bluuebunny/crossref_metadata_2025_split huggingface dataset:
Two human myeloma cell lines, amylase‐producing KMS‐12‐PE and amylase‐non‐producing KMS‐12‐BM, were established from a patient, having the same chromosome marker, t(11;14)(q13;q32)
Authors: Ohtsuki Takemi , Yawata Yoshihito , Wada Hideho , Sugihara Takashi , Mori Masaharu , Namba Masayoshi 
Published: March 2008

SummaryTwo human myeloma cell lines, KMS‐12‐PE and KMS‐12‐BM, were established from a 64‐year‐old woman with a non‐producing type of multiple myeloma. The KMS‐12‐PE line originated from the pleural effusion and the KMS‐12‐BM from the bone marrow. These two lines showed the same chromosome marker, t(11;14)(q13;q32). However, their phenotypes of surface markers differed from each other. KMS‐12‐BM cells were positive to CD20, CD38 and PCA‐1, showing the plasmacytoid (immature plasma cell) stage of B‐cell differentiation, while KMS‐12‐PE cells were positive to CD38 and PCA‐1, but not to CD20, indicating the terminal differentiated stage of B‐cells. As seen in the pleural effusion of the patient, KMS‐12‐PE cells ectopically produced a salivary type of amylase, but KMS‐12‐BM cells did not. Interestingly, the chromosome abnormality of del(1)(p22→pter) near the region of Ip21, where the amylase gene was assigned, was noticed in as many as 76% of KMS‐12‐PE cells.
Identification of H‐ and L‐ferritin subunit binding sites on human T and B lymphoid cells
Authors: Anderson G. J. , Faulk W. Page , Arosio P. , Moss D. , Powell L. W. , Halliday J. W. 
Published: March 2008

SummaryMonoclonal antibodies to the H and L subunits of ferritin were used in indirect immunofluorescent studies to investigate the subunit specificity of ferritin binding to transformed T and B lymphoid cell lines. Pre‐incubation of cells with recombinant H ferritin followed by antibodies to the H subunit produced a labelling pattern in which all cells showed strong fluorescent stippling. Corresponding experiments with liver ferritin and an antibody to the L subunit revealed that most cells were lightly stippled, but 3–5% of the cells were intensely labelled. These two labelling patterns were distinct as assessed by cross‐blocking experiments with recombinant H and liver ferritins. These results imply that different binding sites exist for each subunit.
An immunological cross‐reactant of erythropoietin in serum which may invalidate EPO radioimmunoassay
Authors: Cotes P. Mary , Tam R. C. , Reed P. , Hellebostad M. 
Published: March 2008

SummaryAtypically high radioimmunoassay estimates of erythropoietin in serum samples from two out of a series of 130 normal Norwegian children have been found to be invalid. Invalidity was due to non‐parallelism of radioimmunoassay (RIA) response dilution lines given by these two sera when compared with the second International Reference Preparation (IRP). In contrast, immunoreactive erythropoietin in other sera usually shows parallelism with the IRP. Fractionation by gel filtration of the sera showing non‐parallelism in the RIA showed them to contain an erythro‐poietin cross‐reactant which was eluted before both an 125I recombinant human erythropoietin marker and normal erythropoietin.In family studies, non‐parallelism of RIA response dilution lines and the large molecular size erythropoietin cross‐reactant was found in serum from one sibling but not in five other family members of one of the two index cases.Radioimmunoassays based on two different antisera differed in recognition of the abnormal cross‐reactant of erythropoietin.
Enhancement of monoclonal antibody dependent cell mediated cytotoxicity by IL2 and GM‐CSF
Authors: Bianchi Alessandra C. , Heslop Helen E. , Veys Paul , Macey Marion , Holland Mark , Prentice H. Grant , Brenner Malcolm K. 
Published: March 2008

