bluuebunny/crossref_metadata_2025_split huggingface dataset:
Irreversibly sickled erythrocytes in sickle cell anemia: A quantitative reappraisal
Authors: Rodgers Griffin P. , Noguchi Constance Tom , Schechter Alan N. 
Published: February 2007

AbstractIrreversibly sickled cells (ISCs), considered by some to be of major pathophysiologic significance, have been reported to comprise between 5–50% of the total red cell population in patients with homozygous sickle cell anemia. Since the deformation of erythrocytes containing sickle hemoglobin is highly dependent on the concentration of hemoglobin in the deoxy conformation, any method established to enumerate the true ISC count requires the hemoglobin to be in the full oxy or liganded conformation. Because the oxygen dissociation curve for sickle erythrocytes is significantly shifted to the right, extremely high partial pressures of oxygen are required to approach full saturation. On the other hand, fully liganding the hemoglobin in the oxy conformation with carbon monoxide (CO) can be readily accomplished. We found that there is a significant reduction in the average number of sickled forms in the peripheral blood of sickle cell anemia patients after incubation in CO (to a value of 6.5 ± 3.5%) when compared to conventional methods for ISC preparations. These results suggest that fully liganded erythrocytes should be used in quantitating ISCs in studies of the pathophysiology of this disease, especially since ISCs are likely to affect rheology differently from reversibly sickling cells.
Lymphocyte phenotype and function in chronically transfused children with sickle cell disease
Authors: Wang Winfred , Herrod Henry , Presbury Gerald , Wilimas Judith 
Published: February 2007

AbstractImmunologic studies were performed on mononuclear cells from ten chronically transfused children with sickle cell disease, and the results were compared with those from five other groups: 21 sickle cell patients who were not receiving regular transfusions, 6 chronically transfused children with other forms of refractory anemia, 10 previously splenectomized children, 5 boys with hemophilia A, and 27 normal adult controls. The helper:suppressor T cell ratios (T4:T8) in all groups except hemophiliacs were normal, were unrelated to the number of units transfused, and were not suggestive of findings reported for patients with the acquired immune deficiency syndrome (AIDS) or groups at risk for the syndrome. Percentages of T3, T4, and T8 cells were low in sickle cell and splenectomized patients, but not in chronically transfused patients with other anemias. Serum IgG was frequently elevated, and IgG synthesis in vitro was increased relative to IgM synthesis in sickle cell patients. Coculture experiments indicated that such findings may stem from a selective increase in IgG synthesis by B cells. Thus, transfused sickle cell patients have a particular pattern of immunologic abnormalities that is distinct from that seen in AIDS.
Altered spectrin association and membrane fragility without abnormal spectrin heat sensitivity in a case of congenital hemolytic anemia
Authors: Ravindranath Yaddanapudi , Johnson Robert M. 
Published: February 2007

AbstractIn hereditary pyropoikilocytosis (HPP) and one type of hereditary elliptocytosis (HE), spectrin self‐association is abnormal [5,7]. Spectrin extracted from normal erythro‐cyte membranes at 0°C is nearly all tetrameric, while in HPP and HE (type 1) a substantial amount of the extracted spectrin is dimeric. Abnormal reassociation of spectrin dimers to tetramers can also be demonstrated. We here report the case of a family in which the child has moderately severe hemolysis, with extreme microcytosis and poikilocytosis. The spectrin extracted at 0°C was predominately dimer. Parents had levels of dimer intermediate between patient and control values. The temperature dependence was normal for (a) erythrocyte fragmentation; (b) spectrin extractability; and (c) circular dichroism of purified spectrin. Neither the patient nor either parent had elliptocytic red cells as judged from smears and scanning electron microscopy. The presence of substantial amounts of dimeric spectrin in the parents is consistent with a model in which each parent is heterozygous for a different nonassociating mutant spectrin, while the child has inherited a nonassociating molecule from each parent. In each individual, the degree of mechanical stability of the erythrocyte membrane, determined by ektacytometry, was proportional to the amount of tetramer found in the membrane. The description of this case is consistent with either HPP or a form of homozygous HE which is asymptomatic in the carrier state.
Granulopoiesis in patients with congenital neutropenia
Authors: Kawaguchi Yoshinori , Kobayashi Masao , Tanabe Akio , Hara Michimaru , Nishi Yoshikazu , Usui Tomofusa , Nagai Shinya , Nishibayashi Yoh‐Hei , Nagao Kenji , Yokoro Kenjiro 
Published: February 2007

