bluuebunny/crossref_metadata_2025_split huggingface dataset:
Idiopathic osteonecrosis, hypofibrinolysis, high plasminogen activator inhibitor, high lipoprotein(a), and therapy with stanozolol
Authors: Glueck Charles J. , Freiberg Richard , Glueck Helen I. , Tracy Trent , Stroop Davis , Wang Ying 
Published: February 2007

AbstractIn five patients with idiopathic osteonecrosis (ON) of the hip, four having hypofibrinolysis mediated by high plasminogen activator inhibitor (PAI‐Fx), and one with high Lp(a), our specific aim was to determine whether therapy (Rx) with the anabolic‐androgenic steroid, Stanozolol(6 mg/day), would normalize PAI‐Fx and Lp(a) and thus potentially ameliorate ON. Prior to Rx, none of the four patients with high PAI‐Fx could normally elevate tissue plasminogen activator (tPA‐Fx) after 10 min venous occlusion at 100 mm Hg. After 12‐18 weeks on Rx, PAI‐Fx and stimulated tPA‐Fx normalized in all four patients. Prior to Rx, mean (SD) stimulated tPA‐Fx was low, 0.4 ± 0.3 IU/ml (lower limit of normal 2.28 IU/ml). On Rx, stimulated tPA‐Fx normalized, rising to 2.83 ± 1.9 IU/ml, P = 0.004. Prior to Rx, mean (SD) basal PAI‐Fx was high, 99 ± 68 (upper limit of normal 26.9 U/ml), and fell on Rx to 22.5 ± 22, P = 0.004. In two of the five patients normalization of hypofibrinolysis or high Lp(a) was accompanied by major symptomatic improvement. Prior to Rx, and 2 years after onset of unilateral hip pain, one of the four patients with high PAI‐Fx and low stimulated tPA‐Fx could walk only one block painfully. After 8 weeks on Stanozolol Rx, and continuing through 54 weeks on Rx, he walked 2 miles per day without pain, despite radiographic progression of ON. In three of the four patients with high PAI and with osteonecrosis present 0.3, 2, and 6 years prior to Stanozolol Rx, there was no clinical improvement after 14‐156 weeks of Rx despite normalization of stimulated tPA‐Fx and PAI‐Fx. The fifth patient, 1 month after onset of disabling hip pain, had normal PAI‐Fx but high Lp(a) (27 mg/dl), and MRI evidence of bone marrow edema (“transient osteoporosis”). After 3 weeks on Rx, Lp(a) normalized (14 mg/dl) and there was marked amelioration of symptoms. For the subsequent 11 weeks on Rx, this patient's Lp(a) was 5 mg/dl, and he became totally asymptomatic and remains asymptomatic 14 months later. We speculate that when ON is diagnosed prior to segmental collapse of the femoral head, it may be possible to reverse hypofibrinolysis, and/or to arrest the progression of ON. We postulate that high PAI or high Lp(a) lead to inadequate lysis of venous thrombi in bone, impaired bone venous circulation, venous hypertension of bone, and subsequent, potentially reversible development of ON. © 1995 Wiley‐Liss, Inc.
Treatment of acute myelogenous leukemia in patients over 50 years of age with V‐TAD: A southwest oncology group study
Authors: Bigelow Carolyn L. , Kopecky Kenneth , Files Joe C. , Head David , Lipschitz David A. , Grever Michael , Appelbaum Frederick R. 
Published: February 2007

