bluuebunny/crossref_metadata_2025_split huggingface dataset:
Carrier Detection in Haemophilia A by Immunological Measurement of Factor VIII Related Antigen (VIIIRAg) and Factor VIII Clotting Antigen (VIIICAg)
Authors: Peake I. R. , Newcombe R. G. , Davies B. L. , Furlong R. A. , Ludlam C. A. , Bloom A. L. 
Published: July 2010

Summary.23 obligate carriers of mild and severe haemophilia A and 26 normal females were bled on three occasions, and their plasmas assayed for procoagulant factor VIII (VIIIC), factor VIII related antigen (VIIIRAg) and factor VIII clotting antigen (VIIICAg). A comparison of the ratios VIIIC/VIIIRAg and VIIICAg/VIIIRAg indicated that, although the two ratios gave the same proportional misclassification of carriers as normals (four of 23), the latter ratio showed greater discriminatory power when an unequal variances predictive method was used to calculate likelihood ratios (for carrier status). This greater power was shown to be due to a greater reproducibility between visits for the VIIICAg/VIIIRAg ratio. Discrimination was considerably better when the median of the three median values for each variable was analysed, compared to the median value obtained at the first visit. There was also no statistical difference between VIIICAg/VIIIRAg (or VIIIC/VIIIRAg) ratios obtained from carriers of severe compared to mild haemophilia.
EFFECT OF HYDROCORTISONE ON LONG‐TERM HUMAN MARROW CULTURES
Authors: Suda T. , Dexter T. M. 
Published: July 2010

Summary.In human long‐term marrow cultures a relatively high concentration of hydrocortisone (10−6 M or more) is necessary for the development and subsequent maintenance of some cellular components in the adherent cell layer. However, such concentrations of hydrocortisone seem to be inhibitory for the production of granulocyte/macrophage progenitor cells (GM‐CFC). In further attempts to improve these cultures, therefore, it is important to maintain a balance between a concentration of hydrocortisone which is necessary for the development of a competent adherent layer and the concentration which is inhibitory to the production of GM‐CFC.
Bone marrow histology in myeloma: its importance in diagnosis, prognosis, classification and staging
Authors: Bartl R. , Frisch B. , Burkhardt R. , Moghadam A. Fateh‐ , Mahl G. , Gierster P. , Sund M. , Kettner G. 
Published: July 2010

Summary.A study has been made of 420 bone marrow biopsies from patients with multiple myeloma (220), idiopathic monoclonal gammapathy (50), reactive plasmacytosis (42) and solitary plasmacytoma (22). Histology and immunohistological parameters were more reliable than cytology in distinguishing a reactive from a neoplastic plasmacytosis. Histological variables were correlated with the clinical features of the patients to determine the factors which were of value in predicting prognosis. Plasma cell maturity and the extent of infiltration in the biopsy by myeloma cells proved to be highly significant in predicting the duration of survival. On the basis of these criteria multiple myeloma was classified into two types: plasmacytic of low‐grade malignancy and plasmablastic of high‐grade malignancy; and into three stages which accurately reflected the progression of the disease. We conclude that a bone biopsy provides useful information for the diagnosis, classification and staging of patients with multiple myeloma.
The differential ultrastructural localization of immunoglobulin heavy and light chains in human haematopoietic cell lines
Authors: Newell D. G. , Hannam‐Harris A. , Karpas A. , Smith J. L. 
Published: July 2010

The ultrastructural localization of immunoglobulin heavy and light chains has been investigated in nine haematopoietic cell lines, using a technique which involves the treatment of lightly prefixed cells with saponin to allow penetration of the antibody‐peroxidase conjugate. The synthesis and secretion of immunoglobulin was also studied in these cell lines.Immunoglobulin was found to be localized in the cisternae and on the membranes of the rough endoplasmic reticulum, perinuclear space and/or Golgi apparatus. In each case staining for heavy chains was weak or absent in the perinuclear space while staining for light chains was usually strong. Additionally in three cell lines immunoperoxidase staining indicated that heavy chains were absent from the Golgi apparatus despite the observed presence of light chains in the Golgi apparatus and the secretion of combined immunoglobulin into the supernatant. The results obtained suggest compartmentalization of the synthesis of light and heavy chains and indicate that the technique of immunoelectron microscopy may significantly contribute to an understanding of the mechanisms involved in immunoglobulin synthesis, intracellular transport and secretion.
No evidence for gamma‐interferon mediated haematopoietic inhibition by T cells in aplastic anaemia: an observation in the course of immunosuppressive therapy
Authors: Hanada Takashi , Yamamura Hideshi , Ehara Takao , Iwasaki Nobuaki , Shin Reiko , Nakahara Satoko , Takita Hitoshi 
Published: July 2010

