bluuebunny/crossref_metadata_2025_split huggingface dataset:
Reproducibility and temporal stability of ADP‐induced platelet aggregation: Comparison of the anticoagulants sodium citrate and D‐phenylalanyl‐L‐prolyl‐L‐arginyl chloromethyl ketone
Authors: Horne William C. , Cohen Donald J. , Anderson George M. 
Published: February 2007

AbstractWe compared the reproducibility and temporal stability of ADP‐induced aggregation of platelet‐rich plasma anticoagulated with citrate or with D‐phenylalanyl‐L‐prolyl‐L‐arginyl‐chloromethyl ketone (PPACK), a thrombin inhibitor. Citrate‐ or PPACK‐platelet‐rich plasma (PRP) was stored at room temperature in capped plastic tubes or in plastic syringes from which all air was expelled. At intervals over 1‐5 hr after venipuncture, platelet aggregation was induced by 1‐1.5 μM ADP. Initially, the aggregation of PPACK‐PRP was nearly twice that of citrate‐PRP. The response of PPACK‐PRP stored in the syringe remained essentially constant over the interval of study, in contrast to the responses of the other samples which declined with time. The improved stability of the response obtained from samples anticoagulated with PPACK was due to the a
citrate, since PRP containing both citrate and PPACK became less responsive over time a manner similar to PRP which contained only citrate. Anticoagulation with PPACK rather than citrate results in a more reproducible and stable aggregation response and more closely reproduces the in vivo environment of the platelet.
Hb S/β°‐Thalassemia due to the ˜1.4‐kb deletion is associated with a relatively mild phenotype
Authors: Waye John S. , Chui David H. K. , Eng Barry , Cai Shi‐Ping , Coleman Mary B. , Adams Junius G. , Steinberg Martin H. 
Published: February 2007

AbstractWe report a relatively mild phenotype associated with two siblings who are compound heterozygotes for Hb S and a β°‐thalassemia mutation due to a ˜1.4‐kb deletion of the 5′ region of the β‐globin gene. Each is found to have unusually high levels of Hb A2 and Hb F, accounting for more than 20% of the total hemoglobin. These may interfere with intracellular Hb 5 polymerization, thus leading to a mild clinical course.
Intravenous gammaglobulin treatment for immune thrombocytopenia associated with infectious mononucleosis
Authors: Cyran Elizabeth M. , Rowe Jacob M. , Bloom Robert E. 
Published: February 2007

AbstractSevere thrombocytopenia is an uncommon (incidence <1%) but serious complication of infectious mononucleosis. Corticosteroids have been used for therapy with variable responses reported. Five consecutive patients with infectious mononucleosis‐related severe thrombocytopenia were treated with intravenous gammaglobulin (IVIG) at a dose of 400 mg/kg/day for 2–5 days. All patients appear to have had an immunologic or consumptive etiology for their thrombocytopenia as determined by increased marrow megakaryocytes. All patients were initially treated with oral prednisone 1 mg/kg/day. Due to the relatively slow response to prednisone (platelet count >20,000/γL on the 8th to 13th hospital day) or increased bleeding symptoms, IVIG was initiated. Four of the five patients rapidly developed significant increases in their platelet counts (range 44,000/γL to 97,000/γL). Two of these responses were sustained and two relapses occurred (while on continued steroid therapy) which again responded to booster doses of IVIG at similar doses. IVIG has been previously shown to be effective in treating patients with idiopathic thrombocytopenia purpura. Historically, patients with infectious mononucleosis‐related severe thrombocytopenia often are refractory to corticosteroid therapy and our limited experience suggests that IVIG may also be effective in infectious mononucleosis‐related severe thrombocytopenia.
Hematologic improvement in a patient with gaucher disease on long‐term enzyme replacement therapy: Evidence for decreased splenic sequestration and improved red blood cell survival
Authors: Parker Robert I. , Read Elizabeth J. , Barton Norman W. , Brady Roscoe O. 
Published: February 2007

