bluuebunny/crossref_metadata_2025_split huggingface dataset:
A new mutant erythrocyte glucosephosphate isomerase (GPI) associated with GSH abnormality
Authors: Zanella Alberto , Rebulla Paolo , Izzo Crescenzio , Zanuso Francesco , Sirchia Girolamo , Kahane Itzhak , Molinari Ermete 
Published: February 2007

AbstractA case of congenital nonspherocytic haemolytic anaemia associated with a new abnormal glucosephosphate isomerase (GPI), GSH (reduced glutathione) deficiency, and instability and altered carbohydrate membrane composition is reported. The only functional abnormality of the mutant enzyme seems to be a marked instability to heat, urea, and guanidine‐HCl. Family studies suggest that the propositus is doubly heterozygous for a maternal gene producing an inactive enzyme and a paternal gene responsible for a structural alteration causing marked lability of the coded enzyme. Experiments of incubation of normal GPI and the propositus's GPI with oxidizing and reducing agents seem to indicate that the abnormality resides in the SH groups of the mutant GPI.
Deoxyuridine suppression studies in bone marrow in primary refractory sideroblastic anemia
Authors: Goodman Anita , Bacon Bruce , Hines John D. 
Published: February 2007

AbstractBecause unexplained megaloblastic erythroid maturation occurs in patients with primary refractory sideroblastic anemia (PRSA), deoxyuridine (dU) suppression tests using125 I‐UdR were performed on bone marrow from five patients with PRSA. All patients had megaloblastic alterations in the marrow erythroid precursors (with normal serum folate and B12 levels) and numerous ringed sideroblasts, and most had marrow iron overload. Results of the dU suppression tests were normal in all five, both with and without incubation with pharmacologic amounts of folate, B12, and pyridoxal phosphate with or without 1‐serine.Despite occasional hematologic improvement subsequent to folate, pyridoxine, or pyridoxal phosphate (PLP) administration in some patients with PRSA, normal dU suppression implies that in our group the availability of B12 and folate (and possibly PLP) was not limiting in the utilization of folate for DNA‐thymine synthesis. The marrow erythroid maturation defect remains unexplained.
Adult severe combined immunodeficiency and sarcoid‐like granulomas with hypersplenism
Authors: Edelstein Alan D. , Neiman Richard S. , Miller Aaron , Zimelman Abraham P. , Rocklin Ross E. 
Published: February 2007

AbstractA patient presented with lymphopenia, anergy, hypogammaglobulinemia and hypersplenism. Histologic examination of the spleen and lymph node revealed noncaseating sarcoid‐like granulomas. Despite a significant rise in circulating lymphocytes after splenectomy there was in vivo and in vitro evidence of B‐ and T‐lymphocyte dysfunction. A histologic picture mimicking sarcoidosis may occur in patients with immune deficiency. The granulomatous proliferation may represent an altered host response to antigen.
Cytosine arabinoside modulation of the mitotic index in treatment of adult acute leukemia
Authors: Mackinney Archie A. , Flynn Bridgett 
Published: February 2007

AbstractTwenty‐three unselected adult acute leukemic patients were treated with an initial intravenous pulse of cytosine arabinoside (Ara‐C) 5 mg/kg. The mean mitotic index dropped from 7.7/1,000 to 2.8/1,000 at 4 hours after Ara‐C pulse and rebounded to 6.7/1,000 at 18 hours. Infusions of Ara‐C 7‐10 mg/kg were given for 9 or 12 hours, depending on changes in the mitotic index. The group of responders (M1 or M2 marrows) could be predicted by the initial blast count in the marrow and the change in the mitotic index 18 hours after the Ara‐C pulse. There were 9 (39%) complete and partial remissions. Six patients remained in remission more than 200 days. Since the changes in the mitotic indices induced by Ara‐C were indistinguishable from those of another more successful trial, we infer that factors other than modulation of the cell cycle are critical to the success of this mode of therapy.
Proliferative function of cadaveric bone marrow cells
Authors: Liu Paul L. , Ogawa Makio , Crook Lynn , Ochia Roland , Upshur Jane K. 
Published: February 2007

AbstractThe proliferative function of human and murine cadaveric bone marrow was studied using methylcellulose clonal cell culture assays and the murine spleen colony technique. The study revealed persistence of hemopoietic functions for as long as 19 postmortem hours in cadaveric marrows of some patients. Studies of murine cadaveric marrows corresponded with those of human marrows. These results strongly suggest that human pluripotent hemopoietic stem cells survive in cadaveric marrows.
Permanent preparations of granulocyte macrophage cultures in agar gel
Authors: Mendelow B. , Grobicki D. , De La Hunt M. , Katz J. 
Published: February 2007