SummaryTwo patients with aplastic anaemia were treated with immunosuppressive agents and peripheral blood T cells were cryopreserved serially. Inhibitory activity of T cells to autologous CFU‐E, γ‐IFN production by T cells and T cell subpopulations were assayed after remission. Inhibitory activity to autologous CFU‐E was not correlated with the numbers or ratios of T cell subpopulations. γ‐IFN production by T cells were within the normal range when inhibitory activity was found. In addition, γ‐IFN production increased after haematopoietic recovery. These findings suggest that γ‐IFN is not a soluble mediator of T cell‐mediated haematopoietic inhibition in aplastic anaemia.
Cloning of human erythroid progenitors (BFU‐E) in the absence of fetal bovine serum
Authors: Migliaccio Giovanni , Migliaccio Anna Rita 
Published: July 2010

SummaryWe describe culture conditions which enabled us to clone early human erythroid progenitors (BFU‐E) in the absence of fetal bovine serum (FBS). Our medium, which is chemically fully defined, supports proliferation and differentiation of erythroid progenitors comparable to that in FBS‐supplemented cultures. Furthermore, it allows cloning of BFU‐E from all human haemopoietic tissues (adult marrow, embryonic liver and yolk sac) and adult or perinatal blood. This system should facilitate the investigation of human erythroid differentiation in vitro (e.g. cell‐cell interaction. mechanism of action of haemopoietins and inhibitors, regulation of Hb synthesis) as well as the mechanisms underlying myeloproliferative disorders. As an example, we have found that recombinant Ep shows, in this culture system, the same dose response as Ep from conventional sources.
Androstane therapy to treat aplastic anaemia in adults: an uncontrolled pilot study
Authors: Gardner Frank H. , Juneja Harinder S. 
Published: July 2010

Summary.During the past 9 years, 43 adult patients with severe aplastic anaemia have been treated with a combination of 3a etiocholanolone and nandrolone decanoate in an uncontrolled pilot study. Eleven patients were considered acute and the remainder as chronic severe aplasia. 50% (22 patients) had a haematopoietic recovery and 40% have had a sustained remission varying from 1‐5 to 8 years. Three patients who did not respond to 3a etiocholanolone had a haematologic reponse when treated with the isomer 3β etiocholanolone. The recovery did not appear to be associated with age or duration of marrow aplasia. In view of the reproducible chemical structure of these androstanes, we believe this group of steroids should be evaluated further in the treatment of severe aplastic anaemia.
α, β and γ T‐cell receptor genes: rearrangements correlate with haematological phenotype in T cell leukaemias
Authors: Foroni L. , Foldi J. , Matutes E. , Catovsky D. , O'Connor N. J. , Baer R. , Forster A. , Rabbitts T. H. , Luzzatto L. 
Published: July 2010

SummaryWe have studied the arrangement of the α, β and γT cell receptor (TCR) genes in 27 patients with T cell lymphoproliferative disorders. Nine patients had acute lymphoblastic leukaemia (T‐ALL), nine patients had prolympho‐cytic leukaemia (PLL), six patients presented with a T‐CLL/T‐lymphocytosis syndrome, two patients had Sezary syndrome (SS) and one patient had HTLV‐I positive T‐cell leukaemia/ lymphoma (ATLL). α TCR gene rearrangement could be demonstrated by the use of three available probes in only one case. By contrast, both β and γ TCR gene rearrangement could be demonstrated by Southern blot analysis of DNA samples digested with appropriate restriction enzymes in the majority of cases. In general, when rearrangements were present they involved both alleles. The proportion of rearranged chromosomes was lower in T‐ALL than in other forms of T‐cell leukaemia and it was lower in cases with the CD4 —/ CD8+ phenotype than in those with a CD4 +/CD8—phenotype. In three out of 34 cases of B‐cell leukaemia the TCR β‐gene but not the TCR γ‐gene was rearranged, just as in two out of 26 cases of T‐cell leukaemia the immunoglobulin (Ig) heavy chain but not the light chain genes were rearranged.These data suggest that development of the machinery required for gene rearrangement may precede commitment to B or T cell lineage. The use of this technique is especially useful for the classification of cases of ALL in which the cells are negative with respect to most current phenotypic markers and in cases of T cell lymphocytosis in which the finding of a gene rearrangement identifies a monoclonal cell population.
A multivariate analysis of prognostic factors in chronic myelomonocytic leukaemia according to the FAB criteria
Authors: Ribera Josep‐Marǐa , Cervantes Francisco , Rozman Ciril 
Published: July 2010

