bluuebunny/crossref_metadata_2025_split huggingface dataset:
Macrophage‐megakaryocyte interaction in bone marrow after high‐dose intravenous immunoglobulin therapy
Authors: Saito Nagahito , Takemori Nobuo , Hirai Katsuyuki , Onodera Ryuichi , Watanabe Shinji , Namiki Masayoshi 
Published: February 2007

AbstractLocalization of IgG in bone marrow after high‐dose intravenous immunoglobulin therapy (IVIG) was investigated via light and electron microscopy. IgG was incorporated into the cytoplasm of various types of bone marrow cells of all the three lineages, particularly in reticulum cells, fat cells, and megakaryocytes. In addition, both reticulum cells and fat cells showed many elongated cytoplasmic protrusions, which were in contact with the various types of blood cells, especially the megakaryocytes. A filamentous structure was also seen near the point of contact between the cells. Ultrastructural changes of macrophages in bone marrow after IVIG suggest that these cells were activated by IVIG. © 1993 Wiley‐Liss, Inc.
Weak n activity of en(a−) human erythrocyte membranes
Authors: Shinozuka Tatsuo , Miyata Yoshihisa , Ohyama Kunio , Takei Setsuko , Yamakawa Toshio , Kurihara Katsuyoshi , Yanagida Junichi 
Published: February 2007

AbstractThe propositus's erythrocytes with phenotype En(a−), which was found for the first time in a Japanese family, reacted more weakly with anti‐N serum than the ordinary phenotype N erythrocytes. The En(a−) erythrocytes lack the major membrane sialoglycoprotein (glycophorin A) as demonstrated by Bio‐Gel 1.5m gel filtration from active sialoglycoproteins, which were isolated from En(a−) erythrocyte membranes by the method of lithium diiodosalicylate (LIS)‐phenol extraction. It is suggested from observation via enzymelinked immunosorbent assay (ELISA) that N activity is derived from the glycophorin B molecule on En(a−) erythrocyte membranes. © 1993 Wiley‐Liss, Inc.
TNF‐α induces spreading of B‐CLL via the CD11C/CD18 molecule
Authors: Van Kooten Cees , Rensink Irma , Aarden Lucien , Van Oers Rien 
Published: February 2007

AbstractActivation of malignant B cells can lead to extensive morphological changes of these cells. A combination of PMA and TNF‐α can induce adherence of purified B‐CLL cells, which acquire a dendritic cell‐like appearance. This phenomenon that we have termed spreading, is accompanied by upregulation of expression of both β1 and β2 integrin molecules. Spreading was inhibited by the addition of antibodies against CD18 or CD11c. In other B cell malignancies (HCL and NHL), morphological changes could be induced by PMA in the absence of TNF‐α. Culturing in the presence of prednisolone resulted in an inhibition of spreading, most likely mediated via a down regulation of CD18 and CD11c expression. These data indicate that the CD11c/CD18 complex might be important for the adhesive properties of B cells. © 1993 Wiley‐Liss, Inc.
Peripheral neuropathy in IgM monoclonal gammopathy and wäldenstrom's macroglobulinemia: A frequent complication in elderly males with low MAG‐reactive serum monoclonal component
Authors: Baldini Luca , Nobile‐Orazio Edoardo , Guffanti Andrea , Barbieri Sergio , Carpo Marinella , Cro Lilla , Cesana Bruno , Damilano Isabella , Maiolo Anna T. 
Published: February 2007

