bluuebunny/crossref_metadata_2025_split huggingface dataset:
CD 7 positive acute myeloid leukaemia: a subtype associated with cell immaturity
Authors: Coco Francesco Lo , Rossi Giulio De , Pasqualetti Daniela , Lopez Manuela , Diverio Daniela , Latagliata Roberto , Fenu Susanna , Mandelli Franco 
Published: March 2008

SummarySeventeen patients with acute myeloid leukaemia (AML) whose blasts co‐expressed the T‐cell associated CD7 antibody were identified among 160 consecutive AML cases. Fourteen had FAB defined AML according to morphocyto‐chemical criteria, whereas three patients were classified as ‘MO’ on the basis of immunophenotype. The incidence of CD7 positivity was particularly significant in the less differentiated subtypes MO and M1 compared with other FAB groups (P < 0.001). In all cases the myeloid determinants CD13 and/or CD33 were associated with CD7 expression. Other B‐lymphoid (CD10, CD19) or T‐lymphoid (CD2, surface and cytoplasmic CD3) markers were analysed and found to be negative. Five out of 15 cases examined were TdT+. Clonal rearrangements of the immunoglobulin heavy chain (IgH) and/or T‐cell receptor (TcR) β chain genes were identified in only three out of 13 cases. Among these, one out of five coexpressing TdT showed IgH rearrangement when analysed at the DNA level.Clinical features at presentation and response to induction therapy did not allow us to consider CD7 + AML patients as a distinct subgroup with prognostic significance. Our data indicate that CD7 expression is a common finding in immature AML, being generally found in the absence of other T‐cell features. Rather than suggesting the occurrence of ‘mixed leukaemia’, such cases confirm a broader spectrum of CD7 reactivity and its possible identification of a particular subset of myeloid progenitors.
Multiple Xbal polymorphisms for carrier detection and prenatal diagnosis of haemophilia A
Authors: Chan Vivian , Tong T. M. F. , Chan T. P. T. , Tang Mary , Wan C. W. , Chan F. Y. , Chu Y. C. , Chan T. K. 
Published: March 2008

SummaryThree Xbal restriction fragment length polymorphisms (RFLPs) can be detected using the factor VIII‐intron 22 probe (p482.6) in a Xbal‐KpnI double digest of genomic DNA. The Xbal (A) site had been reported by Wion et al (1986) to be in intron 22. while the two additional sites. Xbal (B) and Xbal (C), are shown here to be X‐linked and close to the Xbal (A) site. The frequencies of heterozygosity for these three sites are 0.49. 0.18 and 0.30 respectively. In 75 females the observed heterozygosity rate for the Xbal (A) site is 0.41 and this increased to 0.57 with the two additional sites. Care should be exercised when interpreting the Xbal RFLPs, since the 1.4 kb Xbal/Kpnl fragment and the 4.8 kb Xbal fragment are associated with both positive Xbal (A) and Xbal (B) sites. By the combined use of the multiple Xbal polymorphisms with the Bell site in intron 18, the carrier detection rate would increase to 67%. Four prenatal diagnoses had been performed using the multiple Xbal polymorphisms.
Bepridil protects sickle cells against the adverse rheological effects of cyclical deoxygenation
Authors: Johnston M. N. , Ellory J. C. , Stuart J. 
Published: March 2008

SummaryCalcium influx into sickle cells, with consequential activation of the Ca2+‐activated K+ efflux (Gardos) channel, is a potential cause of cellular dehydration and loss of deformability. Bepridil, a recently described inhibitor of the Gardos channel, was found at pharmacological concentration (1 μmol/1) to inhibit significantly (P<0.01) the loss of deformability when sickle cells were subjected to cycles of oxygenation‐deoxygenation for 15 h at 37°C. Bepridil also inhibited significantly (P < 0.005) the formation of irreversibly sickled cells. Drugs that preserve the K+ and therefore water content of erythrocytes are of potential value for hydrotherapy of sickle cell disease.
Decreased amount of the Rh antigen D in hereditary spherocytosis (HS)
Authors: Szymanski I. O. , Araszkiewicz P. , Odgren P. , Snyder L. M. 
Published: March 2008

