bluuebunny/crossref_metadata_2025_split huggingface dataset:
Is the HLA‐linked haemochromatosis allele implicated in idiopathic refractory sideroblastic anaemia?
Authors: Simon M. , Beaumont C. , Briere J. , Brissot P. , Deugnier Y. , Edan G. , Fauchet R. , Garo G. , Ghandour C. , Grolleau J. , Grosbois B. , Krempf M. , Leblay R. , Mignon L. Le , Prise P. Y. Le 
Published: March 2008

SummaryIn order to assess the previously reported association of HLA‐linked idiopathic haemochromatosis with idiopathic refractory sideroblastic anaemia (IRSA), the prevalence of HLA‐A3 antigen in a group of 22 patients with IRSA was compared to that observed in healthy controls and in patients with homozygous idiopathic haemochromatosis and to that calculated for a population heterozygous for idiopathic haemochromatosis. The prevalence of A3 in patients with IRSA (0.23) was quite similar to that observed in controls (0.29) and significantly different from that observed in homozygous (0.73; P<10−5) and heterozygous (0.57; P<10−3) haemochromatosis. Serum iron, transferrin saturation, serum ferritin and liver iron concentration showed no difference in IRSA patients with or without A3. It is concluded that there is neither systematic association between the haemochromatosis allele and IRSA nor systematic implication of such an allele in the development of iron overload observed in IRSA.
Myelodysplastic syndromes in childhood: three case reports
Authors: Van Wering Elisabeth R. , Kamps Willem A. , Vossen Jaak M. , Van Der List‐Nuver Cathinka J. A. , Theunissen P. M. V. 
Published: March 2008

SummaryThree children with myelodysplastic syndromes (MDS) are described following the diagnostic criteria proposed by the FAB‐cooperative group. Two of the children were of Turkish origin. Two cases fit the criteria for ‘refractory anaemia with excess of blasts in transformation’. The other one is most consistent with ‘chronic myelomonocytic leukaemia’. The patients received ‘ANLL type’ induction. One died during induction, two were grafted, of whom one survives.
Relationships between blood product exposure and immunological abnormalities in English haemophiliacs
Authors: Lee C. A. , Bofill M. , Janossy G. , Thomas H. C. , Rizza C. R. , Kernoff P. B. A. 
Published: March 2008

SummaryAmongst 160 English haemophiliacs treated with clotting factor concentrates, abnormalities of T lymphocyte subset distribution (characterized by low T4/T8 ratios and high total T8 counts), low in vitro phytohaemagglutinin stimulation and raised serum IgG levels, were more common in patients with haemophilia A than B, in patients who had received heavier blood product exposure, and in adults rather than children. A slight reduction in lymphocyte and platelet counts was found in 26% and 17% of patients. In the sample of patients tested, serum α1‐thymosin levels were often raised, but β2‐microglobulin levels were usually normal. Fractionation procedures used to prepare clotting factor concentrates, and the amounts of concentrate used, are more likely to be causally related to these immunological abnormalities than the origins of source donor plasmas.
Persistence of host Langerhans cells following allogeneic bone marrow transplantation: possible relationship with acute graft‐versus‐host disease
Authors: Perreault Claude , Pelletier Micheline , Belanger Robert , Boileau Jacques , Bonny Yvette , David Michele , Gyger Martin , Landry Diane , Montplaisir Serge 
Published: March 2008