AbstractAcute myelogenous leukemia (AML) in the elderly continues to have a poor prognosis and new treatment approaches are needed. This Phase II trial was undertaken to evaluate the complete remission rate and toxicity of a chemotherapeutic regimen including etoposide and 6‐thioguanine, combined with reduced doses of cytosine arabinoside and daunorubicin (V‐TAD) in individuals greater than 50 years of age with AML.Thirty‐five patients, ranging in age from 51 to 80 years (median, 66 years), were registered onto the study. Twenty‐nine patients were entered at the first dose level (daunomycin 20 mg/m2 days 1 and 2, ara‐C 75 mg/m2 days 1‐5, 6‐thioguanine 75 mg/m2 every 12 hr days 1‐5, and etoposide 50 mg/m2 days 1, 2, and 3) and six patients underwent therapy at the second dose level (ara‐C 75 mg/m2 days 1‐7 with the remainder of the regimen unchanged). After achieving a complete remission, patients underwent two to three consolidation cycles of chemotherapy.Thirty‐one patients were evaluable for response. Thirteen patients (ten of twenty‐five at the first dose level and three of six at the second dose level) achieved a complete remission (42%). Median remission duration was 6 months (range 1‐21 months).The current regimen, while tolerated, did not result in improved survival compared with prior treatment regimens because of a high incidence of resistant and recurrent leukemia. © 1995 Wiley‐Liss, Inc.
Characterization of two cases of acquired transitory von willebrand syndrome with ciprofloxacin: Evidence for heightened proteolysis of von willebrand factor
Authors: Castaman Giancarlo , Lattuada Antonella , Mannucci Pier Mannuccio , Rodeghiero Francesco 
Published: February 2007

AbstractWe characterized the cause of two cases of transitory acquired von Willebrand syndrome associated with the administration of ciprofloxacin. Purified lg from the two patients did not inhibit Ristocetin Cofactor activity or binding to collagen of normal plasma, ruling out the possibility of an inhibitor. The analysis of multimeric pattern of plasma von Willebrand Factor (vWF) showed the lack of larger multimers in both patients, with a relative decrease of all the remaining forms in the first patient. The subunit composition of plasma vWF showed a marked reduction of the native 225 Kd subunit (31.9% and 32.9%; normal range 74‐46%) and an increased proportion of the 189, 176, and 140 Kd fragments. These abnormalities disappeared during the follow‐up, without any specific therapy. In conclusion, a common pathophysiological basis is demonstrated in both patients, with a heightened proteolysis of plasma vWF by an unknown mechanism. © 1995 Wiley‐Liss, Inc.
Serum antibodies to distinct epitopes of the tissue‐type plasminogen activator (t‐PA) in patients with systemic lupus erythematosus
Authors: Ruiz‐Arguelles Alejandro , Angles‐Cano Eduardo , Perez‐Romano Beatriz , Ruiz‐Arguelles Guillermo J. , Deleze Margarita , Alarcon‐Segovia Donato , Gaussem Pascale 
Published: February 2007

AbstractDefective fibrinolysis due to decreased tissue‐type plasminogen activator (t‐PA) activity is a well‐established finding in patients with systemic lupus erythematosus (SLE). The possibility that this decrease in t‐PA activity may be related to the presence of autoantibodies directed against t‐PA, and the possible role of these autoantibodies in the patho‐physiology of fibrinolysis in SLE, were investigated. Serum samples from 115 SLE patients and 63 normal volunteers were analyzed for the presence of such antibodies. The search for antibodies to t‐PA was performed by means of several systems, allowing for the identification of epitopes presented in different conformational physical states of t‐PA: free or associated to its inhibitor (PAI‐1) in plasma, specifically bound to fibrin surface, or passively adsorbed to solid supports. Antibodies in variable amounts were detected by all systems used; however, t‐PA activity was not inhibited by the IgG fraction of the positive sera in a fibrin‐agar fibrinolysis system. Moreover, the demonstration of serum anti‐t‐PA antibodies was not associated with clinical or laboratory abnormalities related to vasoocclusive episodes. These results indicate that, as in the case of other autoantibodies, their detection in serum does not imply their direct participation in the pathophysiology of thrombosis in SLE. © 1995 Wiley‐Liss, Inc.
Peripheral blood neutrophil morphology reflects bone marrow dysplasia in myelodysplastic syndromes
Authors: Widell S. , Hellström‐Lindberg E. , Kock Y. , Lindberg M. , Öst Å. , Hast R. 
Published: February 2007