SummaryRodent monoclonal antibodies (MAb) directed against cells of the immune system may be used in vivo for applications including conditioning prior to marrow transplantation and treatment of lymphoid malignancies. Although some MAb may lyse targets by complement fixation, MAb dependent cell mediated cytotoxicity (MAb‐DCC) appears to be an important additional effector mechanism. We have investigated the cellular basis of the phenomenon and the response of the effector cells to recombinant cytokines in an attempt to maximize the efficacy of MAb‐DCC and thereby increase the therapeutic potency of MAbs. Blood mononuclear cells (PBM) coated with CAMPATH 1G (pan lymphocyte reactive rat IgG2b) MAb were used as targets (T) and autologous lymphocytes or granulocytes were used as effector (E) cells. We studied function in normal donors as well as patients 1 week before and 3‐6 weeks after bone marrow transplantation (BMT). In the absence of CAMPATH 1G, specific 51Cr release from autologous PBM was < 1% in all groups, even after pre‐incubation of the effector cells with granulocyte‐macrophage. colony stimulating factor (GM‐CSF) or interleukin‐2 (IL2). In the presence of 5 μg/ml of MAb, and at an E: T ratio of 50:1, lymphocytes from normal donors induced a low level (6.5%) of 51Cr release from auto PBM rising to 9% after pre‐incubation of effector cells with IL2 (P= 0.02). Granulocytes had greater activity inducing 10%51CR release (range 2‐23%) which rose to 21.6% with GM‐CSF (range 12‐48%) (P0.001). Pre‐BMT, killing by lymphocytes and granulocytes was not significantly different from normal, and responded to IL2 and GM‐CSF. In contrast, granulocyte killing after BMT was significantly impaired (51Cr release 3%) and showed no rise with GM‐CSF. Killing by lymphocytes, however, remained normal, as did their IL2 response. Loss of granulocyte mediated MAb‐DCC coincided with significant post‐BMT impairment of oxidative metabolism: expression of Fc receptors II and III, however, was normal. Optimum therapeutic effect of MAb‐DCC is likely to be achieved when MAb are given together with appropriate cytokihes. the choice of which will depend upon the clinical circumstances.
Polyclonal rearrangements of the T‐cell receptor β‐chain in fatal angioimmunoblastic lymphadenopathy
Authors: Knecht Hans , Odermatt Bernhard F. , Hayoz Daniel , Kühn Lukas , Bachmann Fedor 
Published: March 2008

SummaryGenomic rearrangement of germline T‐cell antigen receptor (TcR) and immunoglobulin (Ig) genes was studied by Southern blot analysis in seven patients with angioimmunoblastic lymphadenopathy (AILD). In three cases clinically suspected of transformation into malignant lymphoma, hybridization with the TcRβ probe showed markedly dimished intensity in the 11.5 kb germline band after Eco RI digestion and normal germline configuration after Hind III and Bam HI digestion, indicating polyclonal T cell rearrangements. A clonal rearrangement of the TcRβ gene was detected in only one case at initial biopsy. No monoclonal rearrangement of Ig genes was observed. These data show that in some cases of AILD disease progression is indicated by polyclonal TcR rearrangements and not by outgrowth of a malignant clone, supporting the concept of AILD as an immunoregulatory disorder.
Decreased stability and structural heterogeneity of the residual platelet glycoprotein IIb/IIIa complex in a variant of Glanzmann's thrombasthenia
Authors: Modderman P. W. , van Mourik J. A. , Berkel W. van , Cordell J. L. , Morel M. C. , Kaplan C. , Ouwehand W. H. , Huisman J. G. , von dem Borne A. E. G. Kr. 
Published: March 2008

