bluuebunny/crossref_metadata_2025_split huggingface dataset:
Platelet acquired defect in PDGF and β thromboglobulin content in hairy cell leukaemia: improvement after interferon therapy
Authors: Dupuy E. , Sigaux F. , Bryckaert M. C. , Tobelem G. , Castaigne S. , Flandrin G. , Degos L. , Caen J. P. 
Published: March 2008

SummaryTo investigate the platelet contribution to the development of myelofibrosis in hairy cell leukaemia (HCL), we have studied two platelet α granule components in 15 patients with HCL before chemotherapy: mitogenic activity was measured by 1H thymidine incorporation in BALB/C 3T3 cells and β thromboglobulin (βTG) assayed by radioimmunoassay (RIA). Platelet mitogenic activity and βG content were significantly decreased in the patients as compared to the control subjects. The nine patients who were treated with recombinant human interferon (IFNαA) were restudied after 4 months of therapy. The levels of both mitogenic activity and βG platelet content were significantly increased after IFNα‐treatment with a complete response in five of the nine treated patients, a partial response in two and no response in the two others. HCL seemed therefore to be responsible for an acquired platelet α granule defect. As in the grey platelet syndrome a relationship between this abnormal platelet granule storage and the development of myelofibrosis is suggested in HCL.
Immunohistochemical determination of growth fractions in human permanent cell lines and lymphoid tumours: a critical comparison of the monoclonal antibodies OKT9 and Ki‐67
Authors: Pileri Stefano , Gerdes Johannes , Rivano Maria , Tazzari Pier Luigi , Magnani Monica , Gobbi Marco , Stein Harald 
Published: March 2008

Summary.OKT9 and Ki‐67 monoclonal antibodies have recently been proposed as useful tools for evaluating the growth fraction in malignant tumours, with special reference to non‐1 Hodgkin's lymphomas. In particular, while the former is commonly thought to detect a transferrin receptor present on the cytoplasmic membrane of proliferating cells, the latter recognizes a nuclear antigen, which is expressed in Gl, S, G2 and M phase of continuously cycling elements.To further verify their reliability. OKT9 and Ki‐67 were applied to seven permanent cell lines (four myeloid and three B‐lymphoblastoid) and 100 lymphoid tumours (70 non‐Hodgkin's and 30 Hodgkin's lymphomas) phenotypically characterized on frozen sections.The results obtained showed that OKT9 and Ki‐67 cannot be employed as equivalent means in assessing the growth fraction. In fact, OKT9 is directed to a transferrin receptor which is not only expressed by proliferating cells, but also by some resting elements. On the other hand. Ki‐67 provides a nuclear, easily detectable positivity which is restricted to proliferating cells only. Therefore, it seems to represent the only monoclonal which can confidently be employed in the assessment of the growth fraction.Furthermore, the present study underlines that the immunocytochemical analysis of the proliferation rate in tumours gives similar information to the radionucleide uptake assay, while it represents a more sensitive method than the cytofluorimetric evaluation of the DNA content.
Superoxide dismutase, glutathione peroxidase and catalase in the red cells of patients with malignant lymphoma
Authors: Bewick M. , Coutie W. , Tudhope G. R. 
Published: March 2008

Summary.Erythrocyte superoxide dismutase, glutathione peroxidase and catalase activities have been measured in patients with untreated malignant lymphomas. Marked deficiencies of superoxide dismutase (P<0.001) and glutathione peroxidase (P<0.001) were found, whereas catalase levels were normal. There was no apparent difference in enzyme activities between the different histological types of lymphoma.
Osmotic properties of human erythrocytes treated by phospholipase A2 from bee venom
Authors: Vaysse Jenny , Pilardeau Paul , Gattegno Liliane , Garnier Michel 
Published: March 2008

