bluuebunny/crossref_metadata_2025_split huggingface dataset:
Karyotypic evolution in a granulocytic sarcoma developing in a myeloproliferative disorder with a novel (3;4) translocation
Authors: MYINT H. , CHACKO J. , MOULD S. , ROSS F. , OSCIER D. G. 
Published: March 2008

Summary. A 59‐year‐old man presented with a granulocytic sarcoma arising in the manubrium, and splenomegaly. The blood count showed 1‐2 × 109/1 eosinophils and a marrow aspirate was hypercellular with eosinophilia. Cytogenetic analysis of the marrow revealed a novel t(3;4) (pl3;ql2) and analysis of cells aspirated from the granulocytic sarcoma showed the same abnormality and an additional trisomy 8.Intensive chemotherapy and local radiotherapy led to resolution of the chest mass but persistence of the chromosome translocation in the marrow.
Evaluation of cerebrospinal fluid EBV‐DNA and IL‐10 as markers for in vivo diagnosis of AIDS‐related primary central nervous system lymphoma
Authors: LUCA ANDREA DE , ANTINORI ANDREA , CINGOLANI ANTONELLA , LAROCCA LUIGI MARIA , LINZALONE ANGELA , AMMASSARI ADRIANA , SCERRATI MASSIMO , ROSELLI ROMEO , TAMBURRINI ENRICA , ORTONA LUIGI 
Published: March 2008

Summary. Acquired immunodeficiency syndrome (AIDS)‐related primary central nervous system lymphoma (PCNSL) is almost always associated with the Epstein‐Barr virus (EBV), and EBV‐DNA in cerebrospinal fluid (CSF) has been indicated as a useful tumour marker for this HIV‐related neoplasm. AIDS lymphomas also show an enhanced production of IL‐10 which is generally associated with the presence of EBV in lymphoma cells.We performed a prospective study in 19 HIV seropositive patients with brain mass lesions, and in 21 other AIDS patients with or without other neurological disorders, to assess the in vivo diagnostic value of EBV‐DNA and of IL‐10 levels in the CSF for primary lymphoma of the central nervous system (CNS). EBV‐DNA was detected by a nested polymerase chain reaction (PCR) in the CSF from seven of eight patients with PCNSL, diagnosed by brain biopsy (875% sensitivity) and in none of the 11 controls with brain mass lesions (100% specificity) and of the other 21 AIDS patients with or without neurological disorders. The only patient with PCNSL without detectable EBV‐DNA in the CSF was also negative for EBV‐DNA in the lymphoma tissue, whereas the samples of the other seven brain lymphomas were all positive for EBV‐DNA by nested PCR. Therefore 100% of patients with an EBV‐positive primary CNS lymphoma had detectable EBV‐DNA in the CSF. No patient from the control group without PCNSL with EBV‐negative CSF developed a lymphoma after a mean follow‐up of 157 ± 173 d. IL‐10 levels in the CSF from the patients with PCNSL were not significantly different from those in the other groups of patients with AIDS. Due to uniformly high levels in the CSF from AIDS patients, IL‐10 is not a useful diagnostic marker for AIDS‐related brain lymphoma.The detection of EBV‐DNA from the CSF by nested PCR is an extremely sensitive and specific diagnostic tool for AIDS‐related PCNSL and should be further evaluated as a possible alternative in patients from whom brain biopsy is not advisable.
Patient accrual and quality of participation in a multicentre study on myeloma: a comparison between major and minor participating centres
Authors: Hjorth Martin , Holmberg Erik , Rodjer Stig , Taube Adam , Westin Jan 
Published: March 2008

Summary .The participation of minor centres in randomized trials has been questioned because of inferior quality of participation. We have studied this issue in a multicentre trial on myeloma, in which 5 74 patients were included from 99 participating centres in Sweden, Norway and Denmark from 1 June 1990 until 4 November 1992.Two hundred and eight patients were entered from university hospitals (n= 13), 172 from major county hospitals (n= 25), denned by a population base of ± 100 000 inhabitants, and 194 from minor county hospitals (n= 61) with a population base of < 100 000 inhabitants. The accrual rate was similar for the three hospital categories, averaging 54% of all reported cases, corresponding to 38% of the expected number of newly diagnosed cases. The adherence to the study protocol from an administrative point of view was judged by the completeness of follow‐up forms and the delay in the notification of deaths, and from a clinical point of view by the dose intensity for the principal drugs of the study, melphalan and interferon. For all studied measures of quality, the values were similar for the three hospital categories.We conclude that with due informative and educational support, minor centres can make a considerable contribution to the patient material of a large randomized trial without impairing the quality of the study.
Adhesion molecule expression on CD34+ progenitor cells from normal and aplastic anaemia bone marrow
Authors: Karakantza Marina , Cavenagh James D. , Gordon‐Smith Edward C. , Gibson Frances M. 
Published: March 2008