SummaryA patient is described with a disturbance of platelet function comparable to that in Glanzmann's thrombasthenia. Platelet aggregation and binding of fibrinogen to the patient's platelets were defective and thrombin‐induced clot retraction was absent. The platelet fibrinogen content was only moderately reduced. As measured by monoclonal antibody binding in the presence of divalent cations, the platelets contained about 15% of the normal amount of GPIIb and GPIIIa and only 6% of the normal amount of intact GPIIb/IIIa complex. The residual GPIIb/IIIa complex exhibited a decreased stability as shown by the lack of binding of a complex‐dependent anti‐GPIIb/IIIa antibody to platelets incubated with ethylene diamine tetraacetic acid (EDTA) at 22°C. Crossed immunoelectrophoresis (CIE) in the presence of divalent cations showed partial dissociation of GPIIb/IIIa as well as the presence of two forms of the residual intact GPIIb/IIIa complex. In addition, both CIE in the presence of the EDTA and two‐dimensional sodium dodecyl sulphate (SDS) gel electrophoresis showed the presence of two forms of GPIIb. This form of thrombasthenia is characterized by a defective platelet function, a marked reduction of GPIIb and GPIIIa, decreased stability of the residual GPIIb/IIIa complex and structural heterogeneity of GPIIb.
Blood from healthy animals and humans contains nondiscocytic erythrocytes
Authors: Simpson L. O. 
Published: March 2008

SummaryBlood samples (three drops) from healthy animals and humans were fixed in a 2.5% solution of glutaraldehyde in 0.1 m cacodylate buffer at pH 7.4 within seconds of being drawn. Dehydrated and gold‐coated preparations were photographed at × 600 in a scanning electron microscope, then printed at × 1200. Erythrocytes were classified according to their shape and surface features into six classes, namely, normal red cells, flat cells, cells with surface changes, early cup forms, late cup forms, cells with altered margins. The size of each shape‐determined class was expressed as a percentage of the total number of cells counted. Data relating to 23 healthy females and to a mouse, rat, rabbit and horse are reported. Although the presence of nondiscocytic erythrocytes in peripheral human blood has been described, the presence of similar cells in animal blood has not been reported previously.
In vitro studies of ways to overcome resistance to VAMP‐high dose melphalan in the treatment of multiple myeloma
Authors: Millar B. C. , Bell J. B. G. , Maitland J. A. , Zuiable A. , Gore M. E. , Selby P. J. , McElwain T. J. 
Published: March 2008

Summary Myeloma colonies (MY‐CFUc) from 7/24 patients undergoing treatment with VAMP (vincristine, adriamycin and methyl prednisolone) and high dose melphalan (HDM) were melphalan‐resistant. It was not possible to conclude that VAMP induced melphalan resistance in MY‐CFUc, but that resistance is endogenous in some myeloma cell populations. In 12/13 of the same patients of whom four had MY‐CFUc which were melphalan resistant, the sensitivity of MY‐CFUc and GM‐CFUc to busulphan was similar. Thus resistance of MY‐CFUc to melphalan did not confer resistance to busulphan.MY‐CFUc from 1/7 of a second group of patients were adriamycin‐resistant. This resistance was removed when the cells were treated with a combination of verapamil (3 μg/ml) and adriamycin. Verapamil also enhanced the toxicity of adriamycin to MY‐CFUc, from two patients where there was no evidence for adriamycin resistance. In these three patients the sensitivity of both MY‐CFUc and GM‐CFUc was similar after treatment with verapamil.Verapamil did not affect the uptake or efflux of 3H‐daunorubicin in sensitive and resistant RPMI‐8226 cells (myeloma) and peripheral blood mononuclear cells from a normal donor; neither did it affect the binding of 3H‐daunorubicin to nucleic acid.It is concluded that verapamil may be a useful adjuvant to VAMP chemotherapy and that busulphan may provide an alternative to melphalan in patients whose myeloma cells are melphalan resistant.
Late onset bone marrow failure associated with proximal fusion of radius and ulna: a new syndrome
Authors: Dokal I. , Ganly P. , Riebero I. , Marsh J. , Steed A. , Kendra J. , Drysdale C. , Hows J. 
Published: March 2008