SummaryLangerhans cells (LC) are bone marrow‐derived dendritic antigen‐presenting cells found in the epidermis. In an effort to determine the origin (host versus donor) of LC at different intervals following bone marrow transplantation, we performed skin biopsies in 16 recipients of sex‐mismatched marrow. LC were identified using monoclonal antibody OKT6 in an indirect immunoperoxidase assay and their donor or host origin determined according to the presence or absence of Y body. The presence of Y‐positive (donor) LC could be demonstrated in all (6/6) skin biopsies of female recipients of male marrow tested between days 39 and 730 post‐transplant. Persistence of host LC in male recipients of female marrow was documented in all (6/6) recipients studied on day 39 and in two out of seven patients tested on day 120 post‐transplant. From day 365 onward, no residual host LC could be detected, suggesting that by this time all epidermal LC are donor‐derived. Our study demonstrates that host LC usually persist for 39 and up to 120 d following bone marrow transplantation. The relevance of this observation to the possible role of LC and other host dendritic antigen‐presenting cells in the graft‐versus‐host reaction is discussed.
A genetic marker for elevated levels of haemoglobin F in homozygous sickle cell disease?
Authors: Wainscoat J. S. , Thein S. L. , Higgs D. R. , Bell J. I. , Weatherall D. J. , Al‐Awamy B. H. , Serjeant G. R. 
Published: March 2008

SummaryTen patients with sickle cell (SS) disease from a Jamaican family were found to have unusually high levels of haemoglobin F for this population. Each of them has inherited one sickle cell gene on a chromosome characterized by an arrangement of restriction fragment length polymorphisms (haplotype) which is very rare in the Jamaican population. Genetic analysis of the family suggests that there is a determinant linked to the β‐globin gene cluster, characterized by this haplotype, which is responsible for increased haemoglobin F production in response to anaemia. Interestingly this particular haplotype appears to be common in patients with SS disease in eastern Saudi Arabia in whom a high level of haemoglobin F is the rule rather than the exception. Hence it is possible that this haplotype (++–++) acts as a genetic marker for elevated levels of haemoglobin F in sickle cell disease.
Thalassaemia genes in Peninsular Arabs
Authors: White J. M. , Byrne M. , Richards R. , Buchanan T. , Sheik‐Yousouf I. , White Y. S. , Frost B. 
Published: March 2008

SummaryThe haematological indices of Peninsular Arabs (United Arab Emirate Nationals, Yemeni and Omani) have been examined. The most outstanding feature, seen in 40–50% of all subjects, was one of hypochromia, microcytosis associated with erythrocytosis. In approximately 5% the hypochromia was severe (MCH 19–22 pg) and 20% of these were found to have β thalassaemia trait. In 10% of subjects the hypochromia was moderate (MCH 23–24 pg) and β thalassaemia was confirmed in only 10%. The remaining 25% had a mild hypochromia (MCH 25–27 pg) and no β thalassaemia was detected. The cause of the hypochromia in subjects with a normal Hb A2 (30% of the total population) is probably α thalassaemia, firstly because in those patients with an MCH of 19–24 pg the other haematological parameters are statistically the same as those with proven β thalassaemia and, secondly, in those with an MCH of 25–27 pg iron deficiency is not common (6% of the population). The degree and pattern of the distribution of hypochromia of the three major ethnic groups of the Peninsular Arabs could be explained either by different α and β thalassaemia genes being operative or by different degrees of inbreeding of the same genes.
Marrow cell necrosis in anorexia nervosa and involuntary starvation
Authors: Smith Robert R. L. , Spivak Jerry L. 
Published: March 2008

SummaryMarrow specimens from six patients with anorexia nervosa and three with involuntary starvation were examined for the presence of gelatinous transformation and marrow cell necrosis. Five of the patients had pancytopenia. Acanthocytes were present in the circulation of all nine. In only two patients was marrow cellularity adequate. Gelatinous transformation was present in every marrow specimen and was of a marked degree in eight. Marrow cell necrosis was identified in all nine marrow specimens. The necrosis was extensive in one specimen, focal in the others and limited to the areas of gelatinous transformation. Severe malnutrition was the single element common to all the patients.
Unclassified type of congenital dyserythropoietic anaemia (CDA) with prominent peripheral erythroblastosis
Authors: Bethlenfalvay N. C. , Hadnagy Cs. , Heimpel H. 
Published: March 2008