AbstractWe describe here an investigation of the hematologic response of a child with Gaucher disease to a six‐year therapeutic trial of human placental mannose‐terminated glucocerebrosidase. While on enzyme replacement therapy, the patient's hemoglobin and platelet count significantly increased (6.9 g/dl to 12.2 g/dl, and 39,000/γL to 74,000/γL, respectively). Over the same time interval his absolute reticulocyte count decreased (88.1 × 103/γL to 31.5 × 103/γL). The patient's splenic volume decreased by over 60% (2108ml to 797ml) and his bone marrow demonstrated a dramatic clearing of Gaucher cell infiltration. Serum erythropoietin levels were at the lower range of normal at points in time when he was severely anemic and did not change with improvement in hemoglobin. Coincident with the improvement in the patient's hemoglobin, in vivo survival of autologous radiolabeled RBCs increased 34%. The improved hematopoietic profile appeared to result primarily from an increase in the in vivo survival of mature hematopoietic elements secondary to a decrease in splenic sequestration. Improved marrow function secondary to the clearing of Gaucher cell infiltrates may also playa role in his improved hematologic status.
Hyperammonemia following allogeneic bone marrow transplantation
Authors: Tse Nielson , Cederbaum Stephen , Glaspy John A. 
Published: February 2007

AbstractHyperammonemia is a rare and serious complication of intensive cytotoxic chemotherapy. We report the case of a patient who developed profound idiopathic hyperammonemia following bone marrow transplantation for chronic myelogenous leukemia. Despite rapid institution of hemodialysis, sodium benzoate, and the experimental agent sodium phenylacetate, the patient ultimately succumbed to complications of this metabolic derangement. The literature is reviewed, and recommendations for an approach to this complication of marrow transplantation are proposed.
Double heterozygosity of the β‐malay and a novel β‐thalassemia gene in a thai patient
Authors: Fucharoen Supan , Fucharoen Goonnapa , Laosombat Vichai , Fukumaki Yasuyuki 
Published: February 2007

AbstractThe β‐globin genes from a Thai patient compound heterozygous for β+‐ and β° thalassemias were investigated. Amplification and DNA analysis of genomic DNA by the polymerase chain reaction procedure permitted the identification of the β‐Malay mutation (β 19;AAC‐AGC) in one allele and a novel β°‐thalassemia mutation (41;TTC‐TT) in another allele.
Gelatinous transformation of marrow in a case of acute myelogenous leukemia post‐chemotherapy
Authors: Feng Chi‐Shun 
Published: February 2007

AbstractGelatinous transformation of marrow in a patient with acute myelogenous leukemia was reported. This case was at variance with other reported cases of gelatinous transformation of marrow in that a) it was associated with a primary bone marrow disease and b) there was no fat atrophy and the patient was not cachetic. The fact that it occurred abruptly post‐chemotherapy suggested that marrow hypoplasia induced by chemotherapy could be the cause of this lesion in this particular situation.
A novel α‐thalassemia‐2 (−2.7‐kb) observed in a chinese patient with Hb H disease
Authors: Zhao Jing‐Bo , Zhao Lin , Fei You‐Jun , Liu Jin‐Cai , Huisman Titus H. J. 
Published: February 2007

AbstractWe describe a newly detected α‐thalassemia‐2 (α‐thal‐2) deletion characterized by a small −2.7‐kb deletion involving the α1 globin gene. This deletion has thus far been observed in only one Chinese subject with Hb H disease.
Intravenous immunoglobulin in the treatment of human immunodeficiency virus‐related thrombocytopenia
Authors: Rarick Mark U. , Montgomery Terri , Mazumder Amitabha , Gill Parkash S. , Loureiro Carmen , Levine Alexandra M. , Groshen Susan , Sullivan‐Halley Jane , Jamin Denise 
Published: February 2007

AbstractFourteen patients with sexually transmitted human immunodeficiency virus (HIV)‐related immune thrombocytopenia were treated with intravenous gammaglobulin (IVIG). The patients were treated with a uniform program consisting of 1 g/kg of IVIG on day 1 and day 2, followed by 1 g/kg on day 15. Most patients had pretreatment bleeding symptoms, which included petechiae, spontaneous and traumatic ecchymoses, gum bleeding, and epistaxis. Median baseline platelet count was 17,000/mm3 (range 3–61,000/mm3). After the infusion of the IGIV, all patients had a resolution of their bleeding by day 8. The median maximum platelet count achieved with the IGIV was 220,000/mm3 (range 76–426,000/mm3). No patient achieved either a sustained complete or partial remission after the conclusion of the IVIG therapy. Toxicities were minimal with the majority being headache and nausea. In conclusion, patients with sexually transmitted HIV infection and immune thrombocytopenia respond favorably to IVIG. This treatment should be considered as first‐line therapy for patients with HIV‐related immune thrombocytopenia who require immediate but temporary increase in their platelet count, attributable to symptoms or signs of clinical bleeding or because of the need for an invasive procedure.
Plasma levels of soluble thrombomodulin increase in cases of disseminated intravascular coagulation with organ failure
Authors: Asakura Hidesaku , Jokaji Hiroshi , Saito Masanori , Uotani Chika , Kumabashiri Ichiro , Morishita Eriko , Yamazaki Masahide , Matsuda Tamotsu 
Published: February 2007

