bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intrasplenic lymphoma resembling multiple hematomas in a hemophiliac
Authors: Sweeney Joseph D. , Hassett James M. 
Published: February 2007

AbstractA 42‐year‐old patient with hemophilia A developed acute left hypochondrial pain 8 days postnephrolithotomy. A colloidal scan indicated multiple hematomas, and conservative management was given. Postoperatively, recurrent discomfort necessitated splenectomy. Multiple space‐occupying lesions were found, and the histology was that of a large, noncleaved lymphoma. Contiguous spread outside of the capsule was evident, but abdominal nodes and liver appeared normal. Chemotherapy was administered, with evidence of tumor response, but refractoriness supervened, and the patient died 4 months after the diagnosis. Space‐occupying lesions in a hemophiliac are generally ascribed to hematomas. The exposure of this population to the human immunodeficiency virus (HIV) should raise the consideration of a non‐Hodgkin lymphoma in any situation where rapid clinical resolution of a hematoma is not occurring.
Failure of high‐dose I.V. Gammaglobulin in the treatment of spontaneously acquired factor VIII inhibitors
Authors: Heyman Meyer R. , Chakravarthy Anuradha , Edelman Bennett B. , Needleman Samuel W. , Schiffer Charles A. 
Published: February 2007

AbstractTwo patients with spontaneously acquired factor‐VIII‐CC inhibitors were treated with highdose i.v. gammaglobulin. Minimal inhibfiion of anti‐VIII‐CC activity was demonstrated in vitro in one patient when i.v. gammaglobulin was mixed directly with his plasma. In neither patient, however, was there any signficant reduction in titer of antifactor VIII‐C after i.v. gammaglobulin administration, nor was there any shortening of the aPTT or reduction in bleeding. The possible mechanisms of the previously reported salutary benefits of high‐dose i.v. gammaglobulin in patients with spontaneously acquired factor‐VIII inhibitors as well as the potential reasons for failure in our patients are discussed. Our cases demonstrated that high‐dose i.v. gammaglobulin is not uniformly successful in the treatment of patients with acquired factor‐VIII‐C inhibitors. The true frequency of clinically signficant responses will require further clinical trials.
Busulfan‐induced sideroblastic anemia
Authors: Fernandez Louis A. , Zayed Ekram 
Published: February 2007

AbstractPatients in the stable phase of chronic myelogenous leukemia (CML) are usually treated with busulfan. The bone marrow of patients with CML may be exquisitely sensitive to busulfan, and occasionally such patients develop pancytopenia, secondary to hypoplasia or aplasia of the bone marrow, which is presumed to be due to busulfan‐induced marrow toxicity. We report a case of Philadelphia chromosome‐positive CML who developed pancytopenia while being treated with busulfan; however, the patient's bone marrow was not hypoplastic or aplastic but rather hyperplastic with sideroblastic changes. Busulfan is not known to cause sideroblastic changes, so this was considered to herald a transformation into acute leukemia. Busulfan was stopped, and only supportive treatment was given. To our surprise approximately 22 weeks after busulfan was stopped, the sideroblastic changes had disappeared and the bone marrow again showed features of CML. This case suggests that busulfan may cause sideroblastic anemia.
Physiologic formation of intracellular vesicles in mature erythrocytes
Authors: Sills Richard H. , Tamburlin Judith H. , Barrios Nilka J. , Yeagle Philip L. , Glomski Chester A. 
Published: February 2007

AbstractThe ability of mature erythrocytes to spontaneously form intracellular vesicles has been implied from clinical studies but has not been examined experimentally. An in vitro model was developed to demonstrate whether mature erythrocytes are capable of spontaneously forming intracellular vesicles. Normal human erythrocytes were incubated in vitro at 37°C for 144 hr in a synthetic medium. During the course of these incubations, approximately 4% of erythrocytes developed intracellular vesicles which were quantitated by using interference contrast microscopy. Electron microscopic studies confirmed the intracellular nature of these vesicles. Incubation of erythrocytes in autologous plasma produced similar results. The rate of vesicle acqulsition in vivo was measured by quantitating erythrocyte vesicles immediately prior to and following splenectomy. The rates of vesicle acquisition in vivo and in vitro were comparable. This in vitro model confirms the ability of mature erythrocytes to spontaneously form intracellular vesicles and strongly supports the concept that this is a physiologic process.
Some characteristics of human red blood cells separated according to their size: A comparison with density‐fractionated red blood cells
Authors: Vaysse Jenny , Vassy Roger , Eclache Virginie , Gattegno Liliane , Bladier Dominique , Pilardeau Paul 
Published: February 2007

