bluuebunny/crossref_metadata_2025_split huggingface dataset:
Relevance of class, molecular weight and isoelectric point in predicting human light chain amyloidogenicity
Authors: Bellotti Vittorio , Merlini Giampaolo , Bucciarelli Eleonora , Perfetti Vittorio , Quaglini Silvana , Ascari Edoardo 
Published: March 2008

SummaryThe ability to predict the amyloidogenicity of certain light chains may facilitate an earlier diagnosis of AL amyloidosis and, possibly, lead to more effective treatment. Using current methods, available in clinical chemistry laboratories, we assessed the class, the relative molecular mass (Mr) and the isoelectric point of urinary monoclonal light chains from 35 patients with AL amyloidosis (A +) and 51 without amyloidosis (A –). The light chain class (LCC) was λ in 77% and 45% of A + and A – patients, respectively. Light chain fragments (LCF) with low Mr (12–18 x 103 were detected in the urine of 30/35 A + patients and in 15/51 A – ones. The mean (SD) isoelectric point (pI) of A + light chains was 4.8 (1.1) while in A – patients it was 6.2 (1.6). Univariate analysis showed significant differences between the two groups for the three parameters. Discriminant analysis gave a function which allowed a correct allocation of 81% of the cases between the two groups.
Antithrombin III and procoagulant activity: sex differences and effects of the menopause
Authors: Meade T. W. , Dyer Sandra , Howarth D. J. , Imeson J. D. , Stirling Yvonne 
Published: March 2008

SummaryAmong participants in the Northwick Park Heart Study, antithrombin III activity was lower in pre‐menopausal women than in men of the same age. In the women, however, the menopause was associated with a significant increase in antithrombin III, mean levels in these older women then exceeding levels in men of the same age. The occurrence of the menopause was also accompanied by large increases in factor VII coagulant activity, VIIc, and in plasma fibrinogen, these increases being greater in those experiencing a natural menopause than in those whose menopause was artificial. Sex differences in antithrombin III may form part of the explanation for the observed differences between men and women in their experience of ischaemic heart disease (IHD) and also for the contrasting effects of oral contraceptives and of hormone replacement therapy on the risk of thromboembolic disease.
Receptor–ligand complexes are cleared to the open canalicular system of surface‐activated platelets
Authors: Leistikow Elizabeth A. , Barnhart Marion I. , Escolar Gines , White James G. 
Published: March 2008

SummaryHuman platelets were incubated with gold particles coupled to fibrinogen to label the glycoprotein IIb–IIIa (GPIIb–IIIa) receptor after initial activation of the cells by contact with formvar‐coated grid and glass surfaces. Fibrinogen–gold (Fgn–Au) markers were absent on discoid platelets, but diffusely spread over the surface and extended pseudopods of early dendritic cells. Conversion to spread platelets resulted in movement of ligand‐receptor complexes away from the cell margin toward cell centres. However, Fgn–Au gold did not concentrate in the central region. Rather, the Fgn–Au, GPIIb–IIIa complexes in the middle of spread platelets appeared to move toward a belt‐like, intermediate zone, as did the ligand receptor complexes from the cell margin and pseudopods. The ultimate destination of the mobile receptor‐ligand complexes, however, appeared to be channels of the surface‐connected open canalicular system (OCS). Fgn–Au was concentrated in OCS channels of most dendritic and a small proportion of spread platelets. The decreased frequency of Fgn–Au filled channels in more transformed platelets may have been due to collapse or evagination of the OCS. Examination of platelets exposed to Fgn–Au after spreading on glass and then prepared for thin sections confirmed that the OCS was the final destination for mobile ligand receptor complexes on surface‐activated platelets. Findings of this study are consistent with previous work showing clearance of mobile receptor–ligand complexes to the OCS of platelets activated in suspension.
Genetic thrombocytopenia with autosomal dominant transmission: a review of 54 cases
Authors: Najean Yves , Lecompte Thomas 
Published: March 2008