Summary Members of two unrelated families, one from England and the other from Portugal, presented with aplastic anaemia. Both families had clinical and laboratory features in common. The proposita presented with progressive bone marrow failure in adult life involving all three haemopoietic cell lines. In both families individuals with bone marrow failure also had proximal fusion of the radius and ulna bilaterally which segregates as an autosomal dominant trait. No cytogenetic defect was identified in myeloid or lymphoid cells, sampled from the marrow and peripheral blood.Proximal fusion of the radius and ulna is uncommon and its co‐existance with late onset bone marrow failure may indicate an association. Growth failure, non‐skeletal congenital malformations and cytogenetic abnormalities were absent suggesting that this syndrome is distinct from Fanconi's anaemia. The onset in adult life of generalized bone marrow failure involving all three haemopoietic cell lines associated with proximal fusion of the radius and ulna also makes this syndrome distinct from the thrombocytopenia with absent radii (TAR) syndrome.
Complete recovery of marrow function after treatment with anti‐lymphocyte globulin is associated with high, whereas early failure and development of paroxysmal nocturnal haemoglobinuria are associated with low endogenous G‐CSA‐release
Authors: Nissen Catherine , Moser Yolanda , Carbonare Verena Dalle , Gratwohl Alois , Speck B. 
Published: March 2008

Summary 24 patients who were treated with antilymphocyte globulin (ALG) for severe aplastic anaemia (SAA) were tested for endogenous release of granulocyte colony stimulating activity (G‐CSA) prior to, and at various intervals after treatment. CSA‐production in vitro was induced with auto‐ logous serum as a source of ‘releaser’ activity, avoiding the use of plant mitogens. Before treatment, G‐CSA‐release was highly variable. Though mean values were higher in the 17 patients who subsequently responded to ALG treatment than in the six non‐responders, this difference was not statistically significant. In the 17 responders, G‐CSA‐release strongly increased prior to improvement of peripheral blood counts. In one responder patient tested before, and at regular intervals after ALG, CSA‐release was high before, abnormally low at 7 d and increased again to high values before the onset of bone marrow reconstitution. In six patients who did not respond to ALG‐treatment, G‐CSA release decreased after treatment, and a second course of ALG was ineffective when given during this low CSA‐phase. Five of the 24 patients developed paroxysmal nocturnal haemoglobinuria (PNH) at 9 months to 3 years after ALG‐treatment. In all, the onset of PNH was associated with very low G‐CSA‐release, whether it had been high or low before treatment.We conclude that low‐CSA‐release after ALG treatment is a poor prognostic sign. It either indicates progression of marrow failure or heralds PNH. Such patients may be candidates for early bone marrow transplantation or treatment with G‐CSF or GM‐CSF.
Chemosensitivity testing of fresh leukaemia cells using the MTT colorimetric assay
Authors: Twentyman Peter R. , Fox Norma E. , Rees John K. H. 
Published: March 2008

SummaryWe have determined the feasibility of using the MTT colorimetric assay for measuring the sensitivity of fresh leukaemia cells to cytotoxic agents. A linear relationship was seen between the number of leukaemic cells plated and the resulting absorbance in the MTT assay at day 4. For 2 × 105 cells/well from CLL peripheral lymphoctes, the mean absorbance (measured at 540 nm) was 0.41. Similar curves were obtained with the assay carried out between days 3 and 7. ID50 values for CLL lymphocytes exposed to adriamycin (ADM) were in the range of 0.02‐0.29 μg/ml, a similar range to that given by the DiSC dye exclusion assay. Sensitization of leukaemia cells to ADM by verpamil or cyclosporin A could be demonstrated in some patients but not in others. The lymphocytes from one patient with prolymphocytic leukaemia were abnormally resistant to ionizing radiation. These cells also showed resistance to melphalan and adriamycin but not to vincristine. The MTT assay appears to offer an attractive option for in vitro chemosensitivity testing in leukaemia, determination of cross‐resistance profiles and studies of resistance modifiers.
A translocation involving a specific breakpoint (q35) on chromosome 5 is characteristic of anaplastic large cell lymphoma (‘Ki‐1 lymphoma')
Authors: Rimokh Ruth , Magaud J.‐P. , Berger Francoise , Samarut J. , Coiffier B. , Germain D. , Mason D. Y. 
Published: March 2008