AbstractPeripheral neuropathy (PN) is a frequent complication during primary macroglobulinemia (PM), whose immunological genesis has been suggested by various authors. This study involved 65 PM patients (44 men and 21 women aged 35‐78), diagnostlcally divided into MGUS (31 cases), and indolent (IWM, 24 cases) or symptomatic (WM, 10 cases) Waldenstrom macroglobulinemia groups. All patients underwent neurological examination, including electrodiagnostic evaluation and the determination of the serum titre of antimyelin‐associated glycoprotein (MAG). An evaluation was made of the prevalence of PN and its correlation with a series of hematological variables.The prevalence of PN was 31.6%: of those with PN, 73.1% manifested both clinical and electrophysiological signs of PN, primarily of the demyelinating type. Significant correlations emerged between the presence of PN and sex (M vs. F P = 0.0001), advanced age (P = 0.049), low MC levels (P = 0.025), high anti‐MAG titres (P = 0.001) and high Hb levels (P = 0.001). No significant correlation with the diagnostic definition of PM was found, although the majority of cases with (particularly demyelinating) PN were MGUS or IWM. At multivariate analysis, the presence of PN significantly correlated with sex (P = 0.0001), age (P = 0.019), and anti‐MAG titre (P = 0.001).Ten of the 26 PN cases showed no MAG reactivity. Significant correlations between PN and low serum MC levels/high MAG reactivity support the hypothesis of the antibody‐mediated origin of many PN, and that the presence of PN depends on the characteristics the proliferating pathological B clone, rather than on the tumor burden of the form of macroglobulinemia. Clinically, our data reconfirm the frequency of PN during PM and indicate simple clinicohematological variables useful for identifying patients at high neuropathic risk. © 1994 Wiley‐Liss, Inc.
Quantities of αQ chain variants in heterozygotes with and without a concomitant β‐thalassemia trait
Authors: Qin W.‐B. , Baysal E. , Wong K. F. , Molchanova T. P. , Pobedimskaya D. D. , Sharma S. , Wilson J. B. , Huisman T. H. J. 
Published: February 2007

AbstractWe have analyzed the quantities of αx chain‐containing hemoglobins (αxβ2 and α2xβ2,) in 14 heterozygotes for Hb Q‐India [α64(E13)Asp→His] or Hb Q‐Thalland [α74(EF3)Asp→Hls]; both amino acid replacements are the result of mutations in the α1‐globin gene. Five of these persons (three with Hb Q‐India and two with Hb Q‐Thailand) had an additional β°‐thalassemia heterozygoslty. The average quantities for Hb Q + Hb Q2 in the four groups were 17.2% (Q/αα; βA/βA), 9.5% (ααQ/αα; βA/β°Th), 26.8% (‐αQ/αα;βA/βA), and 16.95% (‐αQ/αα; βA/β°Th). These variations can best be explained by a posttranslational control mechanism; an imbalance in the αA, αQ, and βA chain ratio will favor the αzQβz formation when an α‐thalassemia is present and will reduce its formation in the presence of a β‐thalassemia heterozygosity. © 1994 Wiley‐Liss, Inc.
Hemophilic pseudotumor: Multicenter experience over 25‐year period
Authors: Magallón M. , Monteagudo J. , Altisent C. , Ibáñez A. , Rodriguez‐Pérez A. , Riba J. , Tusell J. , Martin‐Villar J. 
Published: February 2007

AbstractPseudotumor is an infrequent manifestation of bleeding diathesis seen in hemophiliacs, the management of which is still controversial. To ascertain the effectiveness of the main therapies of choice, we have reviewed 1,831 patients affected by hemophillas A (1,108) and B (172), von Willebrand's disease (329), and other miscellaneous coagulopathles (222) diagnosed between 1965 and 1990 in a multicentric, retrospective study. Pseudotumor was proven in 21 patients. Replacement therapy was given in 15 cases as the first therapeutic approach, with complete success attained in only two, whereas surgery, which was carried out in 14 patients, completely resolved the process in eight (P = 0.017). Patients over 40 years of age demonstrated worse prognosis than younger patients (P = 0.02), but no other clinical parameters were shown to have influenced the patients' evolution. Surgical management was the most effective treatment for pseudotumor, although more conservative therapies cannot be overlooked in selected cases. © 1994 Wiley‐Liss, Inc.
Von willebrand factor availability in platelet concentrates stored for 5 days
Authors: Cesar Jesús M. , Garcia‐Avello Angel , Monteagudo Juán , Espinosa José I. , Lodos José C. , Castillo Ricardo , Navarro José L. 
Published: February 2007