SummaryThis study was done to determine whether hereditary spherocytosis (HS) red blood cells (RBC) have decreased amounts of Rh antigens. Initially we studied the RBC of five members of one family, two of whom had HS. Using automated quantitative haemagglutination tests, we demonstrated that HS RBC agglutinated less with Rh antisera of four specificities than did normal RBC, indicating that Rh antigens are decreased on HS RBC. In this family, the strength of other blood group antigens on HS RBC was estimated by manual titres and agglutination scores. No appreciable differences in the agglutination of HS and normal RBC were observed with non‐Rh antisera. To assess the strength of the D antigen more accurately, the number of D sites was quantitated on the RBC of 19 individuals with HS and 11 of their healthy relatives. HS RBC had 9209±4084 (mean±SD) D sites, whereas the normal RBC had 15 394±5763 D sites. These two means were significantly different (P < 0.01). HS RBC were also compared to normal RBC of unrelated individuals who had the same Rh pheno‐type. These analyses showed that HS RBC had about half of the normal number of D sites. Our data indicate that HS red cells have decreased amount of the Rh antigen D and probably also of other Rh antigens.
Pregnancy associated aplastic anaemia: a report of five cases and review of current management
Authors: Aitchison R. G. M. , Marsh J. C. W. , Hows J. M. , Russell N. H. , Gordon‐Smith E. C. 
Published: March 2008

SummaryThe occurrence of aplastic anaemia in pregnancy has been long recognized but its rarity has made it difficult to establish the relationship between the two conditions and the optimal management. We now report five cases of aplastic anaemia in pregnancy and offer some recommendations for treatment. In two patients the pregnancy was allowed to continue to term and the disease persisted post‐partum leading to death in one case. The other three patients had their pregnancies terminated; one subsequently deteriorated and died, two had spontaneous remissions of their aplasia. We suggest that patients presenting with severe aplastic anaemia in early pregnancy should be offered termination because this may be followed by haematological improvement. If haematological improvement does not occur allogeneic bone marrow transplantation (BMT) may be considered. Aplastic anaemia presenting in late pregnancy should be treated with supportive care until delivery. On the basis of our experience, antilymphocyte globulin may safely be given during pregnancy.
Red blood cell aggregation and sedimentation: the role of the cell shape
Authors: Reinhart Walter H. , Singh Ajit , Straub P. Werner 
Published: March 2008

SummaryThe influence of erythrocyte shape changes on the sedimentation rate was studied in vitro and in vivo. In vitro the highest sedimentation rate was observed with a slight degree of stomatocytosis (morphological index – 0.3; i.e. one red cell out of three being a stomatocyte I). With increasing degrees of stomatocytosis the sedimentation rate gradually decreased. Echinocytosis reduced the aggregation and sedimentation very drastically; the sedimentation rate was virtually zero when echinocytosis I or higher degrees were present. The influence of abnormal cell shapes occurring in vivo was studied in patients with an abnormal blood smear. It was found that a severely abnormal red cell morphology reduced the sedimentation rate in a standardized, fibrinogen‐rich plasma to about half. These results indicate that the shape plays a crucial role in the aggregation and sedimentation of red cells and they may contribute to the understanding of the interaction of red cells with other cells such as endothelium.
Antithrombin Sheffield: amino acid substitution at the reactive site (Arg393 to His) causing thrombosis
Authors: Lane D. A. , Erdjument H. , Flynn A. , Di Marzo V. , Panico M. , Morris H. R. , Greaves M. , Dolan G. , Preston F. E. 
Published: March 2008