AbstractDuring their in vivo ageing, red blood cells (RBC) increase in density and become smaller. Age‐defined RBC subpopulations are usually collected by centrifugation. A fractionation according to RBC volume has been proposed as an improved alternative to such age separation. Because a few data reported in the literature indicate some discrepancies between the two methods, blood samples were separated either by centrifugation or by counterflow centrifugation, and some characteristics of the RBC thus fractionated were studied. The enzyme activities decrease either when the density rises or when the volume (MCV) decreases. However, the comparison of other RBC characteristics strongly suggests that these two procedures do not lead to the collection of the same RBC subpopulations: for instance, the hemoglobin content increases when the MCV rises, whereas it remains constant whatever the RBC density is. With radiolabelled cells, it is shown 1) that the most dense RBC are recovered in all the size‐separated RBC subpopulations, even though they tend to concentrate in the fractions with the largest MCV, and 2) that the smallest RBC are almost fairly distributed in all the RBC subpopulations, whatever their density, whereas the largest RBC are mainly, but not exclusively, present in the high‐density fractions. Thus, fractionation according to size does not match separation according to density. Taken together with results from in vivo experiments carried out in mice and with the fact that reticulocytes are present in all the size‐separated fractions, these data suggest that counterflow centrifugation may be a very questionable procedure to achieve a RBC fractionation according to age and therefore that RBC volume might not be a reliable criterion of RBC age.
Cell cycle distribution defect in PHA‐stimulated T lymphocytes of sickle cell disease patients
Authors: Abdallah J. M. , Kukuruga Mark , Nakeff Alexander , Prasad Ananda S. 
Published: February 2007

AbstractT lymphocytes from normal human controls and sickie cell disease (SCD) patients were isolated from peripheral blood and cultured for 72 hours following addition of phytohem‐agglutinin. The ratio for the fraction of cells in DNA synthesis (S phase) over the fraction in G2 phase (S/G2) was significantly higher in SCD patients in comparison to the controls (mean ± SD) (4.01 ± 0.78 vs. 2.78 ± 0.76, P < 0.02). Following in vivo zinc supplementation to two subjects, the S/G2 ratio was normalized. We conclude that the distribution of T lymphocytes in cell cycle is altered in SCD patients and that this effect may be zinc‐de‐pendent.
Neonatal and maternal platelets: Activation at time of birth
Authors: Suarez Carlos R. , Gonzalez Jose , Menendez Cesar , Fareed Jawed , Fresco Raoul , Walenga Jeanine 
Published: February 2007

AbstractDetermination of the plasma concentrations of beta‐thromboglobulin (BTG), thromboxane B2(TxB2) and platelet factor 4 (PF4) were made at the time of birth in 18 newborns and their respective mothers. Both groups show significant elevation of all these molecular markers, suggesting marked platelet activation. The elevated TxB2levels in the newborn group, 25 ± 8 pg/ml, are compatible with a normally functioning and activated platelet prostaglandin pathway. Mode of delivery, vaginal or caesarean section, does not significantly influence the degree of activation in either group. Ultrastructural platelet examination did not reveal any morphologic differences between maternal and newborn platelets. There appears to be marked activation of the newborn and maternal platelet systems at the time of birth, and we postulate that this may explain in part the transient platelet dysfunction observed in newborns.
Effect of ferrous and ferric chelators on transferrin‐iron‐macrophage interactions
Authors: Baynes Roy D. , Friedman Brian M. , Bukofzer Gail T. , Bothwell Thomas H. , Macfarlane Bruce J. , Lamparelli Rosario D. 
Published: February 2007

AbstractA study was done to evaluate the effect of ferrous and ferric chelators on the interaction between transferrin‐iron and cultured human blood monocytes. This interaction has been previously shown to involve a specific receptor and vesicle protonation. Transferrin‐iron uptake was significantly inhibited by the hydrophobic ferrous chelator 2,2′ bipyridine, and the inhibition was shown not to be a consequence of the mobilisation of intracellular iron by the chelator. Chase experiments and prolonged incubation studies suggested that the chelator prevented the iron released from transferrin from negotiating the unit membrane. The iron and transferrin then appeared to be returned independently to the incubation medium. In contrast, a hydrophilic ferrin chelator, desferrioxamine, had only a very modest effect on the interaction. These findings are compatible with the hypothesis that transferrin‐iron is reduced to the ferrous state during its uptake by the cultured human blood monocyte.
Monoclonal gammopathy and antibody activity against the acetylcholine receptor
Authors: Ahlberg Richard E. , Lefvert Ann Kari 
Published: February 2007