SummaryTwo unrelated cases of congenital dyserythropoietic anaemia (CDA) are described. They show striking similarities which could not be attributed to one of the well‐known types of CDA or any other congenital disease of the erythroid system. Both patients were followed for many years before and after splenectomy. There was a long‐lasting, prominent post‐splenectomy erythroblastosis, suggesting impairment of red cell denucleation. The type of heredity is unknown, and the enzymatic or molecular basis of the changes observed is not understood.
Endothelial cell proliferation in myelofibrosis
Authors: Reilly J. T. , Nash J. R. G. , Mackie M. J. , McVerry B. A. 
Published: March 2008

SummaryThe distribution of laminin and collagen type IV in normal human bone marrow appeared to be limited to a small number of capillary and sinusoidal basement membranes. Cellular elements including megakaryocytes showed a negative staining reaction. In contrast, myelofibrotic bone marrow showed markedly increased quantities of both laminin and collagen type IV. This was consistent with a proliferation of endothelial cells within the marrow as confirmed by factor VIII RAG staining. It is suggested that in myelofibrosis in addition to a marked fibrous reaction proliferation of vascular elements is also prominent.
The red cell antigens A, B, D, U, Ge, Jk3 and Yta are not detected on human granulocytes
Authors: Gaidulis Laima , Branch Donald R. , Lazar Gary S. , Petz Lawrence D. , Blume Karl G. 
Published: March 2008

SummaryWe report the inability to detect the following red blood cell antigens on human granulocytes: A, B, D, U, Gerbich (Ge), JkaJkb (Jk3) and Cartwright (Yta). To study each antigen, granulocytes were purified on density gradients, fixed in glutaraldehyde, and the uptake of specific antisera measured using two direct immunological techniques: 125I‐staphylococcal protein A (125I‐SPA) binding and avidin‐biotin‐complex (ABC) immunoperoxidase staining. Glutaraldehyde fixation was shown not to affect the antigenicity when the antisera were tested using red blood cells. Using three anti‐A, three anti‐B and three anti‐A,B antisera, our 125I‐SPA results of 47 tests with granulocytes from group A individuals and 39 tests with granulocytes from group B individuals indicate that A or B antigens are not expressed on human granulocytes. Tests using ABC were also negative with 37 and 36 granulocytes from group A or B individuals, respectively. In addition, no positive results using 125I‐SPA were obtained with granulocytes from individuals having antigen positive red cells when tested with two anti‐D (number of tests performed (n= 22), three anti‐Ge (n= 22), three anti‐U (n= 20), two anti‐Jk3 (n= 17), and three anti‐Yta (n= 25); control anti‐NA1 or ‐NB1 antisera were invariably positive. Also, using these antisera, no positive results were obtained by ABC except with one anti‐Yta antiserum which was positive with one of seven granulocytes tested. This anti‐Yta was also positive with three of 10 granulocytes by 125I‐SPA. This activity was shown to be due to a granulocyte‐specific antibody; adsorption of the antiserum with human granulocytes removed all activity against granulocytes but did not reduce the activity against red cells. Thus, our results are in agreement with recent reports which demonstrated the absence of the A, B and D antigens on human granulocytes. However, we have been unable to confirm previous reports which indicated the
The importance of thrombin inhibition for the expression of the anticoagulant activities of heparin, dermatan sulphate, low molecular weight heparin and pentosan polysulphate
Authors: Ofosu F. A. , Blajchman M. A. , Modi G. J. , Smith L. M. , Buchanan M. R. , Hirsh J. 
Published: March 2008