SummaryA Sheffield family with a predisposition towards thrombosis has been shown to have a functional abnormality of antithrombin. The abnormality was detected as reduced heparin cofactor activity, with normal antigenic levels of antithrombin. Crossed immunoelectrophoresis performed in the absence and presence of heparin was normal. The antithrombin was isolated by heparin Sepharose affinity chromatography. It had normal mobility on SDS polyacrylamide gel electrophoresis. However, the second order rate constant of inhibition of thrombin was about half that of normal, and this was compatible with a heterozygous abnormality involving the reactive site. The antithrombin was further purified by chromatography on thrombin‐Sepharose (to remove the normal component), reduced, S‐carboxymethylated and fragmented with cyanogen bromide. A pool containing the reactive site region was digested with trypsin and the molecular size of peptides generated determined by fast atom bombardment mass spectrometry. The two peptides adjacent to the Arg393‐Ser394 bond of mass 2290 and 700 were almost absent from the mass spectrum, but an additional peptide of mass 2952 was present. Subdigestion with V8 protease reduced the mass of this peptide to 1748. These peptides generated by trypsin and V8 protease were almost identical to those obtained when another variant, antithrombin Glasgow, was treated in the same way (Erdjument et al, 1988). It is concluded that the molecular abnormality of antithrombin Sheffield is identical to that of antithrombin Glasgow, Arg393 to His.
Human monoclonal anti‐D antibodies. I. THEIR PRODUCTION, SEROLOGY, QUANTITATION AND POTENTIAL USE AS BLOOD GROUPING REAGENTS
Authors: Kumpel B. M. , Poole G. D. , Bradley B. A. 
Published: March 2008

SummaryEight monoclonal IgG antibodies to the Rh antigen D, produced by Epstein‐Barr virus transformed B‐lymphoblastoid cell lines from two individuals, have been assessed for their suitability as blood grouping reagents. All showed similar specificity and agglutinated all red cells with partial D antigens tested except category DVI cells. They gave strong reactions with Du red cells in indirect antiglobulin tests, and they all gave good reactions with R1R1, R2R2 and R0r cells in manual tests using antiglobulin, enzyme or albumin methods. Initial studies showed that some of the monoclonal antibodies worked well at high dilutions on a Technicon Autogrouper 16C when used for routine D‐typing of blood donors. Antibody production by the cell lines was stable for many months in continuous culture. These monoclonal antibodies may be useful diagnostic reagents.
The effect of fresh versus stored blood on post‐operative bleeding after coronary bypass surgery: a prospective randomized study
Authors: Wasser M. N. J. M. , Houbiers J. G. A. , D'Amaro J. , Hermans J. , Huysmans H. A. , Kinijnenburg G. C. van , Brand A. 
Published: March 2008

Summary. In a prospective study, 237 patients undergoing a primary coronary bypass operation were randomized to receive 2 units of fresh whole blood (study group) or stored (2–5 d) blood (control group) at the end of the extracorporeal circulation.Serious post‐operative bleeding necessitating a re‐thoractomy occurred in 4.2% of all patients with an equal distribution over the two groups. Post‐operative haemoglobin content and platelet counts were higher in the study group, but the differences were small and clinically not important. There were no differences in transfusion requirements, post‐operative blood losses and haemostatic parameters between the trial groups.At low post‐operative platelet counts (below 120.109 platelets/1) however, patients in the control group lost significantly more blood and had increased transfusion requirements compared with patients in the study group (7.1 versus 4.8 units). These differences must be attributed to qualitative platelet defects in the transfused units of stored blood.The small, clinically insignificant, differences in two laboratory parameters between the study and control groups, and the increased transfusion requirements of a subpopulation of patients with low platelet counts in the control group do not justify giving fresh blood or prophylactic platelet transfusions to coronary bypass patients.
Phenotypic changes on hairy cells exposed in vitro to interferons: a quantitative FACS study
Authors: Till K. J. , Cawley J. C. 
Published: March 2008