AbstractWe examined the changes in plasma levels of soluble thrombomodulin in 66 cases of disseminated intravascular coagulation (DIC), to investigate the damage to vascular endothelial cells and its relationship to multiple organ failure. A significant elevation of plasma levels of soluble thrombomodulin was observed in most cases of DIC, especially in patients with sepsis. However, no such significant elevation was observed in patients with acute promyelocytic leukemia. Plasma levels of both soluble thrombomodulin and active plasminogen activator inhibitor were higher in the cases of DIC with multiple organ failure than in those without multiple organ failure. The levels of soluble thrombomodulin were decreased with the clinical improvement in most cases of DIC but were further increased or remained at high levels in patients who showed no improvement of DIC. It was suggested that an increase in soluble thrombomodulin indicates the damage to the vascular endothelial cells in cases of DIC and that the damage to vascular endothelial cells plays some role in further progression of multiple organ failure.
An effective acute graft‐vs.‐host disease prophylaxis with minidose methotrexate, cyclosporine, and single‐dose methylprednisolone
Authors: Yau Jonathan C. , Huan Susan D. , Dimopoulos Meletios A. , Woo Shiao Y. , Brunner Lane J. , Wallerstein Ralph O. , Deisseroth Albert B. , Andersson Borje S. , Spencer Verneeda , Spitzer Gary , LeMaistre C. Frederick 
Published: February 2007

AbstractCyclosporine and methotrexate at standard doses (15 mg/m2on day 1 and 10 mg/m2on days 3, 6, and 11, total 45 mg/m2) are effective in the prophylaxis of actue graft‐vs.‐host disease. However, the combination has significant early toxicities with delayed engraftment, increased mucositis, and hepatotoxicity. We modified the combination by adding single‐dose methylprednisolone and lowered the total dose of methotrexate to 35 mg/m2(5 mg/m2on days 1, 3, and 6, and then 10 mg/m2on days 11 and 18) and then to 20 mg/m2(5 mg/m2on days 1, 3, 6, and 11) in an attempt to decrease these side effects in two sequential consecutive groups of patients. We demonstrated that the modified regimens maintained the efficacy with reduced toxicities. The rate of engraftment was comparable to cyclosporine alone and the hepatotoxicity was reduced with reduced doses of methotrexate. Factors such as early immunosuppression of the host, intravenous immunoglobulin, the timing of steroid administration, nucleotide free diet and germ free environment may contribute to the effectiveness of the combination and permit reduction of methotrexate dose.
Platelet thrombospondin and glycoprotein IV abnormalities in patients with essential thrombocythemia: Effect of α‐interferon treatment
Authors: Legrand C. , Bellucci S. , Disdier M. , Tobelem G. , Edelman L. 
Published: February 2007

AbstractPlatelet aggregability and some biochemical parameters were evaluated in seven patients with essential thrombocythemia (ET) compared with seven patients with secondary thrombocytosis (ST). Defective platelet aggregation with one or more agonists was seen in five patients with ET whereas aggregation was increased in two other patients. In addition, three patients with ET demonstrated spontaneous platelet aggregation in citrated plasma. This was associated with increased level of thrombospondin (TSP) in the plasma membrane. Interestingly, the presence of a proteolyzed 160 kDa form of TSP was detected in all patients with ET, whereas it was never found in patients with ST. Furthermore, three patients with ET demonstrated increased levels of platelet surface glycoprotein IV (GP IV), the putative receptor for TSP in the plasma membrane. In two of these patients, this correlated with increased surface expression of TSP and spontaneous platelet aggregation. The results suggest a possible link between the increased number of plasma membrane GP IV molecules, the spontaneous expression of TSP on the platelet surface and platelet hyperaggregability in some ET patients. The levels of plasma membrane GP IV and platelet surface‐associated TSP tended to be normalized during α‐interferon treatment, whereas the presence of an altered form of TSP persisted. This last parameter might be of practical usefulness in the characterization of the disease, permitting a clear distinction from ST.
Acute megakaryoblastic leukemia with translocation t(1;22)(p13;q13) in a 10‐week‐old infant
Authors: Washio Setsuko , Ido Masaru , Azuma Eiich , Fukui Masako , Shibata Takeo , Zhang Shao‐Li , Komada Yoshihiro , Ito Masahiro , Koike Tadashi , Kaneko Yasuhiko , Sakurai Minoru 
Published: February 2007