Summary.During treatment of human red cells with phospholipase A) from bee venom, a linear increase of the MCV and of the osmotic fragility occurs in parallel with the cleavage of the accessible phospholipids. However, even after maximal hydrolysis, i.e. degradation of up to 65% of the phosphatidylcholines and up to 6% of the phosphatidylethanolamines, almost no haemolysis is observed and the median corpuscular fragility is only 7% higher than that of control cells incubated without enzyme. Addition of albumin to the medium results in an important rise of the susceptibility to hypotonic saline solutions. Osmotic fragility curves obtained with red cells submitted to mild phospholipase action show evidence of subpopulations of cells with various sensitivities to osmotic lysis. This phenomenon can be partly explained by the heterogeneity of the cleavage intensity among the cell population. This hypothesis is supported by the studies of the lipid composition of phospholipase treated red cells fractionated according to their sensitivity to hypotonic lysis or to their size.
Peripheral blood stem cell autografts in the treatment of lymphoid malignancies: initial experience in three patients
Authors: Bell A. J. , Figes Ann , Oscier D. G. , Hamblin T. J. 
Published: March 2008

SummaryLarge numbers of circulating haemopoietic progenitor cells were collected from three patients with lymphoid neoplasms for the purpose of haemopoietic reconstitution following intensive chemotherapy or chemoradiotherapy. Each patient underwent four to six leukaphereses at a time when the circulating stem cell pool was expanded, 2‐3 weeks after the end of myelosuppressive chemotherapy. The peripheral blood mononuclear cells (PBMNC) thus obtained were assayed for granulocyte‐macrophage colony‐forming cells (CFU‐GM) and were cryopreserved in liquid nitrogen. Two patients were autografted with PBMNC containing 60 x 104 and 76 x 104 CFU‐GM/kg body weight respectively. Both these patients showed prompt engraftment which is stable at + 10 and + 6 months. A third patient who was autografted with PBMNC containing 33 x 104 CFU‐GM showed only temporary and incomplete engraftment. These observations confirm that the peripheral blood may be used as a source of haemopoietic stem cells for autografting. but criteria for predicting engraftment are at present uncertain.
Post‐transfusion purpura secondary to passive transfer of anti‐P1A1 by blood transfusion
Authors: Ballem Penny J. , Buskard Noel A. , Decary Francine , Doubroff Phillip 
Published: March 2008

Summary.A patient developed severe post‐transfusion purpura (PTP) following transfusion of two units of packed red blood cells. The timing of the patient's thombocytopenia suggested passive immunization rather than the typical anamnestic response associated with classical PTP. Investigation of the blood donors revealed one with evidence of a platelet specific antibody of high titre. This donor was typed as P1A1 negative and the antibody was shown to have anti‐P1A1 specificity.
A microplate ELISA for the detection of platelet alloantibodies: comparison with the platelet immunofluorescence test
Authors: Sintnicolaas Kees , Van Der Steuijt Koos J. B. , Van Putten Wim L. J. , Bolhuis Reinder L. H. 
Published: March 2008

SummaryAn enzyme‐linked immunoassay (ELISA) for the detection of platelet alloantibodies has been compared in detail with the platelet immunofluorescence test (PIFT). The ELISA appeared much simpler to perform than the PIFT. Both tests were comparable with regard to reproducibility and sensitivity. Alloantibodies were detected with ELISA and PIFT in 13 out of 14 patients who were refractory to random donor platelets. The value of these tests as a platelet crossmatch assay was determined in a retrospective comparison of the test results and the clinical transfusion responses expressed as the 1 h post‐transfusion platelet recovery. 39/41 (95%) negative ELISA crossmatches and 30/33 (91%) negative PIFT crossmatches appeared to be associated with a successful platelet transfusion, whereas 7/10 positive ELISA cross‐matches and 2/4 positive PIFT crossmatches appeared to be associated with transfusion failures. The high frequency of‘correct’negative tests indicates the importance of both assays in the prospective selection of compatible platelet donors for alloimmunized patients. However, because of its simplicity, the ELISA appears the method of choice for this purpose.
Late haematological complications in severe aplastic anaemia
Authors: Tichelli A. , Gratwohl A. , Würsch A. , Nissen C. , Speck B. 
Published: March 2008