AbstractVon Willebrand factor (vWF) availability was assessed in platelet concentrates (PCs). After 5 days of storage, 82 ± 9% of basal levels of ristocetin cofactor activity (vWF:RCo) remained in PCs. vWF antigen (vWF:Ag) increased up to 166 ± 38% (P < 0.05) In the same period. Autoradiograph pattern of vW:Ag showed an increase in low molecular weight multimers, and fast migrating multimeric forms were visualized by crossed immunoeiectrophoresis on day 5. Studies carried out in platelet free plasma stored as PCs showed similar changes in vWF:RCo but increments in vWF:Ag were not detected. These data indicate that PCs maintain vWF:RCo levels of clinical value even after 5 days of storage and suggest that vWF comes out from platelets to plasma during storage. © 1994 Wiley‐Liss, Inc.
Changes in longevity and causes of death among persons with hemophilia A
Authors: Chorba Terence L. , Holman Robert C. , Strine Tara W. , Clarke Matthew J. , Evatt Bruce L. 
Published: February 2007

AbstractTo examine recent changes in longevity and the causes of death among persons with hemophilia A, we evaluated death certificate data for persons who died in the United States from 1968 through 1989 and had hemophilia A or congenital Factor VIII disorder (ICD code 286.0) listed on the death certificate as one of the multiple causes of death. Multiple‐cause‐of‐death mortality data for the United States from 1968 to 1989 were examined to compare death rates by year, focusing on death rates and causes of death for 1979‐1981, 1983‐1985, and 1987‐1989. Gender, age group, race, geographic region, and median age at death of persons with hemophilia A and human immunodeficiency virus (HIV)‐related disease listed as a cause of death were compared with those with hemophilia A without HIV‐related disease. From 1968 through 1989, 2,792 hemophilia A deaths were reported. The death rate increased from 0.5 to 1.3 per 1,000,000 persons. From 1979‐1981 through 1987‐1989, mortality increased in all age groups above 9 years of age and age at death shifted markedly to lower ages. Median age at death decreased from 57 years in 1979‐1981 to 40 years in 1987‐1989. The percentage of deaths due to hemorrhage or diseases of the circulatory system decreased markedly as the result of the increase in deaths associated with HIV infection or infections other than HIV infection. Spread of HIV‐1 infection in persons with hemophilia A has disrupted the reduction in mortality seen with factor replacement therapy, implementation of home care, and use of comprehensive hemophilia treatment centers. It is hoped that advances in the care of HIV‐infected persons will improve survival in the hemophilia community. © 1994 Wiley‐Liss, Inc.
Varying populations of CD59‐negative, partly positive, and normally positive blood cells in different cell lineages in peripheral blood of paroxysmal nocturnal hemoglobinuria patients
Authors: Fujioka Seitoku , Yamada Teruo 
Published: February 2007