Summary. In vitro culture of hairy cells (HC) (five patients) with αIFN (100 U/ml) significantly enhanced MHC and CD22 antigen expression and reduced CD25, sIg and FMC7 positivity, together with consistent but not significant reductions in CD9, 19 and HC2. A sixth patient, who was refractory to the effects of αIFN in vivo, was also studied and none of these changes were seen in her HC when cultured with αIFN in vitro. γIFN (100 U/ml) produced a much less marked increase in MHC and reduced sIg. TPA (100 ng/ml) induced changes very similar to those observed with αIFN, α and γIFN had no effect on a range of other antigens in HCL (including CD23 and 38, and transferrin receptors) and also produced no significant antigenic changes in CLL cells.It is concluded that these findings are compatible with partial activation/maturation of HC by αIFN. It is suggested that the specificity of the phenotypic changes induced on HC by αIFN is a consequence of HC representing a stage of B‐cell development at which αIFN has specific immunomodulatory effects.
Human immunodeficiency virus detection: correlation with clinical progression in the Edinburgh haemophiliac cohort
Authors: Cuthbert R. J. G. , Ludlam C. A. , Rebus Selma , Peutherer J. F. , Aw D. W. J. , Beatson Dianne , Steel C. M. , Reynolds Billie 
Published: March 2008

Summary. HIV p24 antigenaemia and virus detection in cultures of peripheral blood lymphocytes were examined in 16 of 18 haemophiliacs infected with HIV by a single batch of Scottish National Blood Transfusion Service factor VIII concentrate. Six (38%) had p24 antigenaemia and 11 (69%) had positive lymphocyte cultures. All seven patients with serious HIV disease (CDC group IV) had positive lymphocyte cultures whereas four (57%) had p24 antigenaemia. Four of nine (44%) patients with asymptomatic HIV disease (CDC groups II and III) had positive cultures and two (22%) had p24 antigenaemia. Twenty‐eight of 36 samples from the symptomatic group were HIV culture positive compared with nine of 30 samples from the asymptomatic group (P < 0·001). None of 14 antibody negative haemophiliacs who also received the implicated batch of factor VIII had p24 antigenaemia or positive HIV cultures.The ability to detect HIV in cultured lymphocytes correlates with the clinical severity of HIV disease in this cohort.
Prevalence of monoclonal Ig with λ light chains in chronic myelocytic leukaemia
Authors: Guglielmi P. , Davi F. , Brouet J. C. 
Published: March 2008

SummaryLight chains from seven of eight monoclonal Ig detected in the serum of patients with chronic myelocytic leukaemia (CML) were of the λ isotype. Although the presence of monoclonal Ig in CML is distinctly unusual, this prevalence of λ chains is unlikely to be a mere coincidence. All patients had otherwise typical clinical, chromosomal and molecular (bcr rearrangements) features of classical CML. The λ locus appeared unaltered. Only two patients had an excess of dystrophic plasma cells suggestive of multiple myeloma.
Red cell glycolytic intermediates in normal, anaemic and transfused human fetuses
Authors: Lestas A. N. , Bellingham A. J. , Nicolaides K. H. 
Published: March 2008

SummaryNormal fetal ranges for red cell glycolytic intermediates at 18–24 weeks gestation, which are useful as reference values for the prenatal diagnosis of erythroenzymopathies, were established for the first time. Characteristic increases in glucose‐6‐phosphate (G6P), fructose‐6‐phosphate (F6P) and particularly fructose‐ 1,6‐diphosphate (FDP) suggest that there is no metabolic block at the phospho‐fructokinase (PFK) step of glycolysis as previously suggested by others for premature infants on the first day of life.Neither reticulocytosis nor anaemia consistently led to further increases in the early metabolites as occurs in adults. However, very large increases in G6P, F6P and particularly FDP may occur independently of anaemia and reticulocytosis. This suggests that activation of hexokinase (HK) and/or PFK can take place as in adults but the stimulus is probably different to adults.The 2,3‐DPG in normal fetuses is higher than in adults and increases still further in anaemic fetuses with or without transfusion of adult blood. The pattern of intermediates found in the fetus suggests that the controlling mechanism for the increased 2,3‐DPG may be an in vivo relative preponderance of PFK activity over that of pyruvate kinase (PK) rather than regulation of HK as proposed for adult subjects with anaemia or high altitude hypoxia.
Secretion of neutrophil secondary granules occurs during granulocyte‐macrophage colony stimulating factor induced margination
Authors: Devereux S. , Porter J. B. , Hoyes K. P. , Abeysinghe R. D. , Saib R. , Linch D. C. 
Published: March 2008