AbstractDysplastic features of cells in peripheral blood and bone marrow were studied in 51 patients with myelodysplastic syndromes (MDS) to evaluate the significance of the degree of neutrophil granulation (G‐score) and the percentage of pelgeroid polymorphs (ppp) in the peripheral blood, as indices of dysplastic changes in the bone marrow. There was a good correlation between peripheral blood and bone marrow findings, both for G‐score figures (r = 0.92, P < 0.01) and ppp (r = 0.82, P < 0.01). Significantly lower G‐score figures were found among patients with an increased percentage of bone marrow blasts (P < 0.05), while high ppp correlated with the presence of ring sideroblasts, the degree of bone marrow fibrosis, and findings of complex chromosomal abnormalities. Patients with a high degree of bone marrow dysplasia had significantly lower G‐score (P < 0.01) and significantly higher ppp (P < 0.05) figures, than those with less pronounced myelodysplasia. In addition, extreme hypogranulation (G‐score <150) or very high ppp (≥20%) was generally a sign of bi‐ and tri‐lineage dysplasia in the bone marrow. The results thus show that quantitative estimation of peripheral blood polymorph dysplasia by G‐score figures and ppp seems to reflect the total degree of bone marrow dysplasia in MDS and may serve as a complement to bone marrow evaluation when the diagnosis of MDS is difficult. © 1995 Wiley‐Liss, Inc.
Comparative study of multidrug resistance evaluated by means of the quantitative immunohistochemical detection of p‐glycoprotein and the functional release of rhodamine 123
Authors: Delville Jean‐Pierre , Pradier Olivier , Pauwels Olivier , Van Onderbergen Anne , Kiss Robert , Feremans Walter , Capel Paul 
Published: February 2007

AbstractThe immunological detection of P‐Glycoprotein (P‐GP) and the functional release of Rhodamine 123 (R123) have been compared in a number of human and murine cancer cell lines, in chemo‐and/or radiotherapy‐resistant subclones, and in clinical specimens from patients. The chemoresistance level was established from the viability index (IC50) in the presence of doxorubicin. Cytocentrifuge preparations were immunostained with JSB‐1 monoclonal antibody followed by the alkaline phosphatase anti‐alkaline phosphatase technique. The strength of the reaction was quantified by a digital image analyser. The kinetic incorporation and release of Rhodamine 123 were evaluated by flow cytometry. The parent cell lines and radiotherapy resistant subclones showed a low IC50, were JSB‐1 negative and retained R123 during the whole experiment, while the chemoresistant and radio‐chemoresistant cell line mutants had a high IC50, were JSB‐1 positive, and actively pumped the R123 out of the cells. Good correlations were obtained between the IC50, the digital image analysis, and flow cytometry. The kinetic profile of the R123 release allowed the distinction between typical and atypical multidrug resistance phenotypes. These findings were confirmed in clincal specimens from patients. We conclude that antigenic and functional studies are complementary and are useful in experimental and clinical approaches to multidrug resistance.
Establishment and characteristics of a gastric cancer cell line (HuGC‐OOHIRA) producing high levels of G‐CSF, GM‐CSF, and IL‐6: The presence of autocrine growth control by G‐CSF
Authors: Baba Masaru , Hasegawa Hiroshi , Nakayabu Masahiro , Shimizu Noriko , Suzuki Shiro , Kamada Nanao , Tani Kenzaburo 
Published: February 2007