AbstractA simple method for the permanent preservation of agar cultures is described. The technique involves fixation of agar cultures after incubation, drying, and subsequent remounting and staining on glass slides. The technique permits differential counting of colony type, approximate quantitation of cell numbers within individual colonies or clusters, and permanent preservation of cultures for purposes of interlaboratory standardization, and for routine reference in patient records.
Control of Hemoglobin Synthesis in Fetal Erythroid Cells by L‐Thyroxine
Authors: Fuhr J. E. , Dunn C. D. R. 
Published: February 2007

AbstractFetal mouse liver cells were incubated in the presence of L‐thyroxine. Globin synthesis by cells from the 13th to 15th days of gestation was stimulated by the presence of the hormone. Although protein synthesis by cells from the 17th day of gestation was stimulated, the protein was not identified as globin. A tentative conclusion was drawn that fetal erythroid tissue may be preferentially stimulated by thyroid hormone.
Elevated plasma levels of fibrinopeptide a during sickle cell anemia pain crisis–evidence for intravascular coagulation
Authors: Leichtman David A. , Brewer George J. 
Published: February 2007

AbstractA role for coagulation in the pathogenesis of sickle cell anemia (SCA) pain crisis has long been suspected. We have observed evidence for in vivo coagulation in patients with SCA during pain crisis utilizing a radioimmunoassay for fibrinopeptide A (FPA). Since the proteolytic activity of thrombin results in the release of FPA from fibrinogen, plasma levels of FPA may be used as an index of in vivo thrombin activity. In 20 normal controls FPA plasma levels had a range of 0.6–1.4 ng/ml and a mean of 0.7 ± 0.2 ng/ml. We observed elevations of FPA, values greater than the mean plus 4 standard deviations of the control group, in 21 consecutive plasma samples obtained from 8 SCA patients during pain crisis. FPA determinations on 7 of 10 plasma specimens obtained from 10 SCA patients during pain‐free periods were within the normal range. Elevations of FPA in 2 of 3 of these pain‐free patients could be explained by recent extensive oral surgery and faulty venopuncture, conditions known to cause elevated levels of the peptide. Plasma specimens were obtained from 2 SCA patients patients with subjective complaints of pain, but who were suspected of malingering prior to FPA determination. In both instances, FPA was within the normal range.In summary, plasma levels of FPA appear to be elevated in SCA patients during crisis as contrasted to pain‐free periods and normal controls. This study provides new evidence for the occurrence of intravascular coagulation during SCA pain crisis. Further studies are needed to assess whether this activation of the coagulation system is a cause or an effect of pain crisis.
Erythroleukemia: In vitro studies of erythropoiesis
Authors: Newcomb M. M. , Balducci L. , Coleman M. B. , Steinberg M. H. 
Published: February 2007

AbstractWe studied the growth of erythroid burst‐forming units (BFU‐E) and erythroid colony forming units (CFU‐E) from bone marrow and blood in six patients with erythroleukemia. Five patients grew CFU‐E, while BFU‐E were found in the marrow of two and in the peripheral blood of only one patient. In all cases with colony growth, the numbers of colonies were markedly decreased with respect to normal controls. Patient BFU‐E were composed of fewer clusters than those of controls. BFU‐E and CFU‐E growth was dependent on the addition of erythropoietin to the medium, and no growth was observed in absence of erythropoietin. At present it is not known if the growth obtained is derived from residual normal erythropoietic stem cells or from abnormal erythroid precursors of the leukemic cells.
The lipids of the erythrocyte in paroxysmal nocturnal hemoglobinuria
Authors: Gockerman Jon P. 
Published: February 2007

AbstractParoxysmal nocturnal hemoglobinuria (PNH) is a disorder characterized by the production of abnormal erythrocytes (PNH‐E). The PNH‐E undergoes early intravascular destruction which appears to be related to an intrinsic membrane defect that results in extreme sensitivity to lysis by complement. The lipids of the PNH‐E have been evaluated because of the variable results reported in the past. This study shows a normal content of cholesterol and lipid phosphorus with a normal distribution of the phosopholipids except for a slight increase in lysophosphatidyl choline (LPC) when measured using the complete lipid extract of the PNH‐E. Fractionation of the lipid extract over a silicic acid column produced an alteration in the distribution of the phospholipids, with LPC and sphingomyelin increasing, and the other phospholipds decreasing. These alterations were seen only when the phospholipids were fractioned over a silicic acid column and were clearly an in vitro phenomenon. The levels of plasmalogen and malonaldehyde were normal, unlike those seen with oxidant stress. Fatty acid analysis of the phospholipids showed small but consistent changes: increased 16:0 and decreased 18:2. The total glycosphingolipids were slightly decreased in the patients with severe PNH. The results support and extend previous lipid data indicating abnormalities in the phospholipids of the PNH‐E. The alterations of the phospholipids with fractionation correlate with previous whole cell data that suggest lipid instability in the PNH‐E.
Unbalanced globin chain synthesis by Hb lincoln park (anti‐lepore) reticulocytes
Authors: Honig George R. , Mason R. George , Tremaine Larry M. , Vida Loyda N. 
Published: February 2007