SummaryThe effects of standard heparin, three low molecular weight derivatives of heparin, dermatan sulphate and pentosan polysulphate on the intrinsic coagulation pathway were compared in order to evaluate the contributions of the anti‐factor Xa and anti‐thrombin activities to their anticoagulant activities. The anticoagulant potency was measured by the ability of each sulphated polysaccharide to inhibit the generation of thrombin activity in plasma. Similarly, the ability of the six sulphated polysaccharides to enhance the rates of inactivation either factor Xa or thrombin in defibrinated plasma containing calcium chloride and cephalin were also determined. Standard heparin was the only sulphated polysaccharide that could equally inhibit thrombin generation and enhance the inactivation of factor Xa and thrombin by plasma. Dermatan sulphate and pentosan polysulphate were more effective as inhibitors of thrombin generation than potentiators of factor Xa inactivation. The two smallest derivatives of heparin, which had high anti‐factor Xa (but low antithrombin) activity, were the poorest inhibitors of thrombin generation. Our results therefore suggest that only sulphated polysaccharides that enhance the inactivation of thrombin by plasma and/or inhibit the generation of thrombin activity in plasma are good anticoagulants. These two activities of sulphated polysaccharides appear to be good predictors of the relative antithrombotic potency in vivo.
Studies on immunological assay of vitamin K dependent factors.
Authors: Mikami S. , Tuddenham E. G. D. 
Published: March 2008

Summary. A radioimmunoassay (RIA) for factor VII was developed using 125I‐factor VII, anti‐factor VII rabbit serum and anti‐rabbit IgG goat serum. The lower limit of sensitivity in normal reference plasma was 3 × 10−3 units/ml. Although the level of factor VII antigen (VII:Ag) in normal plasma samples (= 20) 0.944·0.176 units/ml. correlated with that of factor VII coagulant activity (r= 0.89), VII:Ag level in paired normal sera showed a higher value (1.469 0.376 units/ml).The level of antigen according to RIA of factor VIIa activated by factor Xa increased 2.5‐fold compared with that of unactivated factor VII. It is suggested that the polyclonal anti‐factor VII produced in a rabbit had higher affinity for factor VIIa than for factor VII.In two out of seven patients with congenital factor VII deficiency, VII:Ag was detectable (0.04, 0.31 units/ml, respectively) whereas VII:C was less than 0.01 units/ml.In 12 warfarin‐treated individuals, VII:C showed a lower level (0.121 0.063 units/ml) that that of VII:Ag (0.518 0.186 units/ml). During 4 weeks observation after stopping warfarin, VII:C and VII:Ag reached normal levels in 1 week. However, VII:C did not reach equivalence with VII:Ag until 4 weeks had elapsed.
Increased frequency of DR2 in patients with aplastic anaemia and increased DR sharing in their parents
Authors: Chapuis B. , Von Fliedner V. E. , Jeannet M. , Merica H. , Vuagnat P. , Gratwohl A. , Nissen C. , Speck B. 
Published: March 2008

Summary68 patients with aplastic anaemia, their parents and healthy siblings were typed for HLA‐A, B and DR antigens. Among the patients there is an overrepresentation of DR2. The parents of affected children show an increased compatibility at the DR locus. This situation could favour the expression of recessive gene(s) involved in haematopoiesis and located in the HLA locus.
G6PD‐deficiency infectious haemolysis: a complement dependent innocent bystander phenomenon
Authors: Kasper Michael L. , Miller Wesley J. , Jacob Harry S. 
Published: March 2008