Summary. Aplastic anaemia (AA) is a disease of bone marrow failure. Evidence has been produced for both a stem cell and a stromal cell defect in this disease. The contribution of deficient or defective cell adhesion molecules (CAMs) has not been determined. CAMs have been shown to be important in stem cell‐stromal cell interactions and maintenance of haemopoiesis.In this study the expression of CAMs (LFA‐1, LFA‐3, ICAM‐1, VLA‐4, CD44, sLex and L‐selectin) on CD34+ progenitor cells from 10 normal donors and eight patients with AA was investigated using double immunofluorescence. There was no significant difference in the percentage of CD34+ cells that were CAM+ between normal and AA bone marrow, suggesting that abnormal CAM expression on AA progenitor cells is not responsible for nor contributes to the pathogenesis of the disease. However, these findings do not exclude abnormal CAM function on progenitor cells, or abnormal expression or function of CAM ligands or counter‐receptors on AA stromal cells.
Isodicentric (X)(q13) in haematological malignancies: presentation of five new cases, application of fluorescence in situ hybridization (FISH) and review of the literature
Authors: Dierlamm Judith , Michaux Lucienne , Criel Arnold , Wlodarska Iwona , Zeller Wolfgang , Louwagie Andries , Michaux Jean‐Louis , Mecucci Cristina , Van Den Berghe Herman 
Published: March 2008

Summary. Idic(X)(q13) represents a rare but recurrent chromosomal abnormality in haematological malignancies. We present five new cases characterized by this particular aberration and review the literature on this subject.The patients were elderly females with a diagnosis of refractory anaemia (1/5), refractory anaemia with ringed sideroblasts (2/5), chronic myelomonocytic leukaemia (1/ 5), and Philadelphia chromosome‐negative chronic myeloid leukaemia (1/5). Three out of the five patients demonstrated an increased proportion of bone marrow ringed sideroblasts. After a follow‐up period of 30‐57 months all patients but one are alive.Idic(X)(q13) always occurred as the sole chromosomal abnormality, either in one or in two copies. We confirmed the dicentric nature of the aberration by fluorescence in situ hybridization (FISH) on metaphases as well as interphase nuclei using an X‐chromosome‐specific alpha‐satellite probe, and performed chromosome painting to visualize possible additional chromosomal changes involving the X chromosomes.Our findings and the data of 17 previously published cases indicate that idic(X)(q13): (1) may play a significant pathogenetic role in haematological malignancies affecting exclusively females and deriving predominantly from early progenitor cells; (2) is frequently associated with a pathological iron accumulation; (3) indicates a variable prognosis.
Chimaeric cultures of human marrow stroma and murine leukaemia cells: evidence for abnormalities in the haemopoietic microenvironment in myeloid malignancies and other infiltrating marrow disorders
Authors: DUHRSEN ULRICH , KNIELING GABRIELE , BEECKEN WOLF , NEUMANN STEFAN , HOSSFELD DIETER KURT 
Published: March 2008

Summary. PGM‐1 is a transplantable C3H/HeJ leukaemia which is not viable in unstimulated in vitro culture, differentiates into mature granulocytes and macrophages in response to soluble cytokines, and undergoes self‐renewing cell divisions in coculture with selected human bone marrow stromal cell lines. When PGM‐1 cells were cultured on pre‐established adherent layers from primary human marrow samples, their fate depended on the source of the human marrow. Adherent layers from healthy marrow donors or patients with reactive marrow alterations had no or very little capacity to maintain PGM‐1 cells in an immature colony‐forming state. However, in coculture with adherent layers from patients with myeloid leukaemia or, to a lesser extent, lymphoblastic leukaemia or marrow‐infiltrating lymphoma the colony‐forming potential was retained. There was no correlation between the remission status of the patient and the PGM‐1 activity of the adherent layer. Consistent morphological differences between active and inactive stromal layers were not observed.The PGM‐1 coculture system enables the detection of a hitherto undescribed regulatory abnormality in bone marrow malignancies. Whether the PGM‐1 supporting activity is mediated through differences in the production of a cytokine with close homology to complement factor Bb which has recently been shown to induce self‐renewal in immature PGM‐1 cells, requires further investigation.
IL‐3 is produced by normal stroma in leng‐term bone marrow cultures
Authors: GIBSON F. M. , SCOPES J. , DALY S. , RIZZO S. , BALL S. E. , GORDON‐SMITH E. C. 
Published: March 2008