AbstractA 50‐year‐old woman with muscular fatigue and monoclonal gammopathy of unknown significance was shown to have high levels of antibodies against the acetylcholine receptor of the skeletal muscle endplate. The specific antibody activity was exclusively associated with the monoclonal component. There were no objective signs of myasthenia gravis. This is the first reported case with antibody activity against the acetylcholine receptor found in a monoclonal immunoglobulin fraction.
Atypical haplotypes linked to the βs gene in Africa are likely to be the product of recombination
Authors: Srinivas Rao , Dunda Olga , Krishnamoorthy Rajagopal , Fabry Mary E. , Georges Alain , Labie Dominique , Nagel Ronald L. 
Published: February 2007

AbstractWe report here the haplotypes of 10 Mstll‐defined SS patients and a S/β0 thalassemia from the Central African Republic, exhibiting 7 different atypical haplotypes that are different from the typical Bantu haplotype that characterize over 93% of the βs bearing chromosomes in that region of Africa. Of the seven atypical haplotypes, six can be easily interpreted as the result of recombination around the “hot spot” 5′ of the β gene, between a typical Bantu haplotype and other haplotypes available in the normal population. Except for one case that requires further study, this result demonstrates that the main mutational event leading to sickle hemoglobin in Bantu‐speaking Africa was the mutation of the β gene in a Bantu haplotype background.
Interaction of heterozygous βo‐thalassemia with single functional α‐globin gene
Authors: Galanello Renzo , Paglietti Elisabetta , Melis Maria A. , Crobu Maria G. , Addis Maria , Moi Paolo , Cao Antonio 
Published: February 2007

AbstractIn this study, we analyzed the phenotypic manifestations resulting from the interaction of heterozygous βo‐thalassemia(βo‐39 nonsense mutation) with the functional loss of three α‐globin structural genes in six subjects, of whom four had the [‐αl–] α‐globin genotype and two the [–/αThα] α‐globin genotype. The β‐thalassemia defect was in all cases the nonsense mutation at codon 39. The nondeletion α‐thalassemia αth was the initiation codon mutation (AUG→GUG) of the α‐2 gene. In all these subjects hypochromia and microcytosis were more marked than in βα ‐thalassemia heterozygotes with a full complement of four α‐globin genes. All but one had moderate anemia. The α:β globin chain synthesis ratios were consistently decreased. No cases had Hb H on electrophoresis. Subjects with [–/αThα] α‐globin genotype had more severe thalassemia‐like manifestations than those with [–/‐α] α‐globin genotype.
Treatment of “poor risk” acute nonlymphocytic leukemia with continuously infused low‐dose cytosine arabinoside
Authors: Balaban Edward P. , Cox John V. , Schneider Nancy R. , Harth Cheryl A. , Haley Barbara B. , Sheehan Richard , Frenkel Eugene P. 
Published: February 2007

AbstractThere is currently little experience using a continuous intravenous infusion of low‐dose cytosine arabinoside (LDARA‐C) in the treatment of acute nonlymphocytic leukemia (ANLL). We report the results in 12 patients with ANLL described as either relapsed ANLL, ANLL with a preceding myelodysplastic phase, or ANLL in the elderly treated with 14 days of continuous intravenous LDARA‐C (20 mg/m2/day). Complete responses (CR) were seen in five patients (42%) and partial responses (PR) in three patients (25%). Treatment resulted in overall and clonal cytoreduction, which was evident by serial bone marrow exams and bone marrow cytogenetic analysis. The ability to obtain a CR correlated with the finding of a low initial marrow cellularity (P < .05). This study finds that continuous intravenous infusion of LDARA‐C for ANLL can achieve response rates comparable to standard induction programs in a subset of patients traditionally defined as having a poor prognosis.
Hematologic aspects of human immunodeficiency virus infection: Laboratory and clinical considerations
Authors: Perkocha Luke A. , Rodgers George M. 
Published: February 2007

AbstractHematologic abnormalities are common in patients with HIV infection. This review will focus on HIV‐associated cytopenias and coagulation abnormalities. Their occurrence, laboratory evaluation, and clinical significance and the mechanisms underlying their development are discussed. Therapeutic modalities are presented, with an emphasis on treatment strategies for HIV‐associated thrombocytopenia.
An unusual case of extramedullary blast crisis in chronic myelocytic leukaemia
Authors: Feng Chi‐Shun 
Published: February 2007