SummaryThe effects of recombinant human granulocytemacrophage colony‐stimulating factor (rhGM‐CSF) on neutrophil lactoferrin (LF) and transcobalamin (TC) 1 and 3 secretion were determined in vitro and during in vivo administration in humans. In whole blood, in vitro incubation with GM‐CSF reproducibly produced a rise in plasma LF concentration (P<0.05) whereas in purified neutrophils the results were variable. Exposure of whole blood to GM‐CSF also resulted in a significant rise in plasma TC 1 and 3 (190.60%, P<0.05). The response was dose dependent with maximal effect at GM‐CSF concentrations of 10 ng/ml and above. rhGM‐CSF was administered on seven occasions to six patients with malignant disease prior to chemotherapy. Plasma LF and unsaturated TC 1 and 3 levels rose significantly in each patient studied and the rise coincided with the initial neutropenia due to margination that occurs during infusions of rhGM‐CSF. Patients receiving rhGM‐CSF may therefore have hypofunctional neutrophils due to secondary granule depletion.
Two types of acquired idiopathic sideroblastic anaemia (AISA)
Authors: Gattermann Norbert , Aul Carlo , Schneider Wolfgang 
Published: March 2008

SummaryOn cytological bone marrow examination we distinguished between pure sideroblastic anaemia (PSA), which is confined to dyserythropoiesis, and refractory anaemia with ring sideroblasts (RARS), which is characterized by additional dysplastic features of granulopoiesis and/or megakaryopoiesis. In a follow‐up study of 94 patients with AISA diagnosed according to FAB criteria for myelodysplastic syndromes we found a striking difference in the risk of leukaemic transformation between PSA and RARS (5 year cumulative rate 1.9%v. 48%). Overall survival was much better in PSA than in RARS (5 year cumulative chance 69%v. 19%). Infections and haemorrhages were frequent causes of death in RARS but not in PSA.Bone marrow culture studies (CFU‐GM) were performed on 10 consecutive patients with PSA and RARS, respectively. RARS patients showed grossly impaired colony growth, typical of the myelodysplastic syndromes. Patients with PSA had persisting colony formation, even if moderately decreased in frequency, with numbers of CFU‐GM being inversely correlated with the degree of erythroid hyperplasia in the bone marrow.We conclude that on cytomorphological grounds AISA can be divided into pure (dyserythropoietic) sideroblastic anaemia (PSA) and a true myelodysplastic form (RARS), with both types differing considerably in terms of survival, risk of leukaemic transformation and findings on bone marrow culture (CFU‐GM).
A new human plasma cell line, Karpas 620, with translocations involving chromosomes 1, 11 and 14
Authors: Nacheva Elisabeth , Fischer Patricia E. , Sherrington Paul D. , Labastide Wayne , Lawlor Emer , Conneally Eibhlin , Blaney Clare , Hayhoe Frank G. J. , Karpas Abraham 
Published: March 2008