SummaryOn the basis of a retrospective study of 3600 platelet kinetic studies, we have isolated 54 cases with chronic thrombocytopenia, a normal autologous and homologous platelet lifespan, and increased mean platelet volume without Döhle bodies, the absence of any functional platelet abnormalities, and a normal megakaryocyte count. These cases were either discovered during the first few years of life (i.e. constitutional) and/or were proven to be familial (autosomal dominant transmission). Previous treatments (corti‐costeroids, immunoglobulins, androgens, immunosuppres‐sor agents, splenectomy) were not effective in any of these cases or in their relatives.A new syndrome can therefore be proposed which can easily be suspected on the basis of platelet kinetic studies performed in cases of early onset, increased platelet volume, failure of corticosteroids or evidence of a familial blood disorder. It can be proved when the autologous platelet life span is demonstrated to be normal in spite of a chronic thrombocytopenia and a normal megakaryocytic count. The recognition of this syndrome will avoid neonatal complications (cephal‐haematomas), surgical complications, and the use of expensive and possibly harmful ineffective treatments, both in the propositus and in other abnormal family members.The syndrome is certainly frequent (54 cases are presented here), but the diagnosis is often missed or delayed due to the low risk of haemorrhage. However, it is associated with a certain risk of leukaemia (four cases in three families).
Detection of immunoglobulin gene rearrangement in B lymphoid malignancies by polymerase chain reaction gene amplification
Authors: Deane Miriam , Norton John D. 
Published: March 2008

SummaryImmunoglobulin heavy chain gene rearrangement serves as a marker of cell lineage and clonality in B lymphoproliferative disorders. We have used polymerase chain reaction (PCR) gene amplification to detect immunoglobulin gene rearrangements involving VH251. a heavy chain variable region preferentially utilized in B lymphoproliferative disorders. Using synthetic amplimers derived from VH251 and the heavy chain joining region, under conditions of high stringency, a homogeneous VH251‐specific fragment of approximately 350 bp could be amplified from leukaemic DNA. Of 53 cases of B lineage acute lymphoblastic leukaemia screened for VH2 51 rearrangement by PCR, 10 were positive. A background level of VH251 rearrangement could also be amplified from normal peripheral blood and bone marrow DNA. but a VH251 rearranged leukaemic clone representing 0‐01% of bone marrow mononuclear cells could be readily detected. The application of PCR to detect immunoglobulin gene rearrangement involving VH251 and potentially other preferentially utilized V regions provides a sensitive method both for tracking malignant B cells and for the study of normal B cell developmental pathways through which B lineage malignancies arise.
Elevated white blood cell synthesis of leukotriene C4 in chronic myelogenous leukaemia but not in polycythaemia vera
Authors: Stenke Leif , Samuelsson Jan , Palmblad Jan , Dabrowski Lech , Reizenstein Peter , Lindgren JanÅke 
Published: March 2008

SummaryLeukotriene (LT) formation was studied in ionophore A23187‐stimulated white blood cell (WBC) preparations from patients with chronic myelogenous leukaemia (CML; n=14), polycythaemia vera (PV; n=10) and two control groups consisting of patients with non‐malignant inflammatory disease (n= 4) and normal healthy donors (n= 25). The synthesized products were identified and quantitated using high‐performance liquid chromatography combined with computerized UV‐spectroscopy.White blood cell preparations from the CML patients produced more LTC4 (40.2±7.9 pmol/106 WBC, mean±SEM) than WBC from the healthy donors (9.0±1.8), P<0.0005. In contrast, the formation of LTB4 was normal and there was no increase in the total leukotriene synthesis (the sum of LTC4, LTB4, 20‐OH‐LTB4 and the Δ6‐trans‐isomers of LTB4). The ratio between leukotrienes C4 and B4 was strongly elevated in the CML group; 1.67 ± 0.25 v. 0.37±0.07 in the controls, P<0.0005. No significant correlation was observed between the levels of LTC4 and the number of known LTC4 producing cells (such as monocytes, eosinophils and basophils) in the CML WBC preparations. In contrast, a correlation was found between the sum of neutrophilic granulocytes and metamyelocytes in these suspensions and the amount of LTB4 formed; r=0.600. P<0.05 A number of other laboratory or clinical variables of the CML patients (including total white blood cell and platelet counts, differential counts, previous cytotoxic treatment, time from diagnosis, time from last treatment, post study survival and age) did not significantly correlate with the formation of leukotrienes. No abnormality in the production of LTB4 or LTC4 was observed in granulocyte and WBC preparations from the patients with polycythaemia vera and non‐malignant inflammatory disease, respectively. The results indicate a selectively increased LTC4 producing capacity in CML.
Use of prostaglandin E1 for prevention of liver veno‐occlusive disease in leukaemic patients treated by allogeneic bone marrow transplantation
Authors: Gluckman E. , Jolivet I. , Scrobohaci M. L. , Devergie A. , Traineau R. , Bourdeau‐Esperou H. , Lehn P. , Faure† P. , Drouet L. 
Published: March 2008