AbstractA 10‐week‐old girl without Down syndrome developed an acute megakaryoblastic leukemia (AMKL). Bone marrow aspirates and biopsy showed megakaryoblastic infiltration with myelofibrosis. The diagnosis was made based on the findings that the positive reactions of leukemic cells to platelet peroxidase and to monoclonal antibodies which recognize platelet‐specific surface glycoprotein (GP) Ilb/IIIa and GP78. The blasts also showed myeloid and monocytoid differentiation antigens. The leukemic cells had a karyotype of 46,XX,t(1;22)(p13;q13). Our case and two other infantile cases reported by other investigators establish the novel association of the t(1;22) with AMKL.
Protein‐bound cobalamin absorption declines in the elderly
Authors: Scarlett John D. , Read Helen , O'Dea Kerin 
Published: February 2007

AbstractA modified protein‐bound cobalamin absorption test was used to study dietary cobalamin absorption in healthy adults of different age groups and patients with isolated low serum concentrations of cobalamin. Dietary cobalamin absorption was significantly reduced in healthy adults aged 55‐75 years compared with young adults, with a further reduction in those older than 75 years. No difference was detected between dietary cobalamin absorption in patients with isolated low serum cobalamin and controls of a similar age group. Cobalamin malabsorption was associated with elevated serum gastrin. The diagnostic value of this protein‐bound cobalamin absorption test in the elderly was limited by the frequent finding of reduced absorption in healthy elderly people with normal serum cobalamin concentrations. The performance of such tests should be evaluated in different age groups before application in diagnosis.
Association of thyroid disease with acute leukemia
Authors: Moskowitz Craig , Dutcher Janice P. , Wiernik Peter H. 
Published: February 2007

AbstractThe purpose of this paper is to evaluate the possible clinical association of autoimmune thyroid disease and acute leukemia. This study is a retrospective review of all adult patients with acute leukemia treated at our institutions from 1978‐1989. Those with both leukemia and thyroid disease were evaluated and are presented. Twenty‐seven of eight hundred seventy patients with acute leukemia had evidence of thyroid disease. This is a 3‐fold increase in overall incidence. Twenty‐one patients had acute myeloid leukemia, five had acute lymphoid leukemia, and one had accelerated chronic myeloid leukemia. Thyroid diseaseentities included toxic multinodular goiter (four patients), idiopathic hypothyroidism (eight patients), Graves' disease (eight patients), and Hashimoto's thyroiditis (seven patients). Whereas the association may have had a negative effect on elderly patients, those with Graves' disease and Hashimoto's disease appeared to have an improved outcome of the leukemia. There is an increased association of autoimmune thyroid disease and acute leukemia. Since thyroid hormones are important regulators of hematopoiesis and utilize receptors similar to those of differentiating factors such as retinoids, the association may be important for further study of mechanisms of growth regulations in leukemia.
Severe osteopenia and vertebral compression fractures after complete remission in an adolescent with acute leukemia
Authors: Vassilopoulou‐Sellin Rena , Ramirez Irma 
Published: February 2007

AbstractAcute leukemia frequently presents with skeletal symptoms; however, the etiologies of bone pain and osteopenia are poorly understood. We present the case of an adolescent girl with acute leukemia who developed vertebral fractures after being in complete remission for 3 months. Bone mineral density was very low (<0.6 g/cm2) but remained stable during the subsequent 3 months despite continuation therapy with methotrexate and prednisone. Findings of a bone biopsy were consistent with a recent episode of aggressive bone resorption. We suggest that paracrine secretion of lymphokines may mediate loss of bone mass during the active phase of acute leukemia, although clinical bone disease may not develop until after complete remission is achieved.
Fluctuations in serum cytokine levels in the patient with cyclic neutropenia
Authors: Yujiri T. , Shinohara K. , Kurimoto F. 
Published: February 2007

AbstractThe fluctuations of the levels of serum cytokines in a patient with cyclic neutropenia were studied. The greatest fluctuation was found in granulocyte colony‐stimulating factor (G‐CSF). Tumor necrosis factor‐α level fluctuated inversely with fluctuation of G‐CSF, and oscillation of interleukin (IL)‐6 level preceded that in G‐CSF level. It is likely that the variations of the levels of cytokines play a significant role in regulating hematopoiesis in cyclic neutropenia. Our results suggested that the stage of the granulocyte‐committed stem cell is the major step of the defect, and the defect at the stage of the multipotent stem cell or bone marrow microenvironment was also suggested.
Radioimmunoassay for the measurement of serum IL‐6 and its correlation with tumour cell mass parameters in multiple myeloma
Authors: Solary Eric , Guiguet Michel , Zeller Véronique , Casasnovas René‐Olivier , Caillot Denis , Chavanet Pascal , Guy Henri , Mack Gérard 
Published: February 2007