AbstractGranulopoiesis was investigated in five patients with congenital neutropenia (CN) (one Kostmann type, four benign forms). In semisolid agar culture, the marrow cells of all five patients produced normal numbers of CFU‐c (colony‐forming unit‐culture). The size and classification of colonies were normal. In suspension culture in vitro with exogenous colony‐stimulating factor (CSF) generated from omental‐conditioned medium (OMCM), the myeloid precursors of all patients could proliferate and differentiate into normal polymorphonuclear neutrophils (PMNs). But in the absence of exogenous CSF, myeloid precursors of the patient with Kostmann‐type CN did not proliferate or differentiate into PMNs at all. In the four patients with benign neutropenia, however, PMNs were found even without exogenous CSF similar to normal individuals. These results suggest that patients with CN may have normal granulopoietic stem cells with normal proliferative and differentiating capacity in response to exogenous CSF.When a small amount of normal human serum was added to normal marrow cultures stimulated by exogenous CSF, the colony growth increased in a superadditive manner. The enhancing activity of serum from neutropenic patients differed from that of normal serum. Especially, the addition of serum from the patient with Kostmann type CN to normal marrow cultures did not show this enhancement effect. The sera of patients with benign neutropenia had less enhancement effect than did normal control serum. These findings might be interpreted as showing an imbalance between CSF enhancer and inhibitors in the patients' serum.
T‐cell subpopulations in patients with monoclonal gammopathies: Essential monoclonal gammopathy, multiple myeloma, and Waldenstrom macroglobulinemia
Authors: Miguel J. F. San , Caballero M. D. , Gonzalez M. 
Published: February 2007

AbstractT‐cell subsets defined by monoclonal antibodies (OKT3, OKT4, and OKT8) were analyzed in 117 patients with monoclonal gammopathies—69 multiple myeloma (MM) (30 untreated and 39 treated), 14 Waldenström's macroglobulinaemia (WM), and 34 essential monoclonal gammopathy (EMG) patients. The percentage and absolute numbers of total T‐lymphocytes (E+, OKT3+ cells) were within the normal range in all groups except for the treated MM patients, in which a decrease in the absolute number could be observed. The percentages of OKT4+ cells were significantly lower in MM (35 ± 1.7) than in EMG patients (43 ± 2) and controls (50 ± 2). In contrast, OKT8 cells correspondingly increased in MM (38 ± 1.6) compared with EMG patients (29 ± 1) and controls (27 ± 1). The OKT4/0KT8 ratio was lower in MM than that in EMG patients and controls (p < 0.01) and was shown to be one of the four most significant variables in a linear discriminant analysis used to distinguish between MM and EMG groups. The MM patients in clinical stage III as well as Bence‐Jones myeloma patients showed a more pronounced OKT4/0KT8 imbalance. The treatment did not influence the percent distribution of T‐cell Subpopulations.The patients with WM exhibit an alteration in the distribution of the T‐cell subsets similar to the MM patients with a T4/T8 ratio of 1.1±0.1. This imbalance was more pronounced in WM patients with monoclonal B‐lymphocytes in peripheral blood (leukaemic phase of WM).The functional significance of the altered T‐cell subsets in MM and WM patients remains to be established, though it is probable that such an imbalance plays an important role in regulating these B‐cell proliferations.
Combined factor V/VIII deficiency: A case report including levels of factor V and factor VIII coagulant and antigen as well as protein C inhibitor
Authors: Brown Janet M. , Selik Nancy R. , Voelpel Mary Jo , Mammen Eberhard F. 
Published: February 2007