137 patients with severe aplastic anaemia (SAA) were treated in Basel from 1976 to 1986. 34 underwent bone marrow transplantation (BMT) and 103 received antilymphocyte globulin (ALG) therapy. We have analysed the incidence of late haematological complications in both groups of patients.20 patients treated with ALG developed a late haematological complication. A myelodysplastic syndrome or frank leukaemia occurred in eight and paroxysmal nocturnal haemoglobinuria (PNH) in 13 patients. Nine of the 13 patients with PNH had clinical signs of haemolysis, four only had positive laboratory tests. One patient had PNH and acute leukaemia. The risk of developing a haematological complication increased continuously and reached 57% at 8 years. Neither PNH nor leukaemia occurred in patients treated with BMT. The increased survival rate and the long observation time after ALG therapy have revealed a new perspective of the prognosis of aplastic anaemia. Patients treated with BMT appear to be cured whereas those treated with ALG remain at risk for late complications.
Bone marrow transplantation (BMT) versus immunosuppression for the treatment of severe aplastic anaemia (SAA): a report of the EBMT* SAA Working Party
Authors: Bacigalupo A. , Hows J. , Gluckman E. , Nissen C. , Marsh J. , Van Lint M. T. , Congiu M. , De Planque M. M. , Ernst P. , McCann S. , Ragavashar A. , Frickhofen N. , Wursch A. , Marmont A. M. , Gordon‐Smith E. C. 
Published: March 2008

This is an analysis of 509 patients with severe aplastic anaemia (SAA) treated in Europe between 1981 and 1986; 218 patients were treated by allogeneic bone marrow transplantation (BMT) from HLA identical sibling donors and 291 with immunosuppressive therapy (IS) with antilymphocyte globulin (ALG). The overall actuarial survival was 63% after BMT and 61% after IS therapy at 6 years. All patients fulfilled the criteria of SAA; however, most patients with a neutrophil count of <0.2 × 109/l also had infections and haemorrhages. Therefore a further subclassification was defined by pretreatment peripheral blood neutrophil count: very severe aplastic anaemia (vSAA) (<0.2 × 109/l neutrophils) and moderately severe aplastic anaemia (mSAA) (0.2‐0.5 × 109/l neutrophils).A Cox regression analysis showed that the only significant pre‐treatment variables were a low neutrophil count (P= 0.001) and increasing age (P= 0.05). Thus it seemed reasonabie to analyse survival data after combined stratification for neutrophils (vSAA versus mSAA) and age (cut off at 20 years). BMT was superior to IS in patients with vSAA under 20 years of age (64%v.38%;P= 0.01). IS was superior to BMT in patients with mSAA aged 20 or more (82%v.62%;P= 0.002). The two treatments gave comparable results in young patients with mSAA (BMT = 58%, IS = 62%;P= 0.1), and in older patients with vSAA (BMT = 44%. IS = 43%;P= 0.06).Overall 75/218 and 87/291 patients, given BMT or IS respectively, died. The major cause of failure in BMT patients was graft rejection (n= 22) or problems associated with graft‐versus‐host disease. For ALG patients the major problem was persistence of the aplasia with haemorrhage (n= 32) or infections (n= 46).This study indicates that over 60% of patients with SAA can be successfully treated with either BMT or IS. Overall survival does not differ in the two groups, though significant differences emerge after stratification for severity of the aplasia and age.
Blast cell vacuoles in childhood lymphoblastic leukaemia
Authors: Lilleyman J. S. , Hann I. M. , Stevens R. F. , Richards S. M. , Eden O. B. 
Published: March 2008

As part of a central review of cell morphology in childhood lymphoblastic leukaemia (ALL), marrow smears from entrants to the Medical Research Council trial UKALL VIII, other than those from children with B‐ALL, were studied prospectively for the presence or absence of blast cell vacuoles and for any clinical or biological relevance this feature might have.Adequate slides were available from 733 patients (88% of the trial entrants) after five with B ALL were excluded. Vacuolated blast cells (> 10%) were present in 204 (28%). The presence of vacuoles was associated with PAS positivity (%2= 27.8; P<0.0001), a diagnostic white cell count (WBC) <50 × 109/l (%2= 13.1; P<0.0001), and the immunophenotype of ‘common’ALL (CD10 positive) (%2= 9.1; P<0.01). There was no clear association with French‐American‐British (FAB) type L1 or L2. The 204 patients with vacuoles had a significantly superior disease free survival compared to the remainder (2P= 0.01), a difference which remained significant when the analysis was stratified by FAB type (2P= 0.01), age (2P= 0.02) or sex (2P= 0.02), but which was lost when stratified by WBC (2P= 0.06).These findings provide further evidence that, outside the context of B‐ALL, vacuoles are indicative of a relatively benign disease which responds well to therapy. The French‐American‐British (FAB) classification should be modified to take this into account.
The value of immunocytochemical staining of lymph node aspirates in diagnostic cytology
Authors: Oertel J. , Oertel B. , Kastner M. , Lobeck H. , Huhn D. 
Published: March 2008