AbstractInterleukin‐6 (IL‐6) was demonstrated to be a strong autocrine or paracrine plasmocytoma cell growth factor in humans. Using a bioassay, high serum IL‐6 (S‐IL‐6) levels were correlated with disease severity in plasma cell dyscrasias. Since other cytokines could interfere with the bioassays, we developed a specific radioimmunoassay to study S‐IL6 levels in 102 patients with monoclonal gammopathy (MG). S‐IL‐6 level was studied by a double antibody radioimmunoassay using a rabbit polyclonal anti‐IL‐6 antibody and a human recombinant IL‐6 as the standard. The lowest value of the standard significantly different from zero was found to be 78 pg/ml. Within‐run and between‐run precisions were characterized by a mean coefficient of variation of 3.72 and 5.5%, respectively. The mean analytical recovery was found to be 113% and the immunochemical identity of IL‐6 standard and S‐IL‐6 was shown by dilution tests. IL‐6 was detected in all tested sera. Sera from 66 healthy volunteers and 43 patients with acute leukemia or malignant lymphoma were tested as controls. In healthy subjects, S‐IL‐6 values were 294 ± 86 pg/ml. MG were classified as multiple myeloma (MM), macroglobulinemia, and MG of undetermined significance (MGUS). The distribution of S‐IL‐6 levels in patients with MG was significantly higher than in healthy subjects but lower than in patients with acute leukemia or Hodgkin's lymphoma. Results obtained in 55 patients with MM were related to other biological parameters. S‐IL‐6 levels correlated with bone‐marrow plasmacytosis (P < .0005), serum‐lactate dehydrogenase (S‐LDH; P < .005), serum β2 microglobulin (S −β2m; P < .01), and serum calcium (S‐Ca; P < .025) and inversely correlated with haemoglobin (P < .025). Our results indicate that 1 radioimmunoassay is suitable for the measurement of human IL‐6 in serum; 2 high S‐IL‐6 levels are observed in a small number of patients with MG; and 3 S‐IL‐6 level correlates with tumour cell mass in patients with overt MM.
Immunological evaluation of harvested stem cells obtained by leukapheresis after chemotherapy
Authors: Teixeira G. , Lenormand B. , Jean P. , Fardoun D. , Sumereau‐Dassin E. , Bastit D. , Tilly H. , Piguet H. , Monconduit M. , Vannier J. P. 
Published: February 2007

AbstractSome patients suffering from malignancies may benefit of myeloablative chemotherapy followed by hematological reconstitution with autologous peripheral blood reinfusion. A quick evaluation of the number of hematopoietic progenitors present in leukapheresis blood samples is necessary to ensure the collection of a sufficient number of these cells. A study was performed on a series of 25 leukapheresis following initial chemotherapy.The number of granulomonocytic colony‐forming unit (CFU‐GM) and the number of CD34+ cells were evaluated simultaneously, in each sample. Results have shown a relatively strong linear correlation between both methods of evaluation of hematopoietic progenitors, suggesting that immunophenotyping could be a useful method to estimate the number of progenitors.
Granular lymphocyte leukemia with pure red cell aplasia: Usefulness of gene analysis in assessing therapeutic effect
Authors: Motoji Toshiko , Yamada Osamu , Takahashi Masatomo , Oshimi Kazuo , Mizoguchi Hideaki 
Published: February 2007

AbstractA patient with granular lymphocyte leukemia (GLL) of the CD3+, CD4‐, CD8+ phenotype accompanied by pure red cell aplasia (PRCA) is described. Surface marker analysis, nonmajor histocompatibility complex (MHC)‐restricted cytotoxicity assay, gene analysis, and in vitro colony assay were performed on the granular lymphocytes before and after treatment. Cyclophospharnide therapy was highly effective, and after remission clonal granular lymphocytes were no longer identified by T‐cell antigen receptor (TCR) gene analysis or surface marker analysis. Lymphocytes obtained after remission did not exhibit elevated levels of non‐MHC‐restricted cytotoxicity, nor did they demonstrate a suppressive effect on erythroid colony formation. TCR gene analysis proved to be a sensitive parameter for evaluating the residual malignant granular lymphocytes. Gene analysis will be useful both for timing the discontinuation of treatment and for the early detection of relapse. Various factors possibly related to the development of PRCA in this patient were investigated and their significance is discussed.