Summary.In an attempt to characterize the prognostic significance of the main initial clinical, haematological and biochemical features of chronic myelomonocytic leukaemia according to the FAB criteria, 29 such patients were analysed by means of univariate and multivariate statistical methods. At the time of the analysis 21 patients had died, with a median survival for the overall series of 8‐2 months. The univariate analysis identified three parameters associated with poor prognosis: high monocyte counts, low platelet counts and splenomegaly. When all the initial features wereincluded in a multiple regression model, only high monocyte counts and spleen enlargement retained their unfavourable prognostic influence (P=0‐002 and P = 002, respectively). Based on the presence or not of these prognostic factors, two populations of patients with different survival (median survival 5‐6and 16‐5months,respectively,P< 0‐005)could be identified. It seems therefore that monocytic proliferation serves not only as the diagnostic marker for chronic myelomonocytic leukaemia but also as the most important feature in the assessment of the patient's prognosis.
Room temperature ADP induced first stage hyperaggregation of human blood platelets: a previously undescribed phenomenon and its relationship to spontaneous cold induced platelet aggregation
Authors: Cohen Ian J. , Fuchs Jacob , Kaplinski Chaim , Krugliak Judith , Stark Batia , Vogel Ruth , Weinberger Itzhak , Rotenberg Zvi , Agmon Jacob , Jerushalmyi Zohara , Zaizov Rina 
Published: July 2010

Summary.ADP induced human platelet aggregation was shown to be accentuated when tested at 20‐30°C as increased sensitivity and as a greater change of optical density although second stage aggregation and the release reaction did not occur. This previously undescribed phenomenon is defined as room temperature ADP induced first stage hyperaggregation. Aggregation, which occurs under the above mentioned conditions with a quantity of ADP insufficient to maintain the aggregation (usually less than 1 5 μm), is reversible when the temperature is raised to 3 7°C. After rewarming to these temperatures, second stage aggregation appeared in the presence of larger quantities of ADP (usually more than 2 μm) and could be blocked by aspirin. The absence of the release reaction was demonstrated with a lumi‐aggregometer. Spontaneous cold induced platelet aggregation seen after chilling platelets to 0‐4°C is shown to be a distinct phenomenon.
Phenotypic and genotypic analysis of chronic myelogenous leukaemia with T lymphoblastic and megakaryoblastic mixed crisis
Authors: Yasukawa Masaki , Iwamasa Kikue , Kawamura Shinichi , Murakami Satoshi , Takada Kiyonori , Hato Takaaki , Shiosaka Takahiko , Tamai Tomonori , Fukuoka Tamotsu , Fujita Shigeru , Kobayashi Yuzuru 
Published: July 2010

SummaryA case of blast crisis in chronic myelogeneous leukaemia (CML) in which two distinct cell lineages were involved is presented. The phenotype of blasts in lymph nodes was T11 (CD2)+, Ia+, TdT+, suggesting T cell lineage. On the other hand, blasts in bone marrow and peripheral blood expressed platelet glycoprotein IIb/IIIa complex on their surface, suggesting megakaryocyte lineage. Cytogenetic analysis of lymph node and bone marrow cells revealed the abnormalities. inv(7) (p15q34) and t(1;3) (q23;q21), respectively, as well as the presence of the Ph1 chromosome in both cell types. Rearrangement of the T cell receptor β‐chain gene was detected in lymph node blasts, although blast cells in peripheral blood showed a germ line configuration. The involvement of T cell and megakaryocyte lineages in the blast crisis phase of CML was confirmed in our phenotypic and genotypic analysis, and the pathogenic association between blast crisis lineages and the additional chromosome abnormalities present is discussed.
Fibrinogen Aarhus and factor XIII induced polymerization and gel formation
Authors: Hessel Birgit , Adamson Lars , Procyk Roman , Therkildsen Lisbeth , Stenbjerg Stenar , Blombauck Birger 
Published: July 2010