This study investigates the applicability of immunocytochemical techniques towards improving the cytological diagnosis of lymph node disorders. Cytocentrifuge preparations of fine needle aspirates were examined using an indirect immunoperoxidase method and the alkaline phosphatase–antialkaline phosphatase method.36 reactive lymph nodes were evaluated. 23 patients showed T cell predominance as assessed by the presence of CD3+ cells. 13 patients showed an excess of CD24+ cells over CD3+ cells. In all patients the B cell population showed no light chain class restriction.48 aspirates from patients with B‐non‐Hodgkin's lymphoma of low malignancy were investigated. The majority of the cells in each of these cases expressed CD24 and 47 cases were monoclonal with respect to their light chain determinants. In 37/48 cases the antibody OKT9 reacted with <15% of cells.18 aspirates were obtained from patients with B‐non‐Hodgkin's lymphoma of high malignancy. 17 patients showed light chain class restriction and a high percentage of CD24+ (>80%) and OKT9+ (>40%) cells.8 malignancies were considered to be of T‐cell origin. A low percentage (<15%) of Ig+CD24+ cells with a high number of CD3+ and/or CD4+ cells suggests a T‐cell lymphoma. The majority of neoplastic cells in peripheral T‐cell lymphomas (excluding mycosis fungoides) expressed OKT9 and CD3 expression was found to be variable.In 15 cases of Hodgkin's disease, there were 11 correct cytologic diagnoses. A significant number of BerH2 (CD30)+ cells having the morphology of Sternberg‐Reed cells supports this diagnosis. 25 aspirates were obtained from patients with metastatic malignant tumours. Marker studies in eight cases helped in distinguishing metastatic malignant tumours from malignant lymphomas.Our findings indicate that the immunocytochemical technique is applicable to cytological material and that the simplicity of the procedure merits application to routine diagnostic cytology.
Erythroid progenitor cell kinetics in chronic haemodialysis patients responding to treatment with recombinant human erythropoietin
Authors: Reid C. D. L. , Fidler J. , Oliver D. O. , Cotes P. Mary , Pippard M. J. , Winearls C. G. 
Published: March 2008

The response of bone marrow and peripheral blood erythroid progenitors to human recombinant erythropoietin (rHuEPO) was studied in nine haemodialysed renal failure patients receiving this hormone for the correction of their anaemia. The haematocrit rose in all patients in response to thrice weekly injections of escalating rHuEPO doses (12–192IU/kg). Both the numbers of CUF‐e and BFU‐e and their proliferative state in the bone marrow as well as BFU‐e numbers in the peripheral blood were estimated before treatment and again after correction of the anaemia, at 16 h following an intravenous dose of rHuEPO.Following treatment bone marrow BFU‐e numbers fell to a mean of 24.5% (P <0.01) of the pre‐treatment values although there was no significant change in CFU‐e or circulating BFU‐e numbers. The mitotic rate (percentage S‐phase cells) estimated by tritiated thymidine suicide rose from 45.2% to 68.4% (P <0.05) in the case of CFU‐e and from 16.4% to 45.1% (P <0.05) for BFU‐e following treatment with rHuEPO thus indicating in‐vivo sensitivity of both the primitive as well as the mature erythroid progenitors to the hormone. The fall in BFU‐e numbers in the bone marrow after several months of treatment may be due to a loss of cells from this progenitor pool by maturation that is uncompensated by replacement from the pluripotential stem cell compartment.
Effects of cis‐diamminedichloroplatinum (II) upon haemopoietic progenitors and the haemopoietic microenvironment in mice
Authors: Nikkels Peter G. J. , De Jong Johannes P. , Ploemacher Rob E. 
Published: March 2008