Summary. Interleukin‐3 (IL‐3) has been shown to have significant effects on haemopoiesis in vitro, but early investigations of normal human long‐term bone marrow cultures (LTBMC) have failed to demonstrate LL‐3 production by stromal cells, either by Northern blotting for mRNA, or assaying for bioactivity in culture supernatants. One recent report, using reverse transcription‐polymerase chain reaction (RT‐PCR), demonstrated IL‐3 expression in only one of eight cultures.We have developed a sensitive bioassay for the detection of IL‐3 production from normal stroma in LTBMC. LTBMC were grown until confluent, irradiated, and stroma harvested by trypsinization to yield single‐cell suspensions. These cells were then cocultured with target bone marrow mononuclear cells (BMMC), or CD344 cells in clonogenic assays, either in the presence or absence of anti‐IL‐3 neutralizing antibodies. We have demonstrated IL‐3 production in 32β4 cases. In addition, by separating stroma from target cells using cell culture inserts, we have shown that direct stroma:stem cell contact is not necessary for colony growth, suggesting that LL‐3 diffuses into the supernatant. However, when supernatants from LTBMC were assayed by enzyme‐linked immunoassay (ELISA), no IL‐3 was detected. This suggests that IL‐3 is probably produced at low levels and has a short‐range interaction.Stroma production of IL‐3 was confirmed by the detection by RT‐PCR of IL‐3 mRNA in 3β cases. The simultaneous detection of CD2 mRNA demonstrated that T cells are part of the bone marrow stroma. It is therefore possible and probably likely that these cells are the source of IL‐3.
Persistence of RARa‐PML fusion mRNA detected by reverse transcriptase polymerase chain reaction in patients in long‐term remission of acute promyelocytic leukaemia
Authors: TOBAL KHALID , SAUNDERS MELANIE J. , GREY MARK R. , LIU YIN JOHN A. 
Published: March 2008

Summary. Acute promyelocytic leukaemia (APL) is characterized by t(15;17), which results in the formation of two chimaeric genes, PML‐RARa and RARa‐PML. PML‐RARa transcripts have been detected in all cases of APL whilst those of RARQ‐PML have been detected in only about 67% of cases. We have used reverse transcriptase polymerase chain reaction (RT‐PCR) to detect both fusion transcripts serially in 18 patients in remission of APL after chemotherapy and bone marrow transplantation. All patients were negative for PML‐RARa, whereas in six patients (remission 3‐9 years) RARa‐PML was consistently detected. Only one patient at remission showed the 5’breakpoint RARa‐PML, with the rest showing the 3’breakpoint 144 bp RARa‐PML. The level of sensitivity for detecting RARa‐PML was some 10‐fold higher than that for PML‐RARa.Serial negative tests for PML‐RARa have been correlated with durable remissions, suggesting possible eradication of residual leukaemia in APL. Our results, however, show persistence of t(15;17) cells expressing RARa‐PML fusion mRNA in patients in long‐term remission of APL. They indicate that patients considered clinically‘cured’of APL still have molecular evidence of minimal residual disease and also provide further insight into the biology of acute myeloid leukaemia.
Predicting the severity of haemolytic disease of the newborn: an assessment of the clinical usefulness of the chemiluminescence test
Authors: NOBLE A. L. , POOLE G. D. , ANDERSON N. , LUCAS G. F. , HADLEY A. G. 
Published: March 2008

