bluuebunny/crossref_metadata_2025_split huggingface dataset:
Expression of multiple β1 integrins on circulating monoclonal B cells in patients with multiple myeloma
Authors: Jensen Gitte S. , Belch Andrew R. , Mant Michael J. , Ruether Bernard A. , Yacyshyn Bruce R. , Pilarski Linda M. 
Published: February 2007

AbstractWe have previously reported the presence of monoclonal, tumor‐related B lineage cells in the blood of myeloma patients. The cells are continuously differentiating, and the majority are at a very late stage of B cell differentiation into plasma cells, consistent with the hypothesis that they comprise a precursor cell subset responsible for disseminating and possibly for relapse of the disease. The pattern of β1 integrin expression on monoclonal B lineage cells from blood and bone marrow of myeloma patients was evaluated using multiparameter flow cytometry in comparison to normal blood or tissue B cells and malignant B cells from B‐CLL, B lymphoma, or plasma cell leukemia. The α4 and β1 chains were found on the majority of normal B cells, usually with a higher expression of α4 compared to β1. α5 was detectable at low density on B cells from lymph node, bone marrow, and lamina propria. The α2 and α6 chains are absent on B cells localized in normal lymphoid tissues as well as on normal blood B cells and in vitro activated B cells. In myeloma, the blood B cells express α2, α5 and α6, suggesting important functional differences between these tumor‐related B cells and their normal counterparts. The plasma cells located in myeloma bone marrow express no α2, and almost no α6, although they have variable expression of α4, α5, and β1. Thus the end‐stage plasma cells appear to lack receptors that would support a propensity for invasion of basement membranes and exit to extravascular spaces. In contrast, the circulating plasmablasts in a patient with plasma cell leukemia make up a large subset of early plasma cells expressing all integrin receptors analyzed, including α2 and α6. Malignant cells from B‐CLL and B lymphoma express only the α4 and β integrins, and some B‐CLL have very low levels of α3, but no α2, α5, or α6, suggesting that they may be limited to the vascular spaces and do not extravasate, at least for the stages of disease analyzed here. Our results are therefore consistent with a working hypothesis that invasive capacity in myeloma is to be found within the abnormal monoclonal B cells in peripheral blood, which alone among B cells or plasma cells, and like plasma cell leukemia, express multiple β1 integrins, including the α2 and α6 integrin receptors, necessary for cellular translocation across the endothelial basement membranes and into extravascular spaces. This study raises the possibility that expression of, in particular, the α2 and α6 integrins may underlie the invasiveness of these diseases and offers a new perspective for future avenues of clinical intervention.
Ultrastructure of plasma cells in a patient with J chain disease
Authors: Sawamura Morio , Kurabayashi Hitoshi , Murakami Hirokazu , Sakura Tohru , Tamura Jun'Ichi , Kubota Kazuo , Naruse Takuji , Tsuchiya Jun 
Published: February 2007

AbstractUltrastructural analysis of plasma cells was performed in a 74‐year‐old female patient with J chain disease. By electron microscopy, various characteristic findings were observed: lobulated nuclei, multinuclei, single sac loop‐like structures, flocculent filament‐like substances, nuclear inclusion bodies, cytoplasmic inclusion bodies, and multilamellar bodies. Our prior studies pointed out a poor prognosis in myeloma patients with these findings.
Correction of neutrophil chemotaxis defect in patients with gaucher disease by low‐dose enzyme replacement therapy
Authors: Zimran A. , Abrahamov A. , Aker M. , Matzner Y. 
Published: February 2007

AbstractWe have recently described a chemotactic defect in severely afflicted Gaucher disease patients. Two of the patients were treated with low‐dose intravenous enzyme replacement (Ceredase). Marked improvement in their hematological status, organomegaly, and growth was observed. In addition, their chemotactic defect and their tendency towards infections were corrected within 1 year of treatment.
Adult‐onset cyclic neutropenia responsive to cyclosporine therapy in a patient with ankylosing spondylitis
Authors: Storek Jan , Glaspy John A. , Grody Wayne W. , Susi Ellen , Slater Evan D. 
Published: February 2007