AbstractA 27‐year‐old man with chronic myelocytic leukaemia sustained two episodes of extramedullary blast transformation. The first episode was a lymphoblastic transformation in his cervical lymph nodes, which was treated and in remission for 15 months when a second blastic transformation occurred in the meninges. All the while, the bone marrow was free from blastic crisis.
Polyclonal B‐cell lymphocytosis and hypergammaglobulinemia in patients with gaucher disease
Authors: Marti G. E. , Ryan E. T. , Papadopoulos N. M. , Filling‐Katz M. , Barton N. , Fleischer T. A. , Rick M. , Gralnick H. R. 
Published: February 2007

AbstractSera from 23 individuals with Gaucher disease (GD) were analyzed for hypergammaglobulinemia and oligoclonal and monoclonal gammopathies. Serum IgG level was elevated in 15/23 (65%) patients, and a diffuse hypergammaglobulinemia was present in 10/23 (43%) patients. An oligoclonal gammopathy was noted in six patients, and a monoclonal gammopathy in two. Lymphocyte subset analysis was also carried out in eight individuals with GD. Four of five individuals showed increased surface Ig‐positive lymphocytes, while 7/7 were positive for either increased CD19‐ and/or CD20‐positive lymphocytes. An eighth patient was found to have a B‐cell leukemia. Statistical analysis of k and λ histograms were suggestive of a monoclonal excess. However, restriction enzyme analysis of four individuals with GD and increased B cells failed to show any evidence of Ig gene rearrangements. Serum Ig abnormalities and perhaps B‐cell lymphocytosis appear to be common in the GD patient population and are not associated with circulating monoclonal lymphocytes.
Impaired echinocytic transformation of ankyrin‐ and spectrin‐deficient erythrocytes in mice
Authors: Reinhart Walter H. , Amy Sung L.‐P. , Sung K.‐L. , Bernstein Seldon E. , Chien Shu 
Published: February 2007

AbstractThe membrane skeleton of the red blood cell plays an important role in the determination of cell deformability and cell shape. Under various in vitro conditions, red blood cells undergo an echinocytic or stomatocytic shape transformation. The mechanism of this fundamental process is not well understood. We have studied the red cell shape transformation in normoblastic anemia mice (nb/nb) and spherocytic anemia mice (sph/sph), which are deficient in ankyrin and spectrin, respectively. We found that both ankyrin‐deficient cells (nb/nb) and spectrin‐deficient cells (sph/sph) have a reduced capacity to undergo echinocytic transformation with various echinocytogenic treatments, that is, incubation with sodium salicylate (40 and 120 mM), calcium loading (50 μM A23187 + 2.2 mM Ca2+), or metabolic depletion (24 hr at 37°C). These results suggest that the functional integrity of the membrane skeleton is essential for the maintenance and transformation of the red cell shape.
Familial type II protein C deficiency associated with warfarin‐induced skin necrosis and bilateral adrenal hemorrhage
Authors: Conlan Maureen G. , Bridges Alan , Williams Eliot , Marlar Richard 
Published: February 2007

AbstractA family is described in which venous thromboembolic disease is associated with reduced plasma protein C activity and normal levels of protein C antigen. Immunoelectrophoretic analysis of protein C antigen gave an abnormal pattern in all affected members, suggesting that the disorder is related to the presence of a structurally and functionally abnormal form of protein C. The propositus developed simultaneous warfarin‐induced skin necrosis and bilateral adrenal hemorrhage. This is the first reported instance of warfarin‐induced skin necrosis associated with a dysfunctional protein C molecule and the first reported instance of simultaneous warfarin‐induced skin necrosis and bilateral adrenal hemorrhage.
Hemoglobin southampton (Casper): Characterization of the base mutation
Authors: Heintz Nicholas H. , Howard Phillip L. 
Published: February 2007

AbstractHemoglobin Casper is characterized by the substitution of cytidine for thymidine in condon 106 of the beta globin gene. This substitution results in the creation of a new restriction site for Msp I but not for the isoschizimer Hpa II. The restriction pattern following digestion with Msp I reveals a 9.9‐kb fragment not seen in normal individuals or following digestion of Casper DNA with Hpa II. This finding confirms the predicted base mutation and indicates that the cytidine in the newly acquired CpG site is methylated.
Comparative efficacy of nonheated and heat‐treated factor IX complex concentrate in treatment of hemophiliacs with inhibitors
Authors: Schwartz Richard S. , Ewing Nadia P. , Gorenc Toni J. , Spero Joel A. 
Published: February 2007

