret C. K. , Crowder A. Mary
Published: August 2010

Haemolytic anaemia apparently induced by pregnancy is reported in an otherwise healthy woman, with a negative family history. Folate deficiency, haemoglobino‐pathies, enzymopathies, hereditary spherocytosis, paroxysmal nocturnal haemoglobin‐uria, interstitial haematomata, hypertensive toxaemia, auto‐immunity and infections were excluded. The autohaemolysis test showed increased lysis in the presence of added glucose. This abnormality was corrected by insulin when excess glucose was present, but not by insulin alone. It is suggested that this patient's erythrocytes may have been altered by placental lactogen and/or prolactin in such a way as to make them abnormally dependent on insulin for the uptake of glucose.
Erythropoiesis in Sickle Cell Anaemia during Acute Infection and Crisis
Authors: Haddy Theresa B. , Lusher Jeanne M. , Hendricks Susan , Trosko Beverly Kay
Published: August 2010

Erythropoiesis was evaluated in 37 patients with sickle cell anaemia, 26 of them children under 12 years of age. Mean haemoglobin, haematocrit, reticulocyte, and erythropoietin levels were similar for 11 who were asymptomatic, 11 with infections, and 12 in vaso‐occlusive crisis. Mean haemoglobin, haematocrit, and reticulocyte values were significantly lower and the mean erythropoietin level significantly higher for three patients in aplastic crisis. Reticulocyte counts reflected erythropoietic activity during the asymptomatic state but were variable during infection and crisis. No erythropoietic inhibitory activity was found in any of the four clinical states. It has been suggested that erythropoietin production decreases during infection. Patients in this study responded appropriately to stress, showing no decrease in erythropoietic activity during acute infection or crisis.
Blood Colony‐Forming Cells (CFU‐C) and Leucocyte Colony‐Stimulating Activity (CSA) in Patients with Neutrophilic Leucocytosis
Authors: Morra L. , Ponassi A. , Gigli G. , Vercelli M. , Sacchetti C.
Published: August 2010

The incidence of circulating colony‐forming cells (CFU‐C) and the ability of peripheral leucocytes to stimulate the colony formation (CSA) have been studied through a double layer agar culture system in 26 patients with neutrophilic leucocytosis and compared to the values obtained in 26 normal subjects.

Both mean CFU‐C incidence and mean leucocyte CSA of the whole group of patients were found significantly higher than normal, but considerable variation was observed among singular patients.

The different patterns of blood CFU‐C and leucocyte CSA are discussed. The combined evaluation of blood CFU‐C and leucocyte CSA is found a useful tool to investigate the pathogenetic mechanisms of neutrophilic leucocytosis.
Successful Treatment with Prednisone of Graft‐Versus‐Host Disease in an Allogeneic Bone‐Marrow Transplant Recipient
Authors: Groth Carl‐Gustav , Gahrton Gösta , Lundgren Göran , Möller Erna , Pihlstedt Peter , Ringdén Olle , Sundelin Pär
Published: August 2010

A 51‐year‐old patient with aplastic anaemia in whom a successful allogeneic bone‐marrow transplantation had been performed developed acute graft‐versus‐host disease in spite of prophylactic administration of methotrexate. There was severe liver injury but no involvement of the skin or intestines. When prednisone therapy was introduced the fever and the eosinophilia disappeared and liver damage was rapidly reversed. There was no haematological impairment. It would seem warranted to consider a wider use of prednisone in the treatment of acute GVHD in human recipients of allogeneic bone‐marrow.
Osteogenesis Imperfecta Associated with Multiple Myeloma
Authors: Kutnowski M. , Futeral B. , Verbanck M. , Naets J. P. , Mullier J. P.
Published: August 2010

In a case of osteogenesis imperfecta with multiple fractures already from childhood, myelomatosis was diagnosed at the age of 52 years because of a serum M‐component (IgG, lambda), Bence Jones proteinuria, myeloma cells in the bone marrow, and osteolytic skeletal lesions.