AbstractA 45‐year‐old female with a long history of HLA‐B27‐positive ankylosing spondylitis and ulcerative colitis developed cyclic neutropenia. She was hospitalized for high fever during each of three consecutive episodes of absolute neutropenia. On the third hospitalization, granulocyte‐colony‐stimulating factor (G‐CSF), 5 μg/kg/day, was given by subcutaneous injection and resulted in an increase of absolute neutrophil count from 0 to 2.2 × 109/liter and an associated decrease of platelet count and hemoglobin as well as severe bone and joint pain predominantly in the middle and lower back and purulent diarrhea. The back pain necessitated discontinuation of the drug. Oral cyclosporine therapy was begun, and although the neutrophil count continued to oscillate, both the peaks and the nadirs were higher than previously, and symptoms of neutropenia subsided. We conclude that cyclosporine can be an effective treatment for cyclic neutropenia associated with autoimmunity since G‐CSF may cause exacerbations of autoimmune disorders.
High la (HLA‐DR) and low CD11b (Mo1) expression may predict early conversion to leukemia in myelodysplastic syndromes
Authors: Mittelman Moshe , Karcher Donald S. , Kammerman Lisa A. , Lessin Lawrence S. 
Published: February 2007

AbstractThe FAB classification of myelodysplastic syndromes (MDS) has been useful in predicting prognosis; however, additional methods are required to detect patients at high risk for early conversion to acute nonlymphoblastic leukemia (ANLL). Using a panel of monoclonal antibodies to myelomonocytic surface antigens (MMSA) and flow cytometry, we studied bone marrow cells from 26 patients with MDS of all five FAB subtypes. The MMSA studied included la (HLA‐DR), CD11b (Mo1), CD14 (Mo2, My4), CD13 (My7), and CD33 (My9). Marrows were considered 'positive' for a given MMSA if the percentage of reactive cells exceeded the upper limit of the normal range. Twenty‐four of twenty‐six patients (92.3%) were CD13 (My7)+, suggesting that CD13 may serve as a diagnostic marker for MDS. Ten of twelve patients who developed ANLL during a median follow‐up of 44 weeks were la(HLA‐DR)+. The Kaplan‐Meier estimated median time to leukemia (TTL) was 16 weeks for la+ patients and 88 weeks for la– patients (P = 0.004). All six patients who developed ANLL before 16 weeks from diagnosis were la+, while none of the la– patients converted to ANLL before 24 weeks. Nine of thirteen patients with low CD11b (Mo1) expression (<53% reactive cells) developed ANLL, compared with only two of 11 patients with high CD11b expression (>53% reactive cells). Kaplan‐Meier estimated TTL was 29 weeks for patients with low CD11b, compared to 160 weeks for patients with high CD11b (P < 0.05). Patients who met both criteria, la+ and low CD11b, represented the poorest prognostic subgroup, with median TTL of 13 weeks compared with 88 weeks for the others (P = 0.017), la and CD11b patterns were not specific for MDS subtype, and their expression did not correlate with blast count. These data suggest that MDS patients whose bone marrow cells demonstrate high la (HLA‐DR) and low CD11b (Mo1) expression represent a poor prognostic subgroup with short TTL. These patients may be candidates for early aggressive or investigational treatment.
Phase II study of etoposide, ifosfamide, and mitoxantrone for the treatment of resistant adult acute lymphoblastic leukemia
Authors: Schiller Gary , Lee Myung , Territo Mary , Gajewski James , Nimer Stephen 
Published: February 2007

AbstractAlthough combination chemotherapy induces complete remission in 60–90% of adults with acute lymphoblastic leukemia, only 20–45% of patients remain in continued remission 5 years from diagnosis. For patients with a short first remission, multiple relapses, or patients with disease refractory to initial induction chemotherapy, few salvage treatments are successful. To improve the results of salvage therapy we studied the efficacy and toxicity of a combination of etoposide (100 mg/m2 IV qd × 5), ifosfamide (1.5 g/m2/d × 5), and mitoxantrone (8 mg/m2/d IV × 3) in 11 adult patients with relapsed or refractory ALL. The median follow‐up of all patients completing therapy is 208 days (30–484+ days). Eight of 11 (73%; 95% confidence interval 45–92%) achieved a complete remission, two patients failed to enter remission, and one patient died of multiorgan system failure shortly after receiving therapy. Median DFS is 96 days and median survival from remission is 234 days. Five patients who achieved CR subsequently relapsed with a median time to relapse of 80 days (50–151 days). Median time to granulocyte > .5 × 109/L was 28 days (21–46 days) and the median time to platelet recovery > 20 × 109/L was 24 days (21–39 days). Although gastrointestinal toxicity was common, no patient developed severe cardiac, hepatic, pulmonary, or neurologic complications. These results demonstrate that the combination of etoposide, ifosfamide, and mitoxantrone can be used as an effective salvage therapy for patients with resistant ALL.
α‐interferon therapy for severe chronic idiopathic thrombocytopenic purpura in children
Authors: Cohn Richard J. , Schwyzer Rosemarie , Poole Janet E. , Hesseling Peter B. , Naidoo Joan , Van Heerden Corrieta 
Published: February 2007