SummaryDramatic haemolysis may accompany viral hepatitis and pneumococcal pneumonia in G6PD‐deficient patients. Since red blood cells (RBCs) are richly endowed with receptors for activated complement, particularly C3b, we hypothesized that bulky, complement‐activating immune complexes (IC) consisting of microbes and antibody might attract granulocytes (PMNs), facilitating oxidative‘innocent bystander’RBC damage. Indeed, opsonization with only two type‐2 pneumococcus (PN3)/anti‐PN3/C3b complexes per RBC caused agglutination of RBC, a phenomenon termed immune adherence. Addition of as few as one PMN per 20 opsonized RBCs caused the glutathione (GSH) levels of co‐incubated G6PD‐deficient RBCs to fall by 30% (from 3.5 to 1.8 ± 0.8 μmoles GSH/g Hb) compared to identically incubated, but non‐opsonized, G6PD‐deficient RBCs. GSH levels remained normal (5.2 ± 0.4 μmoles/g Hb) in PMN‐exposed opsonized normal RBCs. GSH depletion in G6PD‐deficient RBC was directly related to disease severity—falling a mean 33% in RBCs from two Black G6PD A‐ subjects but 59% in two Caucasian G6PD deficient RBCs. Prevention of C3b generation (with 10 mm EDTA) during opsonization abrogated both immune adherence and PMN‐mediated GSH decline in oxidant‐sensitive cells. Similarly, removal of C3b receptors by brief trypsin incubation of RBCs eliminated immune adherence and GSH decline. Thus, both phenomena are dependent on IC complement activation and subsequent binding of the bacterial IC to the RBC complement receptors. Although clearance of IC by RBCs may be beneficial in protecting other tissues from inflammatory damage, G6PD‐deficient RBCs are vulnerable to oxidants generated by juxtaposed phagocytes—cells attracted to, and stimulated by, the immune complex/C3b combination. It is suggested that this‘Good Samaritan’activity of RBCs may lead to haemolysis during periods of exuberant antibody response to microbes.
Biochemical characterization of human myeloperoxidase using three specific monoclonal antibodies
Authors: Morishita Yoshihisa , Morishima Yasuo , Ogura Mlchinori , Nagai Yoshiyuki , Ohno Ryuzo 
Published: March 2008

SummaryWe have developed three monoclonal antibodies (moAbs), MA1, MA3 and MB1, which react with different antigenic determinants of human myeloperoxidase (MPO). In MPO‐positive culture cell lines, HL‐60 and NKM1, analysis of MPO by pulse‐chase experiments followed by immunoprecipitation with these moAbs revealed that MPO was composed of subunits of 59K, 18K and 14.8K dalton polypeptides which are plausibly derived from the 89 K precursor. MA1 and MB1 react with both the precursor and the mature forms of MPO. MA3 reacts with only the mature forms of MPO. Blocking experiments on MPO‐related functions revealed that the three moAbs could be divided into two groups. MA1 and MA3 inhibit MPO activities such as tetraguaiacol formation, iodide oxidation and luminol‐dependent chemiluminescence, while MB1 shows no such inhibition.
Infective complications of aplastic anaemia
Authors: Keidan A. J. , Tsatalas C. , Cohen J. , Cousins S. , Gordon‐Smith E. C. 
Published: March 2008

SummaryPatients with aplastic anaemia have a relatively specific immune defect—leucopenia with neutropenia. We have carried out a retrospective analysis of infective episodes in 11 patients with aplastic anaemia. 5723 follow‐up days accrued and 29 infective episodes were documented. Overall the number of infective episodes was significantly associated with the mean white cell and monocyte counts (r = 0.9, 0.02<P<0.05) but not with length of follow‐up, presentation values of white cell, neutrophil and monocyte counts, or mean neutrophil count. The patients appeared to divide clinically into two groups, those at low risk (seven patients) and those high risk (four patients) of infection. Patients in the high risk group had significantly more infections (P = 0.01) and significantly lower monocyte counts (0.02 <P<0.05) than patients in the low risk group. These results are in contrast to similar studies in patients with chemotherapy‐induced neutropenia; in our patients the overall rate and severity of infection was low, Gram negative infections were uncommon and monocytopenia appeared to be of greater importance than neutropenia in determining susceptibility to infection.
Platelet‐derived growth factor stimulates growth of highly enriched multipotent haemopoietic progenitors
Authors: Michalevicz Rita , Katz Fay , Stroobant Paul , Janossy George , Tindle Robert W. , Hoffbrand A. Victor 
Published: March 2008