She died 10 months later. A partial postmortem examination of a larger bone lesion confirmed the diagnosis.
Androgen Therapy of Aplastic Anaemia ‐A Prospective Study of 352 Cases
Authors: Najean Y. , Pecking A. , Danvic M. Le ,   The Cooperative Group for the Study of Aplastic and Refractory Anaemias Secretaries
Published: August 2010

A prospective study of 352 patients with aplastic anaemia on androgen therapy has been performed. The following main observations have been obtained: The actuarial mortality rate at the 20th month is 52 %, half the deaths being observed during the first 3 months; these figures are similar to those previously published, from smaller series of androgen‐treated patients, and lower than those of non‐androgen‐treated cases.

Differences in survival and improvement were observed between groups of patients treated for more than 3 months with either alkylated or non‐alkylated drugs. Signs of liver damage were observed no matter which was the drug used. Continous improvement can be observed even in the 2nd year of treatment indicating that full‐dose androgen therapy should be continued up to 20 months in not fully improved patients.

The degree of initial disease activity is a clear prognostic parameter for the mortality in the first quarter of the course. In case of survival of severe cases, improvement can be obtained to the same extent as in milder cases. This stress the need for adequate maintenance therapy in all types of patients.

Addition of glucocorticoids harms the prognosis, mainly in most granulocytopenic patients. Glucocorticoids have no effect upon the liver damage induced by andro‐gens.
Preparation of Platelet Suspensions from Whole Blood in Buffer
Authors: Schmidt Kai Gjerløff , Rasmussen Jens Weer
Published: August 2010

A simple method for preparation of platelet suspensions from whole blood in buffer is described. The platelets and a fraction of the erythrocytes are processed simultaneously, whereby the erythrocytes serve as a supporting cushion in the centrifugation steps. Using this earlier described principle of platelet separation, platelet yields of 90–100% from whole blood could be achieved. Investigations of platelet aggregation and platelet morphology indicate that this method of separation is gentle. By obviating the selection of certain platelet populations this method may facilitate the interpretation of the results of in vitro and in vivo platelet‐function studies.
Labelling of Human and Rabbit Platelets with 111Indium‐Oxine Complex
Authors: Schmidt Kai Gjerløff , Rasmussen Jens Wæver
Published: August 2010

Platelets suspended in buffer were labelled with 111Indium‐oxine. In the isolation procedure the platelets and a fraction of the erythrocytes are processed simultaneously, whereby the erythrocytes serve as a supporting cushion in the centrifugation steps. Various factors which influence the labelling effectiveness were examined, and the method's utility was estimated by in viva studies in rabbits.

A platelet concentration of more than 800 times 109/l and an oxine concentration of approximately 6 μg/ml in a plasma‐poor system (transfer of platelets from plasma to buffer without further washing steps) will yield a high labelling efficiency. If the platelet isolation yield is to be large, a slight erythrocyte‐leucocyte contamination must be accepted. Our experimental results indicated that this is permissible. In vivo investigations of 5 rabbits showed in vivo recoveries of platelets from 61–74% and mean survival times from 69h to 110h. This supports the assessment that the method of platelet isolation and labelling is gentle.
Quantitative Evaluation of the Mechanisms of the Anaemia in Heterozygous β‐Thalassaemia
Authors: Cazzola M. , Alessandrino P. , Barosi G. , Morandi S. , Stefanelli M.
Published: August 2010