Summary. The ability of the chemiluminescence test (CLT) to predict the severity of haemolytic disease of the newborn (HDN) was determined in 80 alloimmunized pregnant women who delivered antigen‐positive babies. In 54 cases of alloimmunization to D, results from the CLT showed better correlation with fetal outcome than anti‐D concentration measured by AutoAnalyzer (r = 0.70 and 0.36 respectively). Results from the CLT permitted a threshold level of antibody activity (30%) below which 15/20 babies were unaffected or had mild disease, and only one required transfusion therapy in utero. CUT results above 30% were associated with moderate or severe disease in all cases.Results from the AutoAnalyzer proved a less reliable predictor of disease severity; three women with anti‐D levels >20iu/ml delivered unaffected babies, and two women with anti‐D levels <10iu/ml delivered babies who had required transfusion in utero. The clinical usefulness of the CLT derives from the possibility of avoiding invasive monitoring procedures in women with high levels of anti‐D which is relatively non‐functional.
Unexpected high incidence of intracranial subdural haematoma during intensive chemotherapy for acute myeloid leukaemia with a monoblastic component
Authors: Jourdan E. , Dombret H. , Glaisner S. , Micléa J. M. , Castaigne S. , Degos L. 
Published: March 2008

We report a high incidence of subacute, chronic and sometimes occult intracranial subdural haematoma (SDH) occurring during intensive chemotherapy for acute myeloid leukaemia (AML) with a monoblastic component. Between March 1990 and January 1993, 86 AML patients from our institution were randomized in the multicentric French AML 90 trial. Eight patients (9%) presented a grade > 2 haemorrhagic event, which was intracranial SDH in five of them. All these five SDH patients had hyperleucocytic AML4 or AML5 and had experienced at least one lumbar puncture (LP) before SDH diagnosis (with intrathecal chemotherapy in four cases). SDH diagnosis was assessed on a brain computed tomography scan which was performed 1‐9 d after initial SDH symptoms (mainly mild headaches considered a result of prior LP). All these five patients recovered from this severe event after a specified therapy.SDH does not appear to be an uncommon complication of AML4 and AML 5 therapy. Its incidence might be underreported because of poor symptomatology. Lumbar punctures, known to cause exceptional SDH in nonleukaemic patients, might trigger these haemorrhagic events, eventually in combination with other predisposing factors such a haemostasis disorders or leukaemic CNS infiltration.
Interferon response in chronic myeloid leukaemia correlates with ABL/BCR expression: a preliminary study
Authors: Yin Jian‐Lin , Williams Bronwyn G. , Arthur Chris K. , MA David D. F. 
Published: March 2008

α‐Interferon (IFN) has been used to induce cytogenetic remission in chronic myeloid leukaemia (CML), but there are few indicators to predict IFN response. The role of the chimaeric BCR/ABL gene in the malignant process is undisputed. There are, however, conflicting views as to whether the breakpoint site within the BCR gene, and the type of mRNA produced determine disease prognosis and progression. The function and clinical significance of the newly discovered ABL/BCR mRNA has not been investigated for a correlation with CML prognosis or response to therapy. We have used a two‐step reverse transcriptase polymerase chain reaction (RT‐PCR) to detect the transcripts of the chimaeric genes BCR/ABL, ABL/BCR, as well as the normal ABL and BCR genes in 24 CML patients treated with IFN.Because of the variable expression of the four transcripts at presentation, a correlation between gene expression, prognosis and clinical progression was examined. No correlation between prognosis and gene expression was seen. Also, no correlation was found between expression of BCR, ABL or BCR/ABL mRNA and response to treatment with IFN. However. 7/10 ABL/BCR mRNA positive patients achieved a major cytogenetic response to IFN; but of the 13 ABL/BCR mRNA negative patients, only two achieved a major cytogenetic response (P=0‐013). Further studies are required to confirm these findings.
Problems of resistance development in arthropod pests of agricultural crops in Russia
Authors: Sukhoruchenko G. I. , Dolzhenko V. I. 
Published: March 2008

This paper presents the results of long‐term monitoring of insecticide resistance in populations of agricultural pests in Russia. Over the last 45 years, resistance developments were recorded for 36 arthropod pest species in 11 agricultural crops and pastures in relation to nearly all commonly used plant protection products. Development of group, cross and multiple resistance has been revealed in populations of many economically important pests. Toxicological and phenotypical (for Colorado potato beetle) methods have been devised to monitor the development of pesticide resistance. Based on experience over the last century, systems aimed at preventing the development of pest resistance to insecticides and acaricides are elaborated. These systems are based on resistance monitoring and using plant protection measures which minimize the toxic pressure on agroecosystems.
Body mass index in adults with intellectual disability: distribution, associations and service implications: a population‐based prevalence study
Authors: Bhaumik S. , Watson J. M. , Thorp C. F. , Tyrer F. , McGrother C. W. 
Published: March 2008