AbstractCD59‐antigen expression on the surface membranes of erythrocytes, granulocytes, monocytes, lymphocytes, and platelets was determined by flow cytometry in 34 healthy controls and 17 patients with paroxysmal nocturnal hemoglobinuria (PNH). In all PNH patients, CD59‐negative erythrocytes accounted for > 10% of the total erythrocyte population. Two erythrocyte populations (CD59‐negative and normally positlve or CD59‐negative and partly positive), three populations (CD59‐negative, partly positive, and normally positive), and one population (CD59‐negative) were demonstrated in ten, six, and one patients, respectively. However, CD59‐negative granulocytes did not account for > 10% of the total granulocytes in two patients, and one of them had only a CD59 normally positive granulocyte population. A particular granulocyte population extended over both CD59‐negative and partly positive areas was shown in two patients. Two populations (CD59‐negative and normally positive) and one population (CD59‐negatlve) were demonstrated in monocytes and lymphocytes. CD59‐negative lymphocytes accounted for >50% of the total lymphocytes in only two patients. Three patients had a CD59 normally positive lymphocyte population. Percentages of CD59‐positive platelet population in normal controls were widely various. Therefore, it was usually difficult to discriminate between PNH‐affected and normal platelets. Thus, the flow cytometric profiles of CD59‐antigen expression varied not only between PNH patients but between cell lineages. The present results and our prior study indicate that CD59 flow cytometry using erythrocytes and granulocytes is most suitable for diagnosing PNH. © 1994 Wiley‐Liss, Inc.
Topical thrombin and acquired coagulation factor inhibitors: Clinical spectrum and laboratory diagnosis
Authors: Ortel Thomas L. , Charles Linda A. , Keller Frank G. , Marcom P. Kelly , Oldham H. Newland , Kane William H. , Macik B. Gail 
Published: February 2007

AbstractTopical bovine thrombin preparations are used extensively in cardiovascular, neurosurgical, and otolaryngologic procedures. Patients who are treated with these topical thrombin preparations may develop antibodies to bovine coagulation factors that may cross‐react with the endogenous human clotting proteins. We have identified four patients with acquired factor inhibitors following exposure to topical thrombin at Duke University Medical Center and summarize these cases in addition to 13 patients previously reported in the literature. In most cases, the inhibitor developed following a second (or subsequent) exposure to topical thrombin. The clinical course was extremely variable, ranging from totally asymptomatic to life‐threatening hemorrhage. The most consistent laboratory abnormality was a prolonged bovine thrombin clotting time, which corrected, at least partially, when human thrombin was substituted for bovine thrombin. Some of these patients also developed factor V inhibitors with prolonged prothrombin and activated partial thromboplastin times. Although these patients have prolonged clotting times, they should not be considered “autoanticoagulated,” since thromboembolic complications can still occur. Therapeutic intervention is largely empirical and depends on the clinical manifestations of the individual patient. © 1994 Wiley‐Liss, Inc.
Prevalence of abnormal iron studies in heterozygotes for hereditary hemochromatosis: An analysis of 255 heterozygotes
Authors: Adams Paul C. 
Published: February 2007

AbstractIron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 ± 10.2 μg/liter; mean ± standard error) was greater in heterozygotes than in normal subjects (87 ± 8.5 μg/liter; P < .05, Mann Whitney test). Transferrin saturation was greater in heterozygotes (38% ± 0.88%) than in normal patients (29% ± 1.1%; P < .0001). Mean hepatic iron concentration was 54 ± 6 μmol/g (n = 17), and the hepatic iron index was <2 in these patients. Most heterozygotes for hemochromatosis have a normal serum ferritin and transferrin saturation. Heterorygotes with minor elevations in serum ferritin or transferrin saturation do not have significant iron overload as assessed by hepatic iron concentration. © 1994 Wiley‐Liss, Inc.
Skin manifestation of agnogenic myeloid metaplasia
Authors: Loewy Gabriel , Mathew Anna , Distenfeld Ariel 
Published: February 2007

AbstractAgnogenic myeloid metaplasia (AMN) with myelofibrosis is a clonal malignancy of the hematopoletic stem cell. The disease is characterized by increased endothelial cell and fibroblast proliferation, resulting in increased deposition of fibronectin, laminin, and collagen in the bone marrow. In advanced disease, extramedullary hematopoiesis (EMH) is invariably seen in the spleen and liver. The lymph nodes are also frequent sites of EMH, but other organs, especially the kidneys, arenals, lungs, pleura, ovaries, gastrointestinal tract, and dura, may also be involved. Skin manifestations are rare. They may present in several ways: erythematous plaques, nodules, diffuse or papular erythema, ulcers, and bullae. Histopathology of these lesions reveals cells from one or more myeloid lineage in the dermis, erythrold, or megakaryocytic series alone or in combination. In rare cases, all three cell lines are demonstrated. © 1994 Wiley‐Liss, Inc.
Treatment of refractory undifferentiated acute myelogenous leukemia with all‐trans‐retinoic acid
Authors: Griggs Jennifer J. , Henley Susan E. , Rowe Jacob M. 
Published: February 2007