AbstractWe successfully established a human gastric cancer cell line, HuGC‐OOHIRA, from the ascites of a 60‐year‐old patient with advanced gastric cancer (poorly differentiated adenocarcinoma) complicated by peritonitis carcinomatosa and leukocytosis of unknown origin. Morphologically, the cells were polygonal and adhered weakly to the culture flask. They tended to pile up upon reaching confluence. Chromosome analysis revealed that the cell line has two modes of chromosome number, namely near diploidy and tetraploidy. Double minutes (DMs) were present in abundance in each cell. The doubling time was 25‐30 hr. The cell line was successfully transplanted into nude mice, and their peripheral leukocyte counts increased in proportion to the growth of the tumors. At 2 weeks after the transplantation, the serum rG‐CSF level was elevated to 2,893 üg/ml. The concentration of human G‐CSF in the culture supernatants was an extraordinary high level of 145,380 pg/ml/day. Secretion of GM‐CSF and IL‐6 was also detected. The intracellular localization of the G‐CSF was identified for the first time by immunofluorescence. Moreover, Northern blot analysis detected G‐CSF mRNA in this cell line. Anti‐recombinant human G‐CSF serum suppressed the propagation of HuGC‐OOHIRA cell line. Therefore, it is likely that the autocrine growth loop by G‐CSF is present in this cell line. This cell line would be very useful for understanding both the cellular and molecular basis for the production of various cytokines such as G‐CSF as well as cytokine‐dependent tumor proliferation.
High grade malignant lymphoma with clinical characteristics and immunophenotype of natural killer cells
Authors: Schleiffenbaum Boris , Rüegg R. , Zimmermann D. , Fehr JÖRg 
Published: February 2007

AbstractThe malignant proliferation of natural killer (NK) cells which are morphologically characterized as large granular lymphocytes (LGL) is a well known clinical entity which was named after its morphological appearance as LGL‐leukemia/lymphoma. Similar to non‐malignant NK‐cells, these tumors can be divided into those which express the CD3‐T‐cell receptor complex and those which do not. The CD3‐positive type of LGL‐leukemia is immunophenotypologically characterized by the expression of CD16, and variably CD 56 and CD 57, and generally follows a more indolent course. In contrast, malignant proliferations of CD3‐negative LGL express either CD16 or CD 56, and only occasionally CD 57 on their cell surface. Clinically, CD3‐negative NK‐lymphomas tend to progress rapidly. We report here the case of a high grade malignant lymphoma which was characterized by an immunophenotype typical for CD3‐negative NK‐cells (CD2+, CD3−, CD16+, CD56(+), CD57−). The disease proved to be rapidly fatal despite aggressive chemotherapy. Interestingly, the patient suffered from a high turn over pancytopenia, which also characterizes NK‐cell leukemias/lymphomas of the LGL‐type. However, our patient's lymphatic cells appeared highly immature, and cytoplasmic granules, characteristic for LGL‐cells, could not be discerned either microscopically or electronmicroscopically. Furthermore, the malignant lymphatic population had the T‐cell receptor α‐chain rearranged. We therefore concluded that our patient might have suffered from a malignant proliferation of a putative precursor cell intermediate between T‐cells and NK‐cells. © 1995 Wiley‐Liss, Inc.
Anabolic‐Androgenic steroid abuse in weight lifters: Evidence for activation of the hemostatic system
Authors: Ferenchick Gary S. , Hirokawa Shinchirou , Mammen Eberhard F. , Schwartz Kenneth A. 
Published: February 2007