AbstractComprehensive coagulation studies were performed on members of a family with combined factor V/VIII deficiency. The purpose of these studies was to investigate the hypothesis that combined factor V/VIII deficiency is due to a lack of the inhibitor to activated protein C. The analyses performed included routine APTT and PT, factor V and VIII coagulant activity and antigen levels, van Willebrand factor levels, protein C antigen assay, and both protein C inhibitor activity and antigen levels. Three of the 19 family members studied were found to have a deficiency of both factors V and VIII. These three individuals showed prolonged APTTs and PTs and decreased levels of factor V and factor VIII coagulant activity and antigen. Factor VIII related antigen and ristocetin cofactor (von Willebrand factor) levels were normal. Protein C and both protein C inhibitor activity and antigen levels were also found to be normal. These findings confirm the results of other recent investigators and indicate that the autosomal, inherited combined factor V/VIII deficiency is not due to a protein C inhibitor deficiency. The real defect in this combined deficiency remains to be determined.
Impaired IgM antibody responses to an influenza virus vaccine in adults with sickle cell anemia
Authors: Ballester Oscar F. , Abdallah Jørge M. , Prasad Ananda S. 
Published: February 2007

AbstractType‐specific IgM and IgG antibody responses to a polyvalent influenza vaccine were evaluated in 16 adults with sickle cell anemia, with the use of an enzyme‐linked immunosorbent assay. When compared to healthy controls, 8 out of the 16 patients had decreased or undetectable postvaccination anti‐influenza IgM antibody levels. These patients were found to have significantly lower serum IgM levels and nondetectable splenic tissue (by 99Tc scans), as compared to those with normal IgM responses. Impaired IgM antibody primary immune responses may playa role in the pathogenesis of infectious complications seen in adult patients with sickle cell anemia.
Lymphocyte subpopulation abnormalities in sickle cell anemia: A distinctive pattern from that of AIDS
Authors: Ballester Oscar F. , Abdallah Jorge M. , Prasad Ananda S. 
Published: February 2007

AbstractIn this study, we evaluated lymphocyte subpopulations in 23 adults with SCA. When compared to controls, SCA patients had higher lymphocyte counts with normal numbers of T101+ cells (T‐lymphocytes) and T4 + cells. T8+ cells were significantly increased in SCA patients in comparison to controls (1684 ± 243 vs 980 ± 367, p < .001). This increment was largely dependent on a T101 ‐, T8+ cell population. The SCA patients as a group had significantly decreased T4/T8 ratio (p < .0001). The SCA patients with history of blood transfusions had higher T4+ cells numbers and higher T4/T8 ratio, but no other significant differences from nontransfused patients were noted. Our results are different from those reported for nontransfused children with SCA who showed normal T4/T8 ratio.Thus, a distinct pattern of abnormalities is seen in the lymphocyte subpopulations of adult SCA patients, unrelated to their exposure to blood transfusions.
Acute nonlymphocytic leukemia with eosinophilic differentiation
Authors: Gabbas Attilio G. , Li Chin‐Yang 
Published: February 2007

AbstractFour cases of de novo acute nonlymphocytic leukemia (ANLL) with early eosinophilic differentiation are described. The clinical course did not differ from that of the usual forms of ANLL. Morphologic and cytochemical features that can support this diagnosis are discussed. Particularly, the cyanide‐resistant peroxidase stain appeared to be a specific marker of eosinophilic differentiation. Acute eosinophilic leukemia is a distinct entity, and this unusual subtype of ANLL can be set apart from other forms of ANLL characterized by hypereosinophilia.
Decreased adenosine deaminase (ADA) and 5′nucleotidase (5NT) activity in peripheral blood T cells in hodgkin disease
Authors: Murray James L. , Perez‐Soler Roman , Bywaters Daniel , Hersh Evan M. 
Published: February 2007

AbstractThe purine metabolic enzymes adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and 5′nucleotidase (5NT) play an important role in normal lymphocyte differentiation. Abnormal levels of one or all of these enzymes have been associated with immunodeficiency diseases and lymphoproliferative disorders. ADA, PNP, and 5NT activity was measured in peripheral blood T cells from 24 patients with Hodgkin disease (HD) (12 in complete remission and 12 with active disease) to determine whether an association existed between enzyme abnormalities and the decreased cellular immune function previously described in this disorder. HD patients had a significantly decreased absolute lymphocyte count (1,618 ± 1107/mm3; mean ± SD) compared to controls (2,320 ± 980; p <.001). ADA, PNP, and NT activity was assessed in lymphocyte extracts by measuring the conversion of radiolabeled substrates to products over time. ADA activity expressed as mean ± SEM nanomoles/106 lymphocytes/hr was significantly decreased in T cells from HD patients (84.6 ± 7.5) compared to controls (128 ± 12.3; p < 0.025). Likewise, 5NT was significantly decreased in HD patients (12.7 ± 1.3) compared to controls (24.0 ± 3.6; p <.005). There was not a significant difference in PNP activity between both groups. Low 5NT activity was present irrespective of whether patients had active disease (12.1 ± 1.5) or were in unmaintained complete remission (14.5 ± 2.4). These findings suggest that biochemical abnormalities may be responsible for or related to the persistent abnormalities in T‐cell function noted throughout the clinical course of HD.
Influence of the poly (ADP‐ribose) polymerase inhibitor 3‐aminobenzamide on macrophage and granulocyte differentiation of HL‐60 cells
Authors: Damji Nazim , Khoo Kong Eng , Booker Lynda , Browman George P. 
Published: February 2007