SummaryTwo cases of non‐Hodgkin's lymphoma are reported in which a chromosomal translocation was observed involving the same site (q35) on the long arm of chromosome 5. The other breakpoint involved in the translocation was chromosome 2 (p23) in one case and chromosome 3 (q12) in the other. Both cases were large cell lymphomas expressing CD30 antigen (‘Ki‐1 lymphoma'). One was clearly of T lymphoid origin, the other probably B cell derived. One other case of a Ki‐1 lymphoma with 2;5 translocation (involving the same breakpoint on chromosome 5) has been reported previously, and it is suggested that this cytogenetic abnormality may be specifically associated with Ki‐1 lymphoma. The literature contains a further eight cases of lymphoid neoplasms with a translocation involving a breakpoint at q35 on chromosome 5. They have all been described as cases of‘malignant histiocytosis’, but the present findings make it likely that these cases were in reality also examples of Ki‐1 lymphoma. The breakpoint at the q35 region on chromosome 5 is close to the position of the fms proto‐oncogene, suggesting that an abnormality affecting this gene might possibly play a causal role in ‘Ki‐1 lymphoma’. However, DNA restriction fragment analysis of the present cases showed no evidence that the breakpoint on chromosome 5 involves the fms gene or its immediate vicinity.
Mortality and causes of death in Dutch haemophiliacs, 1973–86
Authors: Rosendaal F. R. , Varekamp I. , Smit C. , Bröcker‐Vriends A. H. J. T. , Van Dijck H. , Vandenbroucke J. P. , Hermans J. , Suurmeijer T. P. B. M. , Briët E. 
Published: March 2008

SummaryMortality figures were calculated for a group of 717 Dutch haemophiliacs over the period 1973‐86. Follow‐up was on average 10.9 years; no patients were lost to follow‐up. The data were compared to the general male population by actuarial methods and patient‐year analysis. Forty‐three patients died, while 20 deaths were expected in a hypothetical group of non‐haemophiliacs of the same age distribution. Hence, overall mortality was 2.1 times higher than in the general population. This leads to a calculated life expectancy of 66 years, as compared to 74 years in the general male population. Mortality did not differ much by severity of haemophilia. A possibly beneficial effect of prophylaxis on longevity was observed. Haemorrhage occurred in half of all deaths and among these traumatic bleeding was the most prevalent. The number of deaths due to ischaemic heart disease was significantly lower (80% reduction) than expected and therefore the authors conclude that haemophilia offers protection against ischaemic heart disease. Cancer mortality was significantly higher (2.5 times) than expected.
Anti‐idiotypes to factor VIII antibodies and their possible role in the pathogenesis and treatment of factor VIII inhibitors
Authors: Moffat E. H. , Furlong R. A. , Dannatt A. H. G. , Bloom A. L. , Peake I. R. 
Published: March 2008

SummaryStudies on the production and characterization of anti‐idiotype antibodies (AId) to monoclonal factor VIII antibodies (McFVIIIAb) are reported. Two AIds were produced and one of these exhibited cross‐reactivity with two other McFVIIIAb but showed no reactivity with haemopholic and non‐haemophilic FVIIIAb. This AId was also active against McFVIIIAb which bound immunologically active forms of factor VIII but it did not neutralize McFVIIIAb directed against procoagulant factor VIII. The in vitro effect of therapeutic pooled human IgG concentrates upon haemophilic and non‐haemophilic FVIIIAb was also assessed. Approximately 25% of non‐haemophilic FVIIIAb were inhibited following incubation with human IgG whereas the latter had no effect upon haemophilic FVIIIAb activity. Studies on haemophilic sibling pairs of FVIIIAb and non‐FVIIIAb producing individuals indicated FVIIIAb neutralizing activity in the non‐FVIIIAb producing sibling's IgG fraction which may be of anti‐idiotypic origin. These findings lend further support to suggestions that anti‐idiotypes have a regulatory role in FVIIIAb production and are of potential therapeutic value.
Severe Hb S‐β°‐thalassaemia with a T → C substitution in the donor splice site of the first intron of the β‐globin gene
Authors: Gonzalez‐Redondo J. M. , Stoming T. A. , Kutlar F. , Kutlar A. , McKie V. C. , McKie K. M. , Huisman T. H. J. 
Published: March 2008