SummaryProstaglandin E1 (PGE1) was used to prevent veno‐occlusive disease (VOD) of the liver after allogeneic bone marrow transplantation (BMT) for leukaemia. It was given in continuous i.v. infusion from day – 8 to day 30 after BMT at a dose of 0.3 μg/kg/h. The patients were studied according to the risk factors for VOD: diagnosis, intensification of the conditioning and previous liver abnormalities. The diagnosis of VOD was made on at least two of the following factors: weight gain, hepatomegaly, jaundice, ascitis, pain of the right upper quadrant, increased platelet consumption. 109 patients were studied, 50 were treated by PGE1 and 59 did not receive it. The actuarial incidence of VOD was 12.2% in the PGE1 group and 25.5% in the non PGE1 group (P=0.05). In acute leukaemia, the incidence was 39.1% in the non‐treated group and 12.8% in the PGE1 treated group (P=0.02). Patients with previous hepatitis had an incidence of 62.5% in the non treated group and 15.5% in the treated group (P=0.05). A positive cytomegalovirus (CMV) serology seemed to increase the risk of VOD: the incidence of VOD was 31.4% in non‐treated patients and 22% in PGE1 treated patients. The multivariate analysis of the risk factors for VOD shows that unfavourable factors were: recipient positive CMV serology (P=0.06), hepatic disease prior to transplant (P=0.02) and the absence of PGE1 treatment (P=0.02). This study suggests that prophylactic PGE1 treatment may decrease the incidence of VOD in patients treated for leukaemia by allogeneic bone marrow transplantation.
The effect of molecular weight on heparin binding to platelets
Authors: Horne McDonald K. , Chad Elizabeth S. 
Published: March 2008

SummaryLow molecular weight heparin is reported to be less reactive with platelets than larger heparins. We probed the molecular basis for this pattern of reactivity by characterizing the saturable platelet binding of [3H]heparin in plasma using heparins of different molecular weights (Mr˜ 3000, ˜ 5000, ˜ 10 000, ˜ 15000). Binding affinity increased with increasing molecular weight, as expressed by decreasing apparent dissociation constants (Kdapp˜ 1.3 μM for Mr˜ 3000, to Kdapp˜ 0.31 μM for Mr˜ 15000). After adjusting for the effect of antithrombin III in the plasma, true dissociation constants (Kd) could be calculated and these showed the same trend with molecular weight (Kd˜ 1.1 μM for Mr ˜ 3000 to Kd˜ 0.096 μM for Mr˜ 15 000). Platelet binding capacity for the different heparin fractions also increased with molecular weight, although this correlation appeared to lessen with the largest species. Heparin antithrombin III affinity was shown not to affect heparin binding to platelets. We propose a model in which heparin binding to platelets is mediated by charge interaction. Larger molecules with more charge bind with greater affinity and to sites with a broader range of electronegativity than do smaller, less
Platelet aggregating IgG antibody to platelet surface glycoproteins associated with thrombosis and thrombocytopenia
Authors: Pfueller Sharron L. , David Rosemary , Firkin Barry G. , Bilston Robyn A. , Cortizo W. Fernando , Raines Geoffrey 
Published: March 2008

SummaryPreviously described platelet‐aggregating antibodies associated with thrombosis and thrombocytopenia required heparin for their in vivo and in vitro expression. We have observed a patient with thrombosis who became thrombocytopenic during heparin treatment, but who suffered further thrombotic events and continued thrombocytopenia for 3 months after heparin withdrawal. The patient's plasma contained a potent platelet aggregating factor reactive with both his own and normal platelets in the absence of heparin. It also caused [14C]serotonin secretion from labelled platelets from normal donors and patients with either Glanzmann's thrombasthenia or Bernard‐Soulier syndrome. This factor was an IgG and was neutralized by antibody specific for IgG lambda light chains. While the patient was thrombocytopenic an IgG paraprotein with lambda light chains was detected by isoelectrofocussing. After corticosteroid treatment it disappeared and the patient recovered. The active, but not the recovery serum contained IgG which immunoprecipitated a glycoprotein with characteristics of Glycoprotein IV from platelets labelled with Na[3H]BH4/periodate. Thus platelet‐aggregating IgG antibodies with direct specificity for platelet surface glycoproteins may be associated with thrombosis/thrombocytopenia.
Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients
Authors: Visani G. , Finelli C. , Castelli U. , Petti M. C. , Ricci P. , Vianelli N. , Gianni L. , Zuffa E. , Aloe Spiriti M. A. , Latagliata R. , Pileri S. , Magrini U. , Gugliotta L. , Morra E. , Bernasconi C. , Mandelli F. , Tura S. 
Published: March 2008