AbstractA patient is described with undifferentiated acute myeloblastic leukemia refractory to two courses of daunorubicin and cytosine arabinoside. Because some the myeloblasts developed morphologic features of promyelocytes, the patient was treated with all‐trans‐retinoic acid (ATRA) in an attempt to promote maturation. Cytogenetic studies and sensitive molecular analysis did not reveal any abnormality classically associated with acute promyelocytic leukemia. Serial bone marrow biopsies demonstrated myeloid maturation, and the patient uneventfully went into a sustained complete remission. A review of the literature confirms this to be an apparently hitherto undescribed response to ATRA that may have therapeutic implications in similar patients. © 1994 Wiley‐Liss, Inc.
Molecular abnormality of a unique japanese glucose‐6‐phosphate dehydrogenase variant (G6PD Kobe) with a greatly increased affinity for galactose‐6‐phosphate
Authors: Hirono Akira , Nakayama Shiro , Fujii Hisaichi , Miwa Shiro 
Published: February 2007

AbstractSystematic molecular analysis of a Japanese class 1 glucose‐6‐phosphate dehydrogenase (GGPD) variant (G6PD Kobe) cONA revealed a unique nucleotide substitution (1318 C to T) in exon 11, which predicts a substitution of leucine for phenylalanine at residue 440. This substitution is located in a region surrounding the putative structural NADP‐binding domain. The markedly abnormal kinetics of glucose‐6‐phosphate (GGP) of GGPD Kobe suggest the interaction between both NADP and G6P binding sites. © 1994 Wiley‐Liss, Inc.
A possible survival advantage in adults with acute leukemia receiving ABO‐identical platelet transfusions
Authors: Heal Joanna Mary , Kenmotsu Naomi , Rowe Jacob M. , Blumberg Neil 
Published: February 2007

AbstractIn a small cohort of leukemic patients participating in a randomized trial of ABO‐identical vs. ABO‐unmatched platelet transfusions, a statistically significant decrease in remission duration and increase in mortality were observed in patients receiving ABO‐unmatched platelet transfusions. This difference was unexplained by previously described prognostic factors for remission duration and survival. Further study of this association is warranted. © 1994 Wiley‐Liss, Inc.
von Willebrand's disease: Use of collagen binding assay provides potential improvement to laboratory monitoring of desmopressin (DDAVP) therapy
Authors: Favaloro Emmanuel J. , Dean Mark , Grispo Linda , Exner Tom , Koutts Jerry 
Published: February 2007