AbstractBackground  Previous studies of weight problems in adults with intellectual disability (ID) have generally been small or selective and given conflicting results. The objectives of our large‐scale study were to identify inequalities in weight problems between adults with ID and the general adult population, and to investigate factors associated with obesity and underweight within the ID population.Methods  We undertook a population‐based prevalence study of 1119 adults with ID aged 20 and over on the Leicestershire Learning Disability Register who participated in a programme of universal health checks and home interviews with their carers. We performed a cross‐sectional analysis of the register data and compared the observed and expected prevalences of body mass index categories in the ID and general populations using indirect standardisation for age. We used logistic regression to evaluate the association of a range of probable demographic, physical, mental and skills attributes with obesity and underweight.Results  In those aged 25 and over, the standardised morbidity ratio (SMR) for obesity was 0.80 (95% CI 0.64–1.00) in men and 1.48 (95% CI 1.23–1.77) in women. The SMR for underweight was 8.44 (95% CI 6.52–10.82) in men and 2.35 (95% CI 1.72–3.19) in women. Among those aged 20 and over, crude prevalences were 20.7% for obesity, 28.0% for overweight, 32.7% for normal weight and 18.6% for underweight. Obesity was associated with living independently/with family, ability to feed/drink unaided, being female, hypertension, Down syndrome and the absence of cerebral palsy. Underweight was associated with younger age, absence of Down syndrome and not taking medication.Conclusion  Obesity in women and underweight in both men and women was more common in adults with ID than in the general population after controlling for differences in the age distributions between the two populations. The associated factors suggest opportunities for targeting high‐risk groups within the ID population for lifestyle and behaviour modification.
Teaching picture‐to‐object relations in picture‐based requesting by children with autism: a comparison between error prevention and error correction teaching procedures
Authors: Carr D. , Felce J. 
Published: March 2008

AbstractBackground  Children who have a combination of language and developmental disabilities with autism often experience major difficulties in learning relations between objects and their graphic representations. Therefore, they would benefit from teaching procedures that minimize their difficulties in acquiring these relations. This study compared two teaching procedures, an error prevention procedure and an error correction procedure, for teaching relations between objects and pictures.Method  Participants were two groups of children with autism, aged between 3 and 7 years. In the context of picture‐to‐object requesting, one group was taught using an error correction method and the other group with an error prevention method. The measures for each child were accuracy of correspondences between taught picture and object pairs and accuracy of delayed correspondences in learning outcome tests with all combinations of object and picture pairs presented to them throughout the study.Results  The group receiving the error prevention‐based teaching made significantly fewer errors during the teaching phases and in their learning outcome test for correspondences between all combinations of pictures and objects.Conclusions  The error prevention teaching procedure would seem to provide a more efficient and ecologically valid method than the error correction procedure for teaching relations between objects and their graphic‐based referents. Improvements in the methodology were suggested for providing a stronger basis for comparison between error correction and error prevention teaching methods.
Attention deficits in tuberous sclerosis complex (TSC): rethinking the pathways to the endstate
Authors: De Vries P. J. , Watson P. 
Published: March 2008

AbstractBackground Tuberous sclerosis complex (TSC) is a genetic disorder associated with a range of neurocognitive manifestations, including neuropsychological attention deficits most notably in dual tasking/divided attention. These dual‐task deficits have so far been interpreted as evidence of a vulnerable ‘cognitive module’ in TSC. Here, we suggest that this interpretation represents an ‘adult neuropsychological’ perspective, and argue that a developmental approach would be more appropriate to examine attention deficits in TSC.Method We examined the pathway to ‘endstate’ dual‐task deficits in twenty 6–16 year olds with TSC utilising the Test of Everyday Attention for Children (TEA‐Ch). We predicted that the pattern of attentional deficits in TSC would support a ‘conditional’ model where the establishment of a later‐maturing skill was dependent on the functional maturation of an earlier expected skill.Results Attentional profiles showed statistical support for a conditional model. Only one child showed a deterministic pattern while one showed a hybrid pattern, attributed to the admixture of a surgically acquired lesion and a neurodevelopmental disorder.Conclusion This preliminary study suggests that the developmental cascade in TSC may be arrested at various stages of neuropsychological development, thus leading to different developmental trajectories towards similar ‘endstate’ profiles.