SummaryThe prognostic value of 12 clinical and haematological parameters, recorded at diagnosis, in myelofibrosis with myeloid metaplasia (MMM) was retrospectively analysed in a consecutive series of 133 patients followed for a minimum of 60 months. Multivariate analysis showed that the following features were associated with a significantly shorter survival: (1) short period of time (<13 months) between first symptoms and diagnosis; (2) anaemia (haemoglobin <10 g/dl); (3) leucocyte count >12 × 109/l; (4) peripheral blood granulocyte precursors >10%. Age, splenectomy and percentage of peripheral blood metamyelocytes were found significantly to affect survival only from univariate analysis, whereas sex, size of spleen, thrombocytopenia and thrombocytosis were of no prognostic significance. These data suggest that a more intensive chemotherapy might be useful for younger patients with bad prognostic factors at diagnosis.
Acute monocytic leukaemia in adults: treatment and prognosis in 99 cases
Authors: Fenaux Pierre , Vanhaesbroucke Colette , Estienne Marie Hélégne , Preud'homme Claude , Pagniez Dominique , Facon Thierry , Millot Frédéric , Bauters Francis 
Published: March 2008

SummaryAcute monocytic leukaemia (AMoL) was diagnosed in 99 adults, aged 18–85 years (median 56) over a period of 10 years. Sixty‐five patients had extramedullary leukaemia, 13 had clinical signs of leucostasis, and 19 had disseminated intravascular coagulation. Four patients died before receiving any treatment, 12 received supportive care only and seven received low dose AraC, but only one of them responded. Seventy‐six patients received intensive chemotherapy, 72 of them with an anthracycline‐AraC based regimen, with or without an epipodophyllotoxin.Fifteen patients died within 7 d of diagnosis, due to leucostasis in nine cases. Predictive factors for early death were advanced age, leucostasis, fever, leucocytes above 100 x 109/l, and renal failure. Fifty (66%) of the patients treated intensively reached complete remission (CR). Advanced age, fever and complex cytogenetic abnormalities were significantly associated with a lower CR rate. Median actuarial disease‐free survival was 20.5 months, and was not significantly influenced by any pretreatment parameter. Five patients relapsed in the central nervous system (CNS), in spite of systematic CNS prophylaxis. No differences in CR rates were seen with the three anthracycline–AraC based regimens used in our patients. Significant differences in disease‐free survival were seen between them, however, suggesting that early consolidation chemotherapy and, more hypothetically, epipodophyllotoxin agents could prolong remission duration in AMoL.
Increased red blood cell aggregation in retinal vein occlusion
Authors: Chabanel Anne , Glacet‐Bernard Agnégs , Lelong Françloise , Taccoen Alain , Coscas Gabriel , Samama Meyer M. 
Published: March 2008

SummaryReversible aggregation of red blood cells (RBC) plays an important role in determining the flow properties of blood, and is the cause of the increase in blood viscosity at low shear rates. Retinal venous circulation is characterized by the combination of a low flow state and a high vascular resistance which might severely limit its capacity to adjust to high blood viscosity. These characteristics make the venous circulation in the retina particularly dependent on haemorheological factors. To test the possibility that high RBC aggregation could predispose to the onset and development of retinal vein occlusion (RVO), RBC aggregation and disaggregation (SEFAM erythroaggregameter, France) were measured in 64 patients with RVO. Results were compared to those of a group of 64 controls, similar in age, sex, smoking habit and associated pathologies. Increased RBC aggregation was observed in 52% of the patients, and the mean values showed a highly significant elevation of RBC aggregation parameters in RVO patients (+ 14%) when compared with controls (P<0.001). Subgroups were compared to study the influence of site (central versus branch), form (ischaemic versus non‐ischaemic), duration and severity of the occlusion on the aggregation parameters. No significant differences were found between these various subgroups. An increase in RBC aggregability and in the shear resistance of RBC aggregates, by predisposing to circulatory stasis, is likely to contribute to the onset of RVO.
Hb Icaria–Hb H disease: identification of the Hb Icaria mutation through analysis of amplified DNA
Authors: Efremov G. D. , Josifovska O. , Nikolov N. , Codrington J. F. , Oner C. , Gonzalez‐Redondo J. M. , Huisman T. H. J. 
Published: March 2008