SummaryWe report here the establishment of a new cell line, Karpas 620 (K620), from the peripheral blood of an elderly woman with an IgG‐kappa plasma cell leukaemia (PCL). The line has the same hypotetraploid karyotype as the fresh cells from the patient. The cultured cells have the ultrastructural appearance of plasma cells with abundant rough endoplasmic reticulum (RER) and secrete kappa light chain. They are positive for surface antigens HLA DR, and WR17 (CD 37) and negative for CD1, CD3, CD4 and CD8. Using high resolution (HR) cytogenetic analysis it has been possible to identify all the marker chromosomes including several rearrangements commonly seen in malignancies of B cell lineage. These are a 14q + marker with a typical ‘Burkitt’morphology der(14)(pter→q32.3::8q24.1→qter) but with no reciprocal 8q–, and three translocations involving chromosome 11 at q 13 with partners other than chromosome 14, namely 1q32.1, 8q24.22 and 13q14.3. An earlier report of molecular studies on the DNA of K620 has shown a rearrangement near the region on 11q13 designated BCL‐1 (Rabbitts et al, 1988). This is the first report of a rearrangement in the region of 11q 13 in a cell line originating from a case of plasma cell leukaemia.
Paroxysmal nocturnal haemoglobinuria with coexisting deficiency of the ninth component of complement: lack of massive haemolytic attack
Authors: Yonemura Yuji , Kawakita Makoto , Koito Atsushi , Kawaguchi Tatsuya , Nakakuma Hideki , Kagimoto Tadashi , Shichishima Tsutomu , Terasawa Takashi , Akagaki Yohji , Inai Shinya , Takatsuki Kiyoshi 
Published: March 2008

SummaryA 47‐year‐old woman with paroxysmal nocturnal haemoglobinuria (PNH) was found to have an inherited deficiency in the ninth complement component (C9). In complement‐sensitivity lysis tests, 80% of her erythrocytes were markedly complement‐sensitive (PNH‐III). Laser cytofluorimetry with a monoclonal antibody against decay‐accelerating factor (DAF) revealed that 95% of her erythrocytes were DAF‐negative. Surprisingly, she has suffered only mild haemolysis and has never experienced massive spontaneous haemolysis. Gross haemoglobinuria and jaundice occurred only after receiving postoperative transfusions of whole blood. In her serum, C9 was not detectable either by immunological or by functional assays. Both the Ham test and the sugar water test using normal human serum or plasma yielded marked haemolysis of the patient's erythrocytes. When the patient's serum or plasma was used, only a trace of lysis was detected. Addition of purified human C9 to her plasma fully restored haemolysis. These observations indicated that C9 may play a critical role in haemolytic attacks in patients with PNH and that characteristic haemolysis in PNH may be tempered by coexisting C9 deficiency.
Monoclonal antibodies against myeloperoxidase are valuable immunological reagents for the diagnosis of acute myeloid leukaemia
Authors: Van Der Schoot C. Ellen , Daams G. Marjolein , Pinkster Jantina , Vet Raymond , Kr.vondem Borne Albert E. G. 
Published: March 2008

SummaryWhereas the diagnosis of acute lymphoid leukaemia greatly depends on immunophenotyping on the leukae‐mic cells, the diagnosis of acute myeloid leukaemia (AML) is still only based on morphological and cytochemical criteria. Here we describe that with a monoclonal antibody, directed against myeloperoxidase (MPO), the immunological diagnosis of AML is possible in most cases. A monoclonal antibody against lactoferrin (LF) was used to detect more mature myeloperoxidase‐containing cells. Of the cell samples tested from 206 different patients with AML, 95% were found to express myeloperoxidase in more than 15% of lactoferrin negative cells. Compared with other myeloid‐reactive monoclonal antibodies (VIM2, anti‐CD13, anti‐CD14, anti‐CD15 and anti‐CD33), a higher diagnostic sensitivity and specificity for AML was found. No significant correlation with the FAB classification was found. In most patients, more MPO‐positive cells were detected by the monoclonal antibody than by the cytochemical staining. This could be due to the recognition of enzymatically inactive precursor forms of myeloperoxidase by the antibody. The use of anti‐myeloperoxidase monoclonal antibodies for the diagnosis of AML has the advantage that objective quantification is possible.