SummaryThrough direct sequencing and dot‐blot analyses with synthetic oligonucleotide probes of amplified DNA, a new nucleotide substitution was discovered in a Black teenager with severe Hb‐S‐β°‐thalassaemia. The substitution involved a T → C replacement at the second position of the donor splice site of the first intervening sequence of the β‐globin gene. The clinical and haematological observations made in Black subjects with Hb S‐β°‐thalassaemia, with different types of thalassaemia, suggest severe disease resembling sickle cell anaemia. Only an occasional patient had a milder clinical course, perhaps because of a greatly increased production of fetal haemoglobin.
In vivo regeneration of red cell 2, 3‐diphosphoglycerate following transfusion of DPG‐depleted AS‐1, AS‐3 and CPDA‐1 red cells
Authors: Heaton Andrew , Keegan Thais , Holme Stein 
Published: March 2008

SummaryRegeneration of 2, 3‐diphosphoglycerate (DPG) was determined following transfusion of DPG‐depleted group O red cells into group A recipients. Blood from five donors was stored in the adenine‐containing solutions CPDA‐1, AS‐1 or AS‐3 for 35 d at 4°C. Post‐transfusion red cell DPG and ATP were measured in separated group O red cells over a 7 d period. The studies confirmed rapid in vivo DPG regeneration with ≥ 50% of the maximum level being achieved within 7 h. An average of 95% of the recipients’ pre‐transfusion DPG level was achieved by 72 h and by 7 d mean (±SEM) DPG levels relative to recipient's pre‐transfusion DPG averaged 84% (± 13%), 92% (± 17%) and 84% (± 21%) for CPDA‐1, AS‐1 and AS‐3 red cells, respectively. Results were comparable to those previously reported for blood stored in ACD for 15‐20 d (Valeri & Hirsch, 1969; Beutler & Wood, 1969). The immediate regeneration rate, V, closely approximated first order regeneration kinetics with AS‐3 red cells exhibiting double the rate of CPDA‐1 red cells (P<0·001). AS‐1 red cells exhibited an intermediate rate of regeneration which was not significantly different compared to either CPDA‐1 or AS‐3 (P>0·05). V exhibited a significant (P<0·05) positive correlation with ATP levels 5‐7 h post‐infusion. ATP regeneration of the infused cells was rapid with a mean increase of 1·2 μmol/g Hb above post‐storage levels being achieved 1 h following transfusion.
Identification of normal B‐cell counterparts of neoplastic cells which secrete cold agglutinins of anti‐I and anti‐i specificity
Authors: Stevenson F. K. , J. North G. J. Smith , Hamblin T. J. , Glennie M. J. 
Published: March 2008

Summary. A monoclonal antibody, which recognizes a cross‐reacting idiotypic determinant present on human cold‐reactive autoantibodies with anti‐I or anti‐i binding activity, has been found to specifically inhibit the cold agglutination of red cells. This suggests that an epitope close to the binding site of such autoantibodies is being recognized.The antibody has been used to identify tumour cells in the blood of three patients with cold haemagglutinin disease, and to analyse the heterogeneous nature of the neoplastic B‐cell clone present in the bone marrow of one of the patients. Using S‐phase analysis, it was found that cell proliferation was occurring in the bone marrow but not in the blood, and that the major proliferating population was that of lymphoplasmacytoid cells containing large vesicular inclusions of idiotypic IgM.It has also been possible to locate normal B‐cells which are recognized by the anti‐idiotypic antibody. Such cells have been found throughout the normal adult lymphoid tissue where they account for 2.9–10.8% of the B lymphocyte population. They are also present in fetal spleen at 15 weeks gestation, indicating that immunoglobulins bearing this sequence form part of the immature B‐cell repertoire.