SummaryHb Icaria–Hb H disease was observed in a Yugoslavian teenager who exhibited moderate anaemia with severe microcytosis and hypochromia and 16% Hb H. Four of his relatives were Hb Icaria heterozygotes; their haematological data were comparable to those with a deletional type of α‐thalassaemia‐2. The patient also had an additional α‐thalassaemia‐1 deletion, an ∼20.5 kb deletion, common among Mediterranean populations. The Hb Icaria mutation, i.e. the TAAÁA mutation at codon 142, was identified by hybridization of amplified DNA with specific probes. The mutation is located on the α2‐globin gene; the one remaining α1‐globin gene is apparently able to compensate sufficiently for the loss of the three α‐globin genes to maintain a haemoglobin level of 8–9 g/dl.
Murine monoclonal antibodies against a unique determinant of erythrocytes, related to Rh and U antigens: expression on normal and malignant erythrocyte precursors and Rhnull red cells
Authors: Kr. vondem Borne Albert E. G. , Bos Marjolein J. E. , Lomas Christine , Tippett Patricia , Bloy Christian , Hermand Patricia , Cartron Jean Pierre , Admiraal Lucia G. , Van de Graaf Jettie , Overbeeke Marijke A. M. 
Published: March 2008

SummaryThree murine monoclonal antibodies (Mabs) MB‐2D10, LA‐18.18 and LA‐23.40 were prepared. They reacted with red cells of all common and most rare blood‐group phenotypes, with the exception of those of the RhnullU negative and RhmodU negative phenotypes. So far, only a single example of an alloantibody (Duclos or anti‐Rh38) of a similar specificity has been found.Serological studies indicated that the Mabs were probably not directed against an antigenic determinant of Rh polypeptides, the LWab glycoprotein or glycophorin B, all structures absent from or aberrantly expressed on Rhnull red cells. The antigen was found to be erythrocyte‐specific, and was also present on pro‐erythroblasts, erythroblasts and malignant erythroblastoid cells but not on erythroid progenitors in the bone marrow.The Mabs were found to block each other in an immune rosette method and are thus probably directed against the same epitope or against neighbouring epitopes on the same structure.In immunochemical studies, MB‐2D10 precipitated the 30–32 kDa Rh polypeptides from red cell membranes and a protein or proteins which formed diffuse and overlapping bands in SDS–polyacrylamide gel electrophoresis, with Mrs of 40–200 kDa (probably the Rh‐related glycoproteins). Under certain experimental conditions glycophorin B appeared to be coprecipitated.The 2D10 structure, detected by the Mabs, seems to be part of a complex of proteins and/or glycoproteins, which includes Rh polypeptides, the LWab glycoprotein and glycoproteins recognized by various Mabs with Rh‐related specificities. In the red cell membrane, the complex may be associated with glycophorin B.
Acute ‘bilineal‐biphenotypic’ leukaemia
Authors: Akashi Koichi , Shibuya Tsunefumi , Harada Mine , Morioka Eiji , Oshima Koichi , Kimura Nobuhiro , Takeshita Morishige , Kurokawa Mamoru , Kikuchi Masahiro , Niho Yoshiyuki 
Published: March 2008

SummaryWe describe a unique case of hybrid leukaemia with bilineal and biphenotypic features. The coexistence of lymphoblasts and monoblasts was determined morphologically and cytochemically. Immunofluorescence and immunohistochemical analysis revealed that each blast population had both T lymphoid (CD2, cytoplasmic CD3) and myeloid (CD11, CD13, CD15) markers. Southern blot analysis of DNA extracted from the lymph node biopsy demonstrated the presence of monoclonal rearrangement of the TcR‐Cβ gene. Cytogenetic analysis of the bone marrow cells showed a karyotype of 48, XY, 7q + in all of the metaphases examined. These observations are suggestive of a monoclonal origin for these two distinct blast populations.
Light chain restriction analysis of bone marrow plasma cells in patients with MGUS or ‘solitary’ plasmacytoma: diagnostic value and correlation with clinical course
Authors: Bezwoda W. R. , Gordon V. , Bagg A. , Mendelow B. 
Published: March 2008

SummaryTwenty‐eight patients with a diagnosis of ‘solitary’ plasmacytoma or with gammopathy but with nondiagnostic morphologic examination of the bone marrow were investigated using a short‐term bone marrow culture technique which enriched for the plasma cell fraction. The percentage of monotypic plasma cells in these plasma cell enriched cultures was correlated with the subsequent clinical course. The majority of patients with plasmacytoma and a significant number of those with gammopathy but with otherwise non‐diagnostic investigations were found to have a monoclonal plasma cell component of >20%. There was a significant correlation between the percentage of monoclonal plasma cells as detected by bone marrow culture and subsequent progression to disseminated myeloma. These results indicate that early bone marrow involvement can be detected by means of plasma cell culture prior to morphologic identification of marrow plasmacytosis and that short‐term plasma cell culture distinguishes patients with early, low bulk myeloma from those with monoclonal gammopathy of uncertain significance (MGUS).