AbstractThis report describes studies investigating the use of a collagen binding assay to improve the laboratory monitoring of desmopressin (DDAVP) therapy in patients with von Willebrand's disease (vWD). We evaluated the response of seven patients with vWD (four type I, three type IIA) to DDAVP, administered using a standard protocol, by assessing levels of von Willebrand factor (vWF) and factor VIII, as well as performing skin bleeding times (SBT) prior to, and at sequential time points following, DDAVP administration. The study employed the following assays: von Willebrand factor antigen assay (vWF:Ag; determined by ELISA); a novel functionally based collagen binding assay (CBA; determined by ELISA); ristocetin cofactor assay (RCof; determined by platelet aggregometry); von Willebrand factor muitimer analysis (using SDS‐agarose gels); factor VIII coagulant (FVIIIC; determined by clotting assay); and factor VIII antigen (FVIIICAG; determined by ELISA). All patients showed an initial incremental increase in vWF/FVIII levels using all assays above, and some showed some correction in SBT. Although the absolute levels of vWF/FVIII antigen or activity varied between patients, the CBA was found to provide consistently the greatest proportional incremental increases (i.e., ‐fold) compared to baseline (pre‐ DDAVP) levels. Accordingly, we consistently observed an increase in the CBA to vWF:Ag ratio for all patients evaluated. This supplements previous findings that have suggested a unique ability of our CBA procedure to bind preferentially to higher molecular weight (i.e., more functionaily active) forms of vWF. We therefore propose that the use of the above test combination (e.g., vWF:Ag plus CBA) may provide the basis for more accurate estimation of a patient's functional responsiveness to DDAVP therapy in future studies. © 1994 Wiley‐Liss, Inc.
Stability of factor VIII concentrates after reconstitution
Authors: Schulman Sam , Gitel Sanford , Martinowitz Uri 
Published: February 2007

AbstractAdjusted‐dose continuous infusion of factor VIII (FVIII) has recently been shown to reduce the doses of the factor in patients undergoing surgery by 50‐75%. The main limitation of this method has been the instability of factor concentrates. All manufacturers are recommending infusion of the concentrate within hours after reconstitution. We studied the stability of 15 different lyophillzed F VIII products. Reconstituted samples were stored for periods of 4, 24, and 72 hr and 1, 2, 3, and 4 weeks at temperatures of WC, 20‐23°C, and 37°C in their original glass containers and in plastic tubes and then frozen. Assays were performed in duplicate, using a one‐stage clotting method and a chromoge nlc assay for F VIII, wlth all samples from a single concentrate In the same run. Activation of the coagulation factor occurred In some concentrates, more often at 44°C than at 20‐23°C or 3PC. The stability of all products was substantially better than that declared by the manufacturers. Several concentrates maintained factor activities above 80% of baseline for the entlre period of 4 weeks at 44°C or at 20‐23°C. The results demonstrate that many of the F VIII concentrates may be used for continuous infusion. © 1994 Wiley‐Liss, Inc.
α‐Interferon treatment for idiopathic hypereosinophilic syndrome
Authors: Bockenstedt Paula L. , Santinga John T. , Bolling Steven F. 
Published: February 2007

AbstractIdiopathic hypereosinophilic syndrome (HES) is a poorly understood disorder characterized by a markedly elevated peripheral blood eosinophil count in the absence of known associated causes of hypereosinophilia. Idiopathic hypereosinophilic syndrome is associated with eosinophil‐induced organ damage, including endomyocardial and pulmonary fibrosis, stroke, and gastrointestinal disease. Treatment of idiopathic HES is centered on the reduction of peripheral circulating eosinophils in an effort to diminish tissue infiltration and destruction. Multiple cytotoxic agents have been tried, with variable resutts. Prednisone and hydroxyurea have remained the therapies of choice in long term treatment of idiopathic HES. We report here the successful 2 year treatment of aggressive idiopathic HES, refractory to hyroxyurea and prednisone, with α‐interferon. © 1994 Wiley‐Liss, Inc.
Hereditary hemochromatosis and red cell aplasia
Authors: Adams Paul C. 
Published: February 2007

AbstractA 63‐year‐old white man with myasthenia gravis and red cell aplasia was found to have hepatic iron overload. The differential diagnosis included hereditary hemochromatosis and secondary iron overload related to red cell aplasia. Family studies of siblings, including HLA typing, initially suggested a diagnosis of secondary iron overload. The investigation of a remote sibling led to the final diagnosis of hereditary hemochromatosis. The case illustrates the importance of complete family investigations in the differentiation between hereditary hemochromatosis and secondary iron overload and the potential hazard of using HLA typing as an indirect genetic test for hereditary hemochromatosis. © 1994 Wiley‐Liss, Inc.