AbstractWe investigated the influence of the poly (ADP‐ribose) polymerase inhibitor 3‐aminobenzamide (ABA) on induction of phenotypic markers of granulocyte differentiation by retinoic acid and markers of macrophage differentiation by TPA in HL‐60 cells. The differentiation of HL‐60 cells towards the granulocyte lineage was assessed by hexose monophosphate shunt activity, proportion of cells capable of reducing NBT dye, and the appearance of recognizable neutrophils and bands. The effect of ABA and retinoic acid on NBT dye reduction and appearance of mature neutrophils and bands was synergistic, whereas the effects of these agents on hexose monophosphate shunt activity were additive. The differentiation inducing capacity of ABA in the presence of retinoic acid was dose‐related. The influence of ABA on TPA‐induced markers of macrophage differentiation was assessed by determining the proportion of adherent cells produced after treatment and by measuring acid phosphatase activity in the adherent cell fraction. In the presence of ABA, the number of cells adhering to plastic declined after day 2 of exposure to TPA, and acid phosphatase activity in adherent cells was inhibited fourfold (p = 0.01). The influence of ABA on the phenotypic markers of granulocyte and macrophage differentiation was detectable at concentrations that were not cytotoxic. The influence of ABA on HL‐60 differentiation is similar to that previously reported for human bone marrow CFU‐GM. Our data suggest that poly (ADP‐ribose) polymerase plays a role in differentiation of HL‐60 cells and that HL‐60 might provide a useful model for evaluating control mechanisms involved in the differentiation of CFU‐GM.
Slow infusion of vincristine in the treatment of idiopathic thrombocytopenic purpura
Authors: Manoharan Arumugam 
Published: February 2007

AbstractTen patients with idiopathic thrombocytopenic purpura (ITP) were treated wtih vincristine (VCR), given as a slow infusion over a 4‐hr period, at weekly intervals for 4 weeks. The VCR therapy achieved a sustained recovery of platelet count in five patients with ITP of 2 weeks to 5 months duration; a transient recovery was observed in four patients with ITP of 6 months to 5 years duration. Therapy had no effect at all in one patient who had ITP of 10 years duration. Vincristine therapy appears to be therapeutically more beneficial when given as a slow infusion and can achieve sustained recovery of platelet count in patients with ITP of less than six months duration.
A naturally occurring, warm‐reactive macroglobulin specific for papain‐treated human platelets: Preliminary characterization
Authors: Gill Joan C. , Carlson Patricia , Kunicki Thomas J. , Aster Richard H. 
Published: February 2007

AbstractSera from 28 of the 113 normal children and adults (25%) studied were found to contain an immunoglobulin capable of causing complement‐dependent lysis of normal platelets treated with small quantities of papain. This factor reacts equally well at 4°C and at 37°C with a determinant induced on platelets from normal subjects by treatment with papain or bromelain, but not by trypsin, chymotrypsin, or neuraminidase. It does not bind to red cells treated with any of these enzymes. The site(s) for which the factor was specific could not be induced on platelets from six patients with type I Glanzmann's thrombasthenia (lacking glycoproteins IIb and IIIa), in contrast to platelets from each of 20 normal donors. Isolation and characterization of the factor has been difficult because of its intolerance to chemical and physical manipulation. In 11 of the 20 individuals studied, however, it was found to have the properties of an IgM immunoglobulin. The factor appears to be different from any previously described, naturally occurring human immunoglobulin. It has not yet been shown to be associated with any disease state, but in the presence of complement, it is capable of causing profound damage to platelets previously subjected to minimal proteolysis, and the possibility that it can provoke platelet destruction in some conditions deserves further study.
Hb bicětre or α2β263(E7)His → pro in a white male: Clinical observations over a period of 25 years
Authors: Miller D. R. , Wilson J. B. , Kutlar A. , Hulsman T. H. J. 
Published: February 2007