Summary. We studied the short‐ and long‐term effects of a fractionated injection of cis‐diamminedichloroplatinum (II) (CDDP) upon the haemopoietic stroma and the haemopoietic precursor cell compartment of young and adult mice. The integrity of the stromal microenvironment was investigated using three different assays including quantification of (a) the fibroblastoid progenitor cell compartment, (b) the regenerative capacity after subcutaneous implantation of spleen and femur, and (c) the growth of normal bone marrow progenitors in lethally irradiated CDDP‐treated mice.CDDP treatment induced a slight anaemia which lasted for the observation period of 1 year, and could not be restored by infusion of normal bone marrow cells. The population size of haemopoietic progenitors was severely decreased immediately after CDDP treatment and the CFU‐S recovery in the bone marrow was slow and temporary. Stromal function was significantly decreased and normalization occurred within approximately 40 d, depending on the stromal parameter measured. Subsequently, the regenerative capacity of the stroma showed a second decrease which was still detected at 1 year. This pattern of stromal damage has not been reported for any other cystostatic agent. Since the other two assays did not detect a second decrement in stromal integrity it is implied that the three stromal assays used detect different stromal functions. We conclude that CDDP treatment of both young and adult mice results in severe short‐term damage and a late occurring secondary regenerative defect of the haemopoietic organ stroma.
Von Willebrand disease investigated by two novel RFLPs
Authors: Bernardi F. , Guerra S. , Patracchini P. , Volinia S. , Buzzoni D. , Ballerini G. , Casonato A. , Marchetti G. 
Published: March 2008

SummaryTwo partial cDNAs for von Willebrand factor (vWF) were used to investigate gene lesions and restriction fragment length polymorphisms (RFLPs) in vW disease (vWd) and normal controls. No gene alteration was detected but two TaqI RFLPs, likely to be intronic and originating from point mutations, were found in the 3’ part of vWF gene. The two TaqI RFLPs, identified by the same probe, are informative in approximately 50% of the subjects. Used in combination with two other known RFLPs, they define several haplotypes similarly distributed in vWd and normals. Linkage disequilibrium between loci identified by the RFLPs is present.In a family study the RFLP patterns demonstrate homozygosity for the affected vWF gene in a severe (type III) patient and identify several heterozygous subjects. The RFLPs analysis has been related to the haemostatic values and multimer distribution. In two of the four unrelated patients with severe vWd examined the RFLPs study indicates double heterozygosity for the affected vWF genes.
Two different theta (θ) globin gene deletions observed among black newborn babies
Authors: Fei Y. J. , Fujita S. , Huisman T. H. J. 
Published: March 2008

SummaryTwo different θ1 globin gene deletions have been detected in the Black population of the southeastern U.S.A. Restriction mapping of the α globin gene cluster indicated that one deletion removes ∼ 1.8 kb of DNA including most of the θ1 globin gene, while the second removes ∼ 6 kb of DNA including the entire θ1 globin gene. These defects do not cause haematological alterations in the newborn or in the adult (established for the smaller deletion only), nor were changes observed in polypeptide chain patterns at birth.
A comparative study of combination chemotherapy versus marrow transplant in first remission in adult acute lymphoblastic leukaemia
Authors: Proctor S. J. , Hamilton P. J. , Taylor Penny , Carey P. , Hargrave S. , Evans R. G. B. , Summerfield G. , Finney R. , Saunders P. , Goff D. , Reid M. M. 
Published: March 2008

Summary. The results of conventional chemotherapy in adult acute lymphoblastic leukaemia (ALL) have not improved substantially in recent years. The present study is based on a flexible policy of marrow transplantation (allograft and autograft without marrow purging) in first remission compared with a group treated with standard maintenance therapy after a common induction sequence. The actuarial disease free survival (DFS) and actuarial overall survival (OS) at 3 years for autologous marrow grafted patients was 30% and 65% respectively. The allogeneic transplant group had DFS of 30% and OS at 3 years of 38% compared with DFS (12%) and OS (12%) for patients on 6‐mercaptopurine and methotrexate maintenance. The actuarial disease free survival calculations include patients on protocol not entering remission, therefore, giving the worst possible result.We conclude that high dose chemo/radiotherapy with autologous marrow rescue in first remission followed by no maintenance provides better results in terms of overall survival and quality of life than standard ALL maintenance in adult patients. Results for allogeneic transplant in ALL are less good in terms of duration and quality of survival and the majority of deaths are related to causes other than leukaemic relapse.