SummaryFibrinogen Aarhus is an abnormal fibrinogen for which the clotting time with thrombin is greatly prolonged both in plasma and in the isolated fibrinogen. The whole blood clotting time is only slightly prolonged. The patient with this fibrinogen has no bleeding tendency. In this report we have investigated fibrinogen Aarhus in two alternative, thrombin independent polymerization and gelation pathways. These pathways are the factor XIII dependent oligomerization and gelation of fibrinogen, and heteropolymer (fibrinogen‐fibronectin) formation which also is catalysed by factor XIII. Both of these reactions are qualitatively the same in fibrinogen Aarhus as in normal fibrinogen, but the rate of oligomerization is somewhat slower in fibrinogen Aarhus. This may depend on impaired association between factor XIII and fibrinogen Aarhus.
Determination of the growth fraction in monoclonal gammopathy with the monoclonal antibody Ki‐67
Authors: Lokhorst Henk M. , Boom Saskia E. , Terpstra Wim , Roholl Paul , Gerdes Johannes , Bast Bert J. E. G. 
Published: July 2010

The monoclonal antibody Ki‐67 reacts with a nuclear antigen that is present only in proliferating cells. The proportion of Ki‐67 positive cells may therefore serve as a reliable measurement for the growth fraction in normal and neoplasmic cell populations. We have tested the significance of the MoAb Ki‐67 in the classification of monoclonal gammopathy and compared the results with the plasma cell labelling index. In benign monoclonal gammopathy the percentage of Ki‐67 positive plasma cells (median 1.6%) was significantly lower than in untreated multiple myeloma (median 9.6). Among the patients with more than 10% Ki‐67 positive plasma cells there were some very short survivors.The largest growth fractions (median 41.8%) were found in patients with relapsing multiple myeloma indicating here a different growth pattern more resembling the high‐grade lymphomas. A linear correlation between the proportion of Ki‐67 positive plasma cells and the labelling index was not found. Determination of the plasma cell growth fraction with the monoclonal antibody Ki‐67 in monoclonal gammopathy may help to discriminate benign monoclonal gammopathy from multiple myeloma and will probably identify a subgroup of multiple myeloma patients with a poor prognosis, including those with relapsing multiple myeloma.
A reversible defect of platelet PDGF content in myeloproliferative disorders
Authors: Baglin T. P. , Price S. M. , Boughton B. J. 
Published: July 2010

Platelet PDGF (platelet derived growth factor), platelet associated IgG and plasma levels of circulating immune complexes were measured in patients with chronic myeloproliferative disorders (primary myelofibrosis, primary proliferative polycythaemia and essential thrombocythaemia). Platelet PDGF was low in 11/12 patients, immune complexes were elevated in 11/16, and PlAIgG was elevated in all 14 patients in whom it was measured. There was no significant correlation between platelet PDGF and plasma levels of immune complexes (r= ‐0.5, P>0.1).Treatment with busulphan and prednisolone for 2–3 months restored normal levels of platelet PDGF and suppressed plasma immune complex levels. Plasmapheresis lowered levels of immune complexes but had no effect on platelet PDGF. These results indicate that the low platelet PDGF levels in chronic myloproliferative disorders represent a reversible defect which is not directly related to the presence of immune complexes.
Two novel polyadenylation mutations leading to β+‐thalassaemia
Authors: Jankovic L. , Efremov G. D. , Petkov G. , Kattamis C. , George E. , Yang K‐G. , Stoming T. A. , Huisman T. H. J. 
Published: July 2010

SummaryIn an ongoing effort to identify point mutations causing β‐thalassaemia, we have found two previously unreported mutations which are located in the Poly A site of the β‐globin gene. The screening programme used amplified DNA and dot‐blot hybridization with several 32P‐labelled oligonucleotide probes. DNA samples which remained unidentified by this methodology were subjected to sequencing with 32P‐labelled primers and modified T7 DNA polymerase. The newly discovered mutations were confirmed by the dot‐blot hybridization technique. One type concerned an AATAAA→ÁTGAA mutation in the polyadenylation site and was found in one family from Yugoslavia (including one patient with the C→T mutation at codon 29 in trans), one from Bulgaria (the patient had the G→A mutation at IVS‐I‐110 in trans), and one from Greece (this patient had the C→G mutation at IVS‐II‐745 in trans). Haematological data for three simple heterozygotes suggested a rather mild β+‐thalassaemia. The second type involved an AATAAA→ÁTAGA mutation and was found in one family from Malaysia. The propositus had the βE mutation on the other chromosome, was originally diagnosed as mild Hb E‐β+‐thalassaemia, and had Hb A and Hb E percentages which were nearly the same.
Characterization of polymorphic markers in the von Willebrand factor gene and pseudogene
Authors: Bernardi F. , Marchetti G. , Casonato A. , Gemmati D. , Patracchini P. , Legnani C. , DeRosa V. , Girolami A. , Conconi F. 
Published: July 2010