AbstractWe report the use of α2b‐interferon in 15 children with symptomatic, chronic idiopathic thrombocytopenic purpura, who did not respond to standard therapy. The platelet count increased in nine children during therapy. In six children the increase lasted less than 6 weeks. An increased platelet count was seen when a higher dose was used in two of four initial nonresponders. One responder who relapsed had an identical response when retreated with the same dose. No significant side effects were documented. Further studies are required to establish the optimal dose and administration schedule of α2b‐interferon for use in children with chronic idiopathic thrombocytopenic purpura.
Effects of continuous high dose rhgm‐csf infusion on human monocyte activity
Authors: Perkins Raymond C. , Vadhan‐Raj Saroj , Scheule Ronald K. , Hamilton Raymond , Holian Andrij 
Published: February 2007

AbstractIn this study we describe the time‐dependent effects of a high dose (750 (μg/ml/24 hr) continuous infusion of recombinant human granulocyte‐macrophage colony‐stimulating factor (rhGM‐CSF) on monocyte number, cytokine release, and superoxide anion production. Blood was taken from patients prior to rhGM‐CSF infusion (day 0), and on days 1,7, and 14 of infusion. The mean concentration of monocytes per ml of blood increased progressively from 4.3 × 105 on day 0 to 21 × 105 on day 14 of infusion. There was no significant change in the basal release of tumor necrosis factor α (TNF‐α) or interleukin 1β (IL‐1β) induced by rhGM‐CSF. However, the lipopolysaccharide (LPS)‐stimulated release of TNF‐α by monocytes increased significantly on day 1 of infusion, and by day 14 had increased 8‐fold. IL‐1β release from LPS‐stimulated monocytes increased slightly by day 7, and by almost 10‐fold by day 14 of infusion. When maximally stimulated with phorbol dibutyrate, the monocytes demonstrated an increased (although not significant) capacity to produce superoxide anion on days 7 and 14 of infusion. No change in basal superoxide anion production was seen at any day of infusion. These GM‐CSF‐induced changes in stimulated cytokine and superoxide anion release could not be reproduced by treating monocytes with rhGM‐CSF in vitro. In summary, a two week, high dose infusion of rhGM‐CSF resulted in increases in circulating monocyte concentration, and in the stimulated release of TNF‐α and IL‐1β, and superoxide anion production from these monocytes. These primed monocytes could enhance the ability of neutropenic patients to fight infection.
Successful alpha‐2b‐interferon therapy for chronic neutrophilic leukemia
Authors: Meyer S. , Feremans W. , Cantiniaux B. , Capel P. , Huygen K. , Dicato M. 
Published: February 2007

AbstractThe authors report two patients with characteristic features of chronic neutrophilic leukemia (CNL) treated with alpha‐2b‐interferon (IFN) (Schering‐Plough). At initiation of therapy, both patients had progressive disease and presented with large tumors. A rapid reduction of the tumor mass and a long‐term stabilisation of the myeloproliferative disorder was obtained (therapy duration 16 and 26 months, respectively, and presently ongoing). In one patient, the dose of IFN could be significantly reduced during maintenance without relapse. Neither presented infectious or hemorrhagic complications under therapy. Alpha‐2b‐interferon is active and safe in CNL, even pretreated and progressive. It can also correct the neutrophil and natural killer functional defects frequently observed in CNL.
Hb Saint Nazaire (β103[G5]Phe→Ile): A new example of polycythemia due to a hemoglobin variant with increased oxygen affinity
Authors: Wajcman H. , Kister J. , M'Rad A. , Promé D. , Milpied N. , Rapp M. J. , Harousseau J. L. , Riou J. , Bardakdjian J. , Galacteros F. 
Published: February 2007

AbstractHb Saint Nazaire [β103 (G5) Phe→lle] was found in four apparently unrelated French families. The five patients carrying this hemoglobin have been detected because of a moderate erythrocytosis. The structural abnormality of Hb Saint Nazaire concerns the same residue as in Hb Heathrow [β103 (G5) Phe→Leu)]. A comparative functional study between these two variants showed that the increase in oxygen affinity is much lower in Hb Saint Nazaire than in Hb Heathrow. The replacement of phenylalanine G5, which is localized within the heme pocket, by a leucine abolishes several contacts between the heme and the globin and leads to an environment of the heme having some similarities with that observed in myoglobin. In contrast, the replacement of G5 by an isoleucine is likely to introduce less structural modifications. © 1993 Wiley‐Liss, Inc.
Cd7, CD34‐positive stem cell leukemia arising in agnogenic myeloid metaplasia
Authors: Tasaka Taizo , Nagai Masami , Murao Satoshi , Yamaguchi Masahiro , Kitanaka Akira , Sasaki Kazunori , Murata Michio , Tanaka Terukazu , Kuwabara Hiroko , Ikeda Kazuma , Takahara Jiro , Irino Shozo 
Published: February 2007

AbstractAgnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder arising from a single hematopoietic cell. Approximately 5% of reported cases of AMM have terminated in leukemic crisis; however, the precise characteristics of the leukemic cells have rarely been reported. We report a case of AMM that occurred in a 42‐year‐old man and was complicated by leukemic transformation. The leukemic cells wer morphologically lymphoblastoid cells with a negative reaction to peroxidase staining, and phenotypically characterized as CD7+, CD34+, HLA‐DR+, CD4−, CD8−, CD10−, CD13−, and CD33−. Southern blot analysis revealed that T cell receptor‐β, γ, and immunoglobulin heavy chain genes in leukemic cells were retained in germ‐line configuration. These observations suggest that leukemic cells in our case involved early hematopoietic stem cells rather than those strictly committed to myeloid or lymphoid precursors. To our knowledge, this is the first report of stem cell leukemia arising in a patient with AMM. © 1993 Wiley‐Liss, Inc.
Presence of serum antibodies to coagulation protein C in patients with systemic lupus erythematosus is not associated with antigenic or functional protein c deficiencies
Authors: Ruiz‐Arguelles Alejandro , Vazquez‐Prado Jose , Deleze Margarita , Perez‐Romano Beatriz , Drenkard Cristina , Alarcon‐Segovia Donato , Ruiz‐Arguelles Guillermo J. 
Published: February 2007

AbstractAntibodies directed to immunopurified coagulation protein C (PC) were investigated in serum samples from 108 patients with systemic lupus erythematosus (SLE) and found in 12 of them. However, their presence was not associated with antigenic or functional deficiencies of PC, which were documented in 6 and 17 patients, respectively. © 1993 Wiley‐Liss, Inc.
Mean platelet volume improves upon the megathrombocyte index but cannot replace the blood film examination in the evaluation of thrombocytopenia
Authors: Vukelja Svetislava J. , Krishnan Jayashree , Diehl Louis F. 
Published: February 2007

AbstractThe measurement of the number of platelets larger than 3 microns (megathrombocyte index) is the first element in the evaluation of thrombocytopenia. This is currently performed by counting the number of large platelets on the peripheral blood film. The MPV (mean platelet volume) is an automated measurement of the platelet volume. This study examines the mean values, correlations, sensitivity, specificity and the receiver operating characteristic curve (comparison of two tests) to determine which of these tests better separates the production state. For increased vs. decreased production, the MPV was 10.0 + 1.9 fL and 8.0 + 1.5 fL (P < .0001) respectively and the megathrombocyte index (MEGA) was 19.0 + 17.6% and 11.5 + 14.9% (P < .007) respectively. The correlation with the state of production was better for MPV (R = .47) than for MEGA (R = .20). For the MPV a sensitivity of 80% occurred with the MPV ⩾ 8.4 fL with a specificity of 71%. For a MEGA ⩾ 6%, the sensitivity was 80% but the specificity was 43%. For any MPV the sensitivity and specificity were better than for any MEGA. The Receiver Operating Characteristic Curve demonstrated that the MPV is a better test than the MEGA for separating the production into increased and decreased states. The MPV is a better test than the MEGA and will add to, but not replace, examination of the peripheral blood film in the diagnosis of thrombocytopenia. © 1993 Wiley‐Liss, Inc.
Increased levels of vascular endothelial cell markers in thrombotic thrombocytopenic purpura
Authors: Wada Hideo , Kaneko Toshihiro , Ohiwa Michiaki , Tanigawa Motoaki , Hayashi Tatsuya , Tamaki Shigehisa , Minami Nobuyuki , Deguchi Katsumi , Suzuki Koji , Nakano Takeshi , Shirakawa Shigeru 
Published: February 2007