Ferrokinetic studies were carried out in 8 patients with heterozygous β‐thalassaemia with anaemia of varying severity. Effective and ineffective erythropoiesis, mean red cell lifespan and non‐erythroid iron turnover were estimated from the experimental data through a mathematical model of iron kinetics. Erythropoietic activity was markedly increased in all patients, but was variably ineffective (from 10 to 74%). A negative correlation (r = ‐0.855, P < 0.01) was found between the amount of ineffective erythropoiesis and Hb level. Red cell lifespan was variably shortened and there was a negative correlation between the degree of daily peripheral haemolysis and Hb level (r = ‐0.733, P < 0.05). Non‐erythroid iron turnover was increased in most patients. The results provide quantitative measurements of the mechanisms responsible for the wide variation of the Hb level in heterozygous β‐thalassaemia. Ineffective erythropoiesis seems to be the major reason for the anaemia. Peripheral haemolysis contributes to it, especially in the most severely affected patients. The increased non‐erythroid iron turnover may be responsible for the pathology which characterizes heterozygotes in the adult life.
Effect of Human Serum from Patients with Haematological Disorders on Mouse Pluripotent Haemopoietic Stem Cells (CFU‐S)
Authors: Sawada Umihiko , Kuznetsky Richard D. , Adler Solomon S.
Published: August 2010

Sera from 25 individuals (9 healthy subjects, 6 patients with systemic lupus erythematosus, and 10 patients with a variety of haematological disorders) were tested for anti‐CFU‐S by incubating suspensions of mouse marrow cells with serum and then assaying the cell suspensions for their capacity to form spleen nodules in lethally irradiated mice. The sera from 2 patients (1 with a preleukaemic disorder and the other with a malignant ‘histio’‐lymphoproliferative neoplasm) had anti‐CFU‐S activity which behaved like antibodies, rather than like the non‐specific cytotoxic activity found in various sera for heterologous tissues, which in the past has been attributed to ‘natural antibodies’ and which, in recent years, has, in some cases, been found to be related to activation of the alternative pathway of complement. The anti‐mouse tissue activity in the sera of the 2 patients may be related either to cross‐reactivities between certain mouse and human tissues or to cross‐reactivities between exogenous agents such as bacteria and mouse tissues.
Triamterene‐Induced Immune Haemolytic Anaemia with Acute Intravascular Haemolysis and Acute Renal Failure
Authors: Takahashi Hoyu , Tsukada Tsuneyasu
Published: August 2010

Acute intravascular haemolysis and renal failure developed while a patient was taking triamterene. A direct antiglobulin test with a polyvalent reagent was positive. Serum caused agglutination of normal red cells in the presence of triamterene and caused an increase of partial haemolysis of both trypsin‐treated erythrocytes and red cells from a patient with paroxysmal nocturnal haemoglobinuria (PNH) in the presence of complement. From the results of antibody‐neutralization test and treatment with 2‐mercaptoethanol, the presence of IgM antibody with Λ light chain could be demonstrated.

The triamterene seemed to bind strongly to the red cells in vitra but in vivo there was no detectable adsorption to red cells. Haptenic inhibition was not demonstrated.

From these results, it was assumed that this antibody caused acute intravascular haemolysis by immune complex mechanism. The antibody was found to cross‐react with methotrexate which has a structure similar to that of triamterene.
The Effect of Acetyl Salicylic Acid and Dipyridamole on Thromboembolic Complications in Splenectomized Patients with Myelofibrosis
Authors: Hansen Mogens Sandbjerg , Christensen Bjarne Egelund , Jønsson Viggo
Published: August 2010

The effect of combined treatment with acetyl salicylic acid and dipyridamole on thrombotic phenomena was studied in splenectomized patients with an aggressive form of myelofibrosis. 3 of these patients had thrombotic episodes, shortened platelet survival and an abnormal platelet aggregation pattern. The treatment with anti‐platelet drugs resulted in a decreased platelet utilization as evidenced by clinical improvement and an increasing platelet concentration.
Serum Iron and Transferrin Saturation in Women with Special Reference to Women with Low Transferrin Saturation
Authors: Lennartsson Jan , Bengtsson Calle , Hallberg Leif , Lundgren Birgitta K. , Tibblin Elisabeth
Published: August 2010