SummaryThree TaqI restriction fragment length polymorphisms (RFLP) detected by the central portion of von Willebrand factor cDNA, which recognizes the true gene and in addition pseudogenic sequences, were characterized and mapped. Small cDNA fragments which hybridized with DNA from families with von Willebrand disease were used.Two of the RFLP, recognized by 1.7 and 0.45 kb cDNA fragments, are not in linkage either with von Willebrand disease or with RFLP located in the von Willebrand factor (vWF) gene, which indicates their pseudogenic location. These markers located in 22q11, near to the bcr gene, provide new tools for the study of several somatic and constitutional alterations affecting this chromosomal region.The third RFLP is recognized by a cDNA fragment corresponding to the N‐terminal portion of mature vWF and is localized in the true gene. Since significant linkage disequilibrium with other informative RFLP is not present, this marker contributes to the definition of family haplotypes associated with von Willebrand disease.
Elevated platelet‐free calcium in uraemia
Authors: Moosa A. , Greaves M. , Brown C. B. , MacNeil S. 
Published: July 2010

SummaryBleeding complications in uraemia are not uncommon. The pathogenesis of haemorrhage in uraemia is still a matter of controversy and the pattern of bleeding suggests a defect of primary haemostasis. Platelet aggregation and biochemistry, including calcium levels, have been studied; however, the results are controversial. We have examined platelet aggregation, platelet‐free calcium and calmodulin in platelet‐rich plasma because of the significant role of calcium and calmodulin in regulating platelet and other cells’functions. Platelet aggregation in uraemic subjects was similar to that of controls. Platelet basal free cytosolic calcium and platelet calcium in response to 10 μM Ca + + ionophore A23187 in eight subjects with uraemia were 117 ± 33 nM and 2025 ± 398 nM (mean ± SEM) respectively. By contrast in seven matched healthy controls basal calcium and ionophore‐stimulated calcium values were 47 ± 14 nM and 1354 ± 414 nM, significantly less than in the patients with uraemia (P < 0.05). The sensitivity of uraemic platelets to A23187 was similar to that of controls. Calmodulin activity in platelet‐rich plasma of 12 subjects with uraemia showed no significant difference from that of controls [1.86 ± 0.29 μg/ml (mean ± SEM) and 2.0 ± 0.37 μg/ml (mean ± SEM) respectively] We conclude that despite elevation of platelet calcium in uraemia, which may be due to a plasma factor such as parathyroid hormone, platelet aggregation is normal and bleeding in uraemia is more likely to be due to other factors, including the effect of reduced haematocrit on platelet endothelial interaction. Disturbances in platelet calcium cannot explain the bleeding manifestations in uraemia but warrant further investigation in order to identify the pathogenic mechanisms responsible.
Seasonal changes in red blood cell parameters
Authors: Kristal‐Boneh E. , Froom P. , Harari G. , Shapiro Y. , Green M. S. 
Published: July 2010

Summary. Seasonal changes in blood count (haematocrit and haemoglobin values) have been described in studies of small samples. Since Israel is characterized by high summer temperatures, we attempted to determine whether there are circannual changes in blood count, and how they are influenced by mean monthly temperature.In a cross‐sectional study, venous blood was drawn from 4317 male employees in industry aged 20–64 years. Blood count values were analysed by month. To estimate possible changes in serum osmolality. serum electrolytes were also examined.Values of haemoglobin and haematocrit in August were significantly lower than during the rest of the year. Circan nual rhythms explained between 10% and 57% of the variances of all the variables as assessed by cosinor rhythmo‐metry, although they had wide confidence intervals and most were not statistically significant. The lower mean haematocrit in August reflected reduction in mean corpuscular volume (MCV) rather than a decrease in red blood cell count (KBC).The change observed in haematocrit and haemoglobin in August is possibly related to heat acclimatization rather than to endogenous sinusoidal circannual rhythms. Both plasma and red cell volume expansion appear to occur. Possible seasonal changes should be taken into account in evaluation of blood counts.