AbstractA description is given of clinical observations over a period of 25 years for a 26‐year‐old white male with a heterozygosity for the β‐chain variant, Hb Bicětre, in which β63His is replaced by Pro. The variant was identified through analysis of tryptic peptides from a digest of the βX‐chain isolated by PCMB precipitation and purified by chromatography. Similarity between the clinical histories of the first (French) heterozygote and our patient was most striking, including improvement after splenectomy.
The effect of erythrocyte membrane on the birefringence formation of sickle cell hemoglobin
Authors: Mizukami H. , Bartnicki D. E. , Burke S. , Brewer G. J. , Mizukami I. F. 
Published: February 2007

AbstractThe birefringence formation of sickle cell hemoglobin (HbS) in a thin liquid layer was observed while its environment was deoxygenated at different rates, and the effect of membrane was examined.Under slow rate of deoxygenation at 37°C, at pH 7.4, the birefringence of purified HbS appeared at a concentration higher than 24% and its relative magnitude increased as the concentration was increased. Similarly, the partial presure of oxygen, at which the birefringence formation was evident, increased from 0 to 27 torr as the concentration of HbS was increased from 24 to 28%, but it remained the same above this protein concentration.In all the samples tested relative birefringence was largest at the slow rate of deoxygenation (30 torrO2/min) and the magnitude decreased as the rate of deoxygenation was increased. The samples showed different sensitivity to the rate of deoxygenation. For example, while the total untreated hemolysate made by freeze‐thawing of packed sickle cells was most resistant to the increased rates of deoxygenation, purified HbS was not. Washed open ghosts partially restored the birefringence formation pattern of purified HbS.The results indicate that the inner surface of the membranes of erythrocytes could behave as a template for large HbS polymer formation at relatively higher rates of deoxygenation.
Nuclear bridging of erythroblasts in acquired dyserythropoiesis: An early and transient preleukemic marker
Authors: Bethlenfalvay Nicholas C. , Phaure T. A. J. , Phyliky Robert L. , Bowman Robert P. 
Published: February 2007

AbstractThe clinical, hematologic, and histological characteristics of two patients who progressed from refractory anemia to acute leukemia are described. When first studied, nuclear bridging of erythroblasts, similar to that seen in congenital dyserythropoietic anemia type I and megakaryocytic dysplasia, were the only abnormalities. Within 6 years, both patients died, the first of acute nonlymphocytic leukemia, the second of erythroleukemia. Nuclear bridging of erythroblasts in the marrrow of these patients was an early and transient phenomenon and was not observed during the terminal phase of leukemia.
Deoxythymidine and deoxycytidine incorporation into DNA in B leukemic cells
Authors: Vertongen F. , Lebacq A.‐M. , Fondu P. , Mandelbaum I. M. 
Published: February 2007

AbstractMeasurements of radioactive DNA after incubation of normal and B leukemic peripheral mononuclear cells, from c‐ALL and CLL with labeled deoxythymidine (dTh) and deoxycytidine (dCt) showed that for dTh, incorporation into DNA was similar for normal and c‐ALL cells but lower in B‐CLL cells and that for dCt, incorporation was highest in c‐ALL and lowest in CLL cells. These results contrast with those of dTh and dCt kinase activities; the former has been previously found elevated in c‐ALL cells, and the latter is found, in the present study, similar in the three groups tested.
Vitamin E consumption by human blood platelets activated by latex particles
Authors: Principe Domenico Del , Menichelli Adriana , Lubrano Riccardo , Bandino Donatella , Giulio Stefano Di , Di Corpo Maria Luisa , Giardini Omero 
Published: February 2007

AbstractHuman blood platelet activation elicited by latex particles is associated to a 30% decrease in the cellular content of vitamin E. The vitamin E consumption is inhibited by the addition of catalase (500 U/ml) and azide (1 mM), but it is not affected by potassium cyanide (1 mM). It may be proposed that the challenge of platelets with particulate stimuli causes generation of oxygen reduction products, which leads to vitamin E depletion.