Serum iron and total iron binding capacity (TIBC) were determined in a population sample of 1462 women in age strata between 38 and 60. Serum iron and TIBC values were similar in the various ages studied but with a slight trend towards higher serum iron and lower TIBC values in the upper ages. Transferrin saturation was used to divide the material arbitrarily into women with and without iron deficiency. The dividing point chosen was 16%. The women thus defined as iron deficient had lower mean haemoglobin values than women in the total population sample and were more often anaemic. They had also lower MCV, MCH and MCHC indices than women in the total population sample. Of these indices, MCH seemed to discriminate the state of iron deficiency better than MCV and MCHC. Except for an increased mean menstrual blood loss no obvious cause of iron deficiency could be found in these women with low transferrin saturation.
Factors Interfering with the Cell Morphology in Smears
Authors: Talstad Ingebrigt , Gundersen Jan S.
Published: August 2010

Factors interfering with the cell morphology in smears were studied. The following artifacts were observed: Lysis of cells with release of granules, cytoplasmic and nuclear proteins; fern‐like structures due to detachment of protein from glass; rupture of the surface of smears; crystal formations; leakage from cells interfering with the surrounding medium; cell shrinkage, vacuolization; and aggregation of cells caused by gammaglobulins. The concentration and type of plasma protein in the medium greatly influenced the type of artifacts. The technique by which the least artifacts occurred, was cytocentrifugation of cells suspended in a cell protecting medium.
Platelet Heterogeneity
Authors: Leone Giuseppe , Agostini Augusto , DeCrescenzo Alberto , Bizzi Bruno
Published: August 2010

Human platelets were separated into 2 density populations by repeated centrifugations of platelet‐rich plasma at increasing gravitational force.

The heaviest platelet fraction was rich in larger platelets. The lightest platelet fraction was rich in smaller platelets. In both fractions and in the platelet button, lipid peroxidation (malonaldehyde‐MDA‐production after addition of thrombin) was measured at basal condition, on the 1st, 3rd, 5th, 7th and 9th day after aspirin ingestion. At basal conditions and after ingestion of aspirin, MDA production was higher in the heavy‐large platelets than in light‐small ones, but a parallel increase of MDA production was observed in the light and in the heavy population and in the platelet button. The data are not compatible with the hypothesis that platelet density and size are age‐related. Aspirin inhibits platelet lipid peroxidation by permanently acetylating their cyclooxygenase and if the heaviest platelets were the young ones, lipid peroxidation should reappear sooner in them.
Sézary Syndrome Associated with Sideroblastic Anaemia
Authors: Shirley Janet A. , Sparrow Gillian P.
Published: August 2010

A 66‐year‐old man with acquired sideroblastic anaemia and Sézary syndrome is described. This is believed to be the first such case reported in the literature. We speculate on the possibility that a common stem cell defect could account for the simultaneous development of the two abnormalities. Other features of this case of Sézary syndrome are discussed in relation to previous reports on patients with this disease.
Pel‐Ebstein Fever Coinciding with Cyclical Haemolytic Anaemia and Splenomegaly in a Patient with Hodgkin's Disease
Authors: McKenna William , Lampert Irvin , Oakley Celia , Goldman John
Published: August 2010

A 46‐year‐old man with an aortic valve replacement was investigated for recurrent episodes of fever associated with splenomegaly and haemolytic anaemia. Initially bacterial endocarditis was suspected. At laparotomy he proved to have mixed cellularity Hadgkin's disease confined to the spleen. The undefined mechanism underlying Pel‐Ebstein fever in this patient may also have been the cause of simultaneous haemolysis and splenomegaly.
Pernicious Anaemia in Association with Argyrophil (Sevier‐Munger) Gastric Carcinoid
Authors: Wilander Erik , Sundström Christer , Grimeius Lars
Published: August 2010

3 cases of pernicious anaemia in association with gastric carcinoid (in 1 case multiple) are described. All tumours were rich in argyrophil cells with the Sevier‐Munger and Grimelius staining techniques, indicating that they might be of the enterochro‐maffin‐like cell type. The possible causal relationship between pernicious anaemia and this type of argyrophil carcinoid is discussed.
‘Lennert's Lymphoma’ Terminating as Malignant Histiocytosis
Authors: Economopoulos Theophanis C. , Stathakis Nicolas , Stathopoulos Efstathios , Alexopoulos Constantine
Published: August 2010