AbstractAnabolic‐androgenic steroid abuse has recently been linked with acute vascular events in athletes. To date, the relationship between steroid abuse and the potential for cardiovascular disease has been considered almost exclusively in terms of lipid metabolism. However, recent reports of thrombosis in androgen abusing athletes with no evidence of atherosclerosis suggests the hypothesis that thrombosis risk in such athletes could be mediated through androgen induced abnormalities of coagulation.To determine if anabolic‐androgenic steroid abuse in weight lifters is associated with an activation of the hemostatic system we studied forty‐nine weight lifters recruited through advertisements. History of androgen use or abstinence was confirmed via urine assays. Plasma was assayed for clotting and fibrinolytic activity by measuring thrombin/ antithrombin complexes (TAT), prothrombin fragment 1 + 2 (F1 + 2), and D‐dimers (D‐di); markers of the endothelial based fibrinolytic components were assayed by measuring tissue plasminogen activator antigen (t‐PA Ag) and its inhibitor (PAI‐1); finally, the activity of antithrombin III, protein C, and protein S were measured.Abnormally high concentrations of TAT complexes were noted in 16% of our confirmed steroid using weight lifters compared to 6% of our confirmed nonusers (P = .01). Steroid users also demonstrated abnormally high concentrations of F1 + 2 and D‐dimers when compared to nonusers (44 vs. 24%, P < .001, and 9 vs. 0%, respectively). Non‐steriod users were more likely to have elevated levels of t‐PA Ag and PAI‐1 than our steroid using weight lifters (both P < .001). The activities of antithrombin III and protein S were more likely to be higher in users compared to nonusers (22 vs. 6%, P = .005; 19 vs. 0%, respectively).Some anabolic‐androgenic steroid using weight lifters have an accelerated activation of their hemostatic system as evidenced by increased generation of both thrombin and plasmin. These changes could reflect a thrombotic diathesis that may contribute to vascular occlusion reported in young athletes using these drugs. The predictive value of these coagulation abnormalities in terms of risk of thrombosis to individual steroid using weight lifters or the population as a whole remains to be studied. © 1995 Wiley‐Liss, Inc.
Platelet von willebrand factor abnormalities in myeloproliferative syndromes
Authors: Castaman Giancarlo , Lattuada Antonella , Ruggeri Marco , Tosetto Alberto , Mannucci Pier Mannuccio , Rodeghiero Francesco 
Published: February 2007

AbstractPlasma and platelet von Willebrand factor (vWF) measurements, multimeric pattern and subunit composition of plasma vWF were obtained in 29 consecutive patients with chronic myeloproliferative syndromes. In the 8 patients with chronic myelogenous leukemia (CML), plasma vWF was significantly higher than in the 11 patients with essential thrombocythemia (ET) and in the 10 patients with polycythemia Vera (PV). The RiCof/ vWF:Ag ratio was low in all these groups of patients (mean 0.64 = 0.1, 0.66 = 0.2; and 0.61 = 0.2; normal 0.97 = 0.2). Bleeding time was prolonged (>7.5 min) in 1/8 CML patients, 1/10 with PV, and 3/11 with ET. Plasma vWF multimers showed a minor loss of the largest multimers in 3/8 patients with CML, 4/10 with PV, and a more severe reduction in 9/11 ET patients. The latter pattern correlated with an abnormal proteolysis of vWF, expressed by a major increase of the 140‐Kd fragment and decrease of the intact 225‐Kd subunit in ET patients, whereas the 176‐Kd fragment was significantly increased in all the subgroups of patients. Platelet vWF was significantly higher in CML patients in comparison to ET and normal controls. However, minor losses of the larger multimers were evident in all the subsets of patients. In ET patients also the intermediate forms were lacking in platelets, accompanied by a significant decrease of platelet RiCof. This abnormality was significantly correlated with the occurrence of bleeding symptoms in PV and ET patients (P = 0.007; Fisher's exact test). In conclusion, plasma and platelet vWF abnormalities are common findings in myeloproliferative syndromes and are more severe in ET. The more pronounced platelet vWF abnormalities in ET may reflect the more frequent bleeding symptoms observed in this disorder. © 1995 wiley‐Liss, Inc.
Late appearance of the Philadelphia chromosome with monosomy 7 in a patient with de novo AML with trilineage myelodysplasia
Authors: Matsue Kosei , Miyamoto Toshihiro , Ito Mami , Tsukuda Koji 
Published: February 2007