AbstractWe found that patients with thrombotic thrombocytopenic purpura (TTP) have significantly elevated plasma thrombin antithrombin III complex (TAT) and FDP‐D‐dimer levels, while the plasmin‐α2 plasmin inhibitor complex (PIC) level was only slightly increased. The tissue‐type plasminogen activator (t‐PA) level was increased, but it was well correlated with the plasminogen activator inhibitor‐1 (PAl‐l) level. These findings suggest that hypercoagulable and hypofibrinolytic states coexist in these patients, in contrast to patients with disseminated intravascular coagulation, who exhibit coexisting hypercoagulable and hyperfibrinolytic states. Levels of vascular endothelial cell markers, such as PAl‐l, thrombomodulin (TM), and t‐PA, were increased at the onset of TTP, but the level of von Willebrand factor (vWF) antigen was not increased. The outcome in TTP patients was correlated with plasma t‐PA and TM levels but not with TAT or PIC. These results suggest that vascular endothelial cell markers, such as TM and t‐PA, are released from injured or stimulated endothelial cells, reflecting the degree of vascular endothelial damage, and that the main factor in the pathogenesis of TTP is vascular endothelial cell injury. © 1993 Wiley‐Liss, Inc.
Flow cytometric analysis of changes in cytoskeletal proteins during platelet destruction and activation using a monoclonal antibody against platelet myosin
Authors: Yamaguchi Kazuyuki , Nomura Shosaku , Kido Hirofumi , Kawakatsu Toshihiro , Fukuroi Tsutomu , Suzuki Masahiro , Hamamoto Kenjiro , Yanabu Mutsumasa , Kokawa Terutoshi , Yasunaga Kojiro 
Published: February 2007

AbstractWe developed a new monoclonal antibody directed against platelet myosin (NNKY6‐19). Using this antibody, we analyzed platelet cytoskeletal changes related to stimulation with thrombin and to long‐term storage. Immunoelectron microscopy showed increased binding of NNKY6‐19 to pseudopods and the open canalicular system during treatment with thrombin (0.1 U/ml) and during storage for 7 days. Flow cytometry also showed increased binding to platelets by NNKY6‐19 and an antiactin monoclonal antibody during storage. The binding of NNKY6‐19 showed an increase greater than that with the antiactin antibody after storage of platelets for 7 days and after thrombin treatment. These findings indicated that the increased binding of NNKY6‐19 had some relationship to changes in intracellular myosin and platelet morphology. Thus use of NNKY6‐19 allowed analysis of subtle changes related to platelet activation, which differed from those detected by antibodies against platelet glycoproteins or by the antiactin antibody. This antibody appears to provide a simple method for studying changes in platelet cytoskeletal and surface proteins. © 1993 Wiley‐Liss, Inc.
Synergistic effect of heme and IL‐1 on hematopoietic stromal regeneration after radiation
Authors: Lutton John D. , Chertkov Joseph L. , Jiang Shanlong , Kappas Attallah , Levere Richard D. , Abraham Nader G. 
Published: February 2007

AbstractResults from this study show that a combination of heme and interleukin‐1 (IL‐1) treatment resulted in the most improved recovery of hematopoietic‐stromal regeneration after sublethal irradiation. Less pronounced effects were obtained when heme or IL‐1 were given singly. Sublethal irradiation of mice produced an initial (as early as day 1) intense depression of the hematopoietic system as evidenced by leukopenia. In vivo treatment of animals with heme in combination with IL‐1, accelerated hematopoietic and stromal regeneration as determined by hematopoietic spleen colony forming unit assay (CFU‐S), erythroid (BFU‐E), myeloid (CFU‐GM) clonal cultures, long‐term bone marrow cultures (LTBMC), and the ability to regenerate hematopoiesis by ectopic (renal) stromal hemopoietic transplantation. Sixteen days after irradiation, leukocyte levels in heme and IL‐1 treatment groups were higher than non‐treated animals and were near normal values by 27 days. One day after irradiation, the capacity of stromal progenitors to form new bone and hematopoietic cells (ectopic foci) was severaly impaired, but recovered after 2–4 weeks. This recovery process was accelerated in heme and IL‐1‐treated animals. BFU‐E, CFU‐GM, and CFU‐S capacity was also severely impaired in all animals 1–27 days after irradiation. CFU‐S was only 0.15% of control by day 1 and 5% of control by day 16. Treatment with heme or IL‐1 improved recovery by as much as 70% after 27 days of irradiation. A similar but enhanced recovery was seen for BFU‐E and CFU‐GM, with erythroid recovery the best. Total cellularity, adherent cell layer (ACL) formation, and donogenic capacity by LTBMCs (10 weeks) derived from irradiated animals was severely reduced, whereas the hematopoietic capacity by LTBMCs derived from heme‐ and IL‐1‐treated animals had recovery values similar to non‐irradiated controls. These results suggest therapeutic use of heme and IL‐1 after chemotherapy or bone marrow depression may be beneficial. © 1993 Wiley‐Liss, Inc.