A case of the so‐called ‘Lennert's lymphoma’ terminating as malignant histiocytosis is reported. Such a sequence of diagnoses is discussed as a possible progression of the initial ‘Lennert's lymphoma’ eventually under the influence of cytostatic treatment. A review of other known malignancies ending up in malignant histiocytosis is given.
Serum Erythrocyte Folate Levels in Thalassaemic Patients in Thailand
Authors: Vatanavicharn S. , Anuvatanakulchai M. , Na‐Nakorn S. , Wasi P.
Published: August 2010

The mean serum and adjusted red cell folate levels, as measured by microassay using Lactobacillus casei, respectively were 8.87 ± SD 3.28 μg/1 and 436 ± SD 107 μg/1 in 76 normal subjects, 4.22 ± SD 2.70 μg/1 and 182 ± SD 114 μg/1 in 55 patients with β‐thalassaemia/Hb E disease and 6.36 + SD 2.95 μg/1 and 320 ± 158 μg/1 in 37 patients with Hb H disease. The mean serum and adjusted red cell folate values of the patients with β‐thalassaemia/Hb E disease and the mean serum folate value of the patients with Hb H disease were significantly lower than those of the normal subjects (P < 0.001). 33 % of the β‐thalassaemia/Hb E patients and 8 % of the Hb H patients showed low serum folate levels (< 3 μg/1) whereas 84 % of the former and 45 % of the latter showed low adjusted red cell folate levels (< 270 μg/1). The group of β‐thalassaemia/Hb E disease with low serum folate levels had lower mean haemoglobin concentration and lower mean adjusted red cell folate level than the group with normal serum folate levels. Since the Thai diets have high folate content, the observed low serum and erythrocyte folate levels in thalassaemic patients most likely occur from massively increased erythropoiesis. Folate, 5 mg/d, is now routinely prescribed to such patients especially to those with severe anaemia.
A Platelet Defect in a Patient with Eosinophilic Leukaemia: Absent Ristocetin‐Induced Platelet Aggregation Associated with a Reduced Platelet Sialic Acid Content
Authors: Wautier J. L. , Souchon H. , Dupuis D. , Caen J. P. , Nurden A. T.
Published: August 2010

Platelets from a patient with eosinophilic leukaemia were not aggregated by ristocetin. The defect was not corrected by normal human plasma and was due to a platelet abnormality. The patient's platelets also showed a diminished sensitivity to aggregation by bovine factor VIIIVWF. The defect was not associated with a prolonged bleeding time. No abnormalities were detected in ADP, collagen or thrombin‐induced platelet aggregation. Biochemical studies showed that the platelets were deficient in sialic acid. This deficiency was associated with a reduced staining for glycoprotein I following SDS‐polyacrylamide gel electrophoresis. The results suggest an acquired platelet surface abnormality.
Periodic Acid‐Schiff Reaction Changes During the Course of Relapsing Lymhpoblastic Leukaemia
Authors: Lilleyman J. S. , Britton J. A. , Sugden P. J. , Hoyle B. J.
Published: August 2010

Bone marrow smears taken at diagnosis and subsequent haematological relapse from 41 patients with lymphoblastic leukaemia (ALL) were compared to assess the proportion of blast cells showing a positive periodic acid‐Schiff (PAS) reaction at different stages of the same disease. 20 patients never displayed more than 10% PAS positive blasts at any stage, and of these 8 never had any PAS positivity at all. 7 patients maintained a proportion of PAS positive cells over 10 % of the total but with no loss or gain in the numbers of such cells. 14 patients showed a significant change in the PAS reaction during the course of their disease, 7 showing a marked reduction in PAS positive cells, and 7 showing an equally marked increase.

From these results it seems that the PAS reaction does not identify a sub‐type of ALL or mark the degree of differentiation of a given case. It could be indicative of a specific stage of the lymphoblast mitotic cycle.