AbstractWe report here a patient with de novo acute myelocytic leukemia with trilineage myelodysplasia (AML/TMDS) in whom cytogenetic analysis was normal at diagnosis and in whom Philadelphia chromosome (Ph1) with monosomy 7 emerged at the terminal stage of the disease. Reverse transcription polymerse chain reaction (RT‐PCTR) on the Ph1 positive specimen detected the mRNA encoding p190bcr/ab1 was not detected at diagnosis and or at relapse. These observations support the hypothesis that late‐appearing Phl and monosomy 7 might be the result of clonal evolution during the course of leukemia, and p190bcr/abl might play a role in the terminal transformation or progression of leukemia. © 1995 Wiley‐Liss, Inc.
Severity of β‐thalassemia due to genotypes involving the IVS‐I‐6 (T→C) mutation
Authors: Waye John S. , Eng Barry , Patterson Margaret , Wasi Parveen , Chui David H. K. , Francombe William H. , Sher Graham D. , Olivieri Nancy F. 
Published: February 2007

AbstractAmong individuals of Mediterranean or Middle Eastern descent, the IVS‐I‐6 (T→C) mutation is one of the most common causes of β‐thalassemia. In this report, we describe the clinical phenotypes of a group of β‐thalassemia patients who are compound heterozygotes for the relatively mild IVS‐I‐6 (T→C) β‐thalassemia mutation and more severe β+‐ or β°‐thalassemia mutations. Although most of these patients are transfusion‐dependent, the requirement for regular transfusions generally occurred late in childhood. A correlation between concomitant α‐thalassemia and a mild transfusion‐independent phenotype is not apparent, indicating the involvement of other ameliorating determinants.
Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome secondary to pancreatitis
Authors: Silva Victor A. 
Published: February 2007

AbstractPancreatitis is a rare (∼2.0%) complication of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). The opposite finding has rarely been reported. We present a case of an 18 years old obese male with alcohol associated pancreatitis (amylase 840 IU/L) who three days after onset, as the pancreatitis subsided (amylase 341 U/L), developed TTP/HUS. The TTP/HUS was marked by oliguria and severe renal failure (creatinine 1,326 μmol/L), was treated with daily plasma exchanges, obtained a complete response, and recovered renal function (creatinine 115 μmol/L). Similarly, in six of seven other cases from the medical literature the TTP/HUS occurred within 2–3 days of the onset of pancreatitis.
EDTA‐dependent pseudothrombocytopenia: A clinical and epidemiological study of 112 cases, with 10‐year follow‐up
Authors: Bizzaro Nicola 
Published: February 2007

AbstractIn the past 10 years, we have observed 112 cases of EDTA‐dependent pseudothrombocytopenia (PTCP) due to in vitro platelet clumping at room temperature. 93 patients had antiplatelet antibodies (48 IgM, 30 IgG, 3 IgA, and 12 had two different isotypes concomitantly). In 20% of patients, the presence of IgM antibodies characteristically accompanied platelet agglutination also at 37°C, and in citrated blood. The phenomenon was not age or sex related, nor was it associated with any particular pathology or use of specific drugs, and was present in both healthy subjects and patients with various diseases.Flow cytofluorimetric analysis of CD5‐positive B cells, which are responsible for autoantibody production, did not demonstrate any changes in the percentage and absolute number of this lymphocyte subset.Average follow‐up was 5 years (6 months‐10 years); however, previous clinical records disclosed that PTCP was present for more than 15 years in four cases, and more than 20 years in three others, with no clinical manifestation of disease.This study confirms that EDTA‐dependent PTCP is a phenomenon related to the presence of natural autoantibodies with antiplatelet activity, devoid of pathological significance. Its clinical interest resides in the need for its prompt and certain recognition in order to avoid unnecessary examinations and therapeutic interventions.The best and most rapid technique for obtaining accurate platelet counts in PTCP subjects is to collect and examine EDTA blood at 37°C; however, clumping will still be present in about 20% of these cases, and even in citrated blood. To obviate this phenomenon, blood should be collected in ammonium oxalate, and platelets counted in a Burker chamber.