SummaryPlatelet‐derived growth factor (PDGF) has been shown to stimulate growth of normal and malignant fibroblasts, glial cells and smooth muscle cells. A growth promoting effect on human haemopoietic precursors has also been described, but the interpretation of this haemopoietic proliferative response to PDGF has been hampered by the lack of purity of the target population. In this study we show that PDGF promotes growth of early bone marrow haemopoietic progenitors depleted of either monocytes or T lymphocytes which are known to influence haemopoiesis. Moreover, the action of PDGF is even increased on a highly enriched BI‐3C5 early bone marrow population. BI‐3C5 is a novel monoclonal antibody which recognizes an antigen present on all multilineage colony‐forming cells (CFU‐mix) (Tindle et al, 1985). BI‐3C5 positively and negatively sorted fractions were obtained by fluorescence activated cell sorting (FACS) and PDGF was found to stimulate growth of CFU‐mix in the BI‐3C5‐positive fraction (consisting of only 4–6% of the marrow population), the effect being more marked than that on unsorted bone marrow. The results suggest that the product of the cellular proto‐oncogene c‐sis (the putative structural gene for the β chain of PDGF) may play a regulatory role in the in vivo proliferation of multipotent haemopoietic progenitors.
Prediction of response of patients with acute nonlymphocytic leukaemia to remission induction therapy: use of clinical measurements
Authors: Preisler H. D. , Priore R. , Azarnia N. , Barcos M. , Raza A. , Rakowski I. , Vogler R. , Wlnton E. L. , Browman G. , Goldberg J. , Gottlieb A. J. , Grunwald H. , Rai K. , Mlller K. , Brennan J. , Chervenick P. , Joyce R. , Tricot G. 
Published: March 2008

SummaryTwo hundred patients with acute nonlymphocytic leukaemia received remission induction therapy consisting of cytosine arabinoside and an anthracycline antibiotic. Analysis of the pretherapy characteristics of the patients demonstrated that patient age was the most important factor in determining whether or not the patient would survive remission induction therapy. Assessment of the characteristics of the bone marrow after 6 d of therapy permitted the recognition of patients who were likely to fail to enter remission because of persistent leukaemia. Taken together, these observations demonstrate that it is possible to identify patients for whom conventional chemotherapy is not likely to be of benefit either because it is too intensive or because it is not intensive enough to produce a complete remission.
Production of anti‐cytomegalovirus antibody following T‐cell depleted bone marrow transplant
Authors: Wimperis J. Z. , Berry N. J. , Brenner M. K. , Grundy J. , Hoffbrand A. V. , Griffiths P. D. , Prentice H. G. 
Published: March 2008

SummaryThe serological status to cytomegalovirus (CMV) was examined in 24 patients with no detectable CMV excretion, following T‐iymphocyte depleted bone marrow transplantation. The results show that seropositive recipients continue to produce CMV antibody regardless of the serological status of the donor but that seronegative recipients fail to produce CMV antibody even when the donor is seropositive. These findings suggest that when an effective CMV vaccine becomes available vaccination of the donor would be unlikely to confer protection on the recipient but that vaccination of the recipient may achieve this.
Diagnostic significance of detecting pseudo‐Pelger‐Huet anomalies and micro‐megakaryocytes in myelodysplastic syndrome
Authors: Kuriyama K. , Tomonaga M. , Matsuo T. , Glnnai I. , Ichimaru M. 
Published: March 2008

SummaryTo investigate the kind of morphological dysplastic changes important in the diagnosis of myelodysplastic syndrome (MDS), we examined 50 patients with MDS and 86 control subjects. Micromegakaryocytes and pseudo‐Pelger‐Huët anomalies were detected in 46 patients (92%) with MDS, and were also found in the differentiated types of acute myeloid leukaemia and chronic myeloid leukaemia, but not in other haematological disorders, or healthy subjects. We believe that evaluation of these changes would be helpful in diagnosing MDS.