AbstractTo determine if heat‐treated factor IX complex concentrate is as effective as nonheated factor IX complex concentrate for treatment of subjects with hemophilia A and antifactor VIII antibodies (inhibitor patients), we have retrospectively reviewed consecutive home treatment records of ten inhibitor patients who had been receiving nonheat‐treated factor IX complex concentrate (NHT‐Konyne) and subsequently converted to heat‐treated factor complex concentrate (Konyne‐HT) when it was licensed in late 1984. Overall, 162 of 284 (57%) separate bleeding episodes treated with NHT‐Konyne and 53 of 80 (66.3%) separate bleeding episodes treated with Konyne‐HT required only one treatment course of ∼60‐75 U/kg. The distribution of bleeding sites and the absolute factor IX unitage required per treatment episode were similar for both preparations. These data suggest that the percentage of hemophilic inhibitor patients responding to factor IX complex concentrate remains at least 50%, as was reported several years ago in a controlled study, and that inhibitor bypass activity has not altered by heat treatment.
Biochemical assays of hemoglobin in normal human erythroid clones
Authors: Barr Ronald D. , Koekebakker Marijke 
Published: February 2007

AbstractQuantitation of small amounts (2–3 μg) of hemoglobin (Hb) was achieved by each of three spectrophotometric techniques. An assay employing benzidine base consistently underestimated Hb at this level and gave a wide scatter of results at higher values. The cyanmethemoglobin method was at the limit of its sensitivity and suffers from the disadvantage of having high optical densities in the blank samples. By contrast, pyridine hemochromogen can be detected accurately with 200 ng of Hb (equivalent of 6,000 mature erythrocytes), and the regression line with this technique virtually passes through the origin. Furthermore, the extinction coefficient of pyridine hemochromogen in the Soret band is 13.5‐fold greater than that of cyanmethemoglobin. In normal human erythroid clones, generated in vitro from bone marrow cells, mean cell hemoglobin (MCH) values were determined after various intervals of culture. The MCH after 5, 7, 10, and 14 days were 11.8, 15.8, 26.6, and 34.4 pg, respectively, by the pyridine hemochromogen method. Reaction product was also identified in granulocyte‐macrophage clones, presumably reflecting the content of other heme proteins such as catalase and cytochromes. Account must be taken of this non‐Hb material in computing true MCH values for erythroid cells.
Sex practice correlates of human immunodeficiency virus transmission and acquired immunodeficiency syndrome incidence in heterosexual partners and offspring of U.S. hemophilic men
Authors: Lawrence Dale N. , Jason Janine M. , Holman Robert C. , Heine Peggy , Evatt Bruce L. 
Published: February 2007

AbstractWe assessed the risk of human immunodeficiency virus (HIV) transmission from heterosexual seropositive hemophilic men to their female sex partners through an HIV sero‐survey and questionnaire study conducted during 1984–1987. Five percent of 21 female partners of asymptomatic men and 11% of 35 partners of HIV‐symptomatic (acquired immunodeficiency syndrome [AIDS], AIDS‐related complex [ARC], peripheral generalized lymphadenopathy [PGL]) hemophilic men had been infected when first tested. One of 19 seronegative women tested about 1 year later reportedly seroconverted. Only 18% of a sample of the serosurvey women responding to sex practices questions said their partners used condoms “nearly always.” Over 60% engaged in oral/genital sex in addition to vaginal intercourse. Only 12% of still‐seronegative women followed the preventive strategy of consistent avoidance of oral/genital sex, together with consistent condom use by the male partner.Further evidence for heterosexual transmission comes from the CDC national AIDS surveillance reports showing 25 women who acquired HIV infection through heterosexual contact with U.S. hemophilic men (September 6, 1988). Seven (28%) were diagnosed and reported in the first 6 months of 1988. Their ages range from 20 years to more than 70 years. The dates of infection for the women are unknown but must have been at least 5 years before AIDS diagnosis for at least one. Only approximately 30% of their male partners had already manifested any HIV‐assodaied illness.Through May 18, 1988, six cases of AIDS have occurred in children whose infection was acquired through exposure of the mother to a hemophific partner. Four were diagnosed in latter 1987. The median age at diagnosis was 4.5 months. Four had died. None of their mothers is as yet diagnosed.