AbstractHemoglobin synthesis was studied in vitro in reticulocytes from a patient with the anti‐Lepore variant Hb Lincoln Park. Incorporation of L‐leucine‐3H into the α and βδ chains of Hb Lincoln Park was substantially less than the incorporation into the corresponding globin chains of Hb A, with the rate of synthesis of the βδ chain being similar to that of the δ chain of Hb A2. Synthcis of the a and total non‐α globin components was unbalanced, with a substantial excess of α chain synthesis. These findings help to explain the mild hemolytic disease present in individuals with this hemoglobin variant.
Quantitation of globin chains using cellulose acetate electrophoresis: Analysis of fetal globin chain production
Authors: Barton Jennifer , Smith Merran B. , Cauchi M. N. 
Published: February 2007

AbstractA technique involving the quantitative estimation of globin chains using cellulose acetate electrophoresis is described, and applied to the analysis of fetal globin chain production. When cord blood was analyzed, there was poor correlation between the globin chain ratios namely β/α, γ/α and β/γ ratios with either age or weight of newborn babies between 36–40 weeks gestation: best correlations were obtained for the β/γ ratio against weight (P < 0.05). However, there was highly significant correlation between the various globin chain ratios and either age or weight of the newborn infant up to 12 weeks of age (P < 0.001). This technique allows visual demonstration and quantitation of abnormal haemoglobins in a single procedure.
An acquired cell‐directed inhibitor of neutrophil chemotaxis and hypogammaglobulinemia
Authors: Casciato Dennis A. , Ford Larry C. , Busch David F. 
Published: February 2007

AbstractAn unusual combination of host defense abnormalities was demonstrated in an adult male with recurrent pulmonary infections due to a variety of microorganisms. Polymorphonuclear neutrophil chemotaxis was defective. Other neutrophil and T‐lymphocyte function tests were normal. The patient's serum also showed a severe deficiency of IgG, no detectable IgA, IgM, or IgD, and increased IgE. The chemotactic defect was shown to be due to a cell‐directed inhibitor in the patient's serum. The effect of the inhibitor on chemotaxis could be antagonized by factors in normal serum. The chemotaxis defect persisted for several months, but eventually returned to normal.
Heterogeneity of hemoglobin gamma chains in normal newborns and in cases of alpha and beta thalassemia
Authors: Lie‐Injo Luan Eng , Randhawa Z. I. , Kane J. P. , Ganesan J. , George R. 
Published: February 2007

AbstractThe ratio of Gγ to Aγ chains was studied in 13 normal healthy newborns and in eight neonates with hydrops fetalis due to homozygous α‐thalassemia. The findings in the normal healthy newborns agreed with those of earlier reports. In homozygous α‐thalassemia the Gγ and Aγ ratio appeared generally lower than in healthy control newborns, but one of the hydrops fetalis cases had a very high Gγ value.In all 13 normal healthy newborns, in 8 patients with homozygous α‐thalassemia, and in 4 patients with homozygous β‐thalassemia, Tγ chains with threonine at position 75 were detected in addition to Iγ chains with isoleucine at position 75. In homozygous α‐thalassemia, the Tγ‐to‐Iγ ratio seemed lower and in homozygous β‐thalassemia higher than in normal newborns.
A method for cloning T‐lymphocytic precursors in agar
Authors: Löwenberg B. , de Zeeuw H. M. C. 
Published: February 2007

AbstractA new technique for the culture of T‐lymphocytic colonies is reported. The method may be regarded as a human lymphocyte precursor cell assay, as is the myeloid colony culture for granulocyte‐macrophage progenitors. The colonies arise under the simultaneous stimulation of phytohemagglutin and a leukocyte feeder. A linear relationship is found between colony numbers and cell numbers plated. The colonies represent aggregates of lymphoblast‐like cells, the majority of which are capable of E‐rosette formation, are responsive in mixed lymphocyte cultures, and do not exhibit surface immunoglobulins. Their density distribution profile is very similar to that of myeloid colony‐forming cells. The finding that most of these colony‐forming cells are recovered in the so‐called lymphocyte‐free stem cell fraction following density fractionation suggests that they originate from a lymphocytic precursor.
A simplified [3H] serotonin release assay for the detection of platelet antibodies
Authors: Marmer Daniel J. , Bowman Robert P. , Kennedy Peter S. 
Published: February 2007

AbstractThe [3H] platelet serotonin release assay is a sensitive means for detecting antiplatelet antibodies. We have found that by using prelabeled frozen platelets which have been treated with the cryoprotective agent dimethyl sulfoxide (DMSO) the performance of this assay can be facilitated without interfering with its sensitivity. There was 100% correlation between the standard [3H] platelet serotonin release assay using fresh platelets and the modified assay using prelabeled, frozen, cryopreserved platelets when sera from 12 patients with known antiplatelet antibodies were tested. When normal serum samples (n = 111) were tested against ten platelet donors, a false‐positive rate of 3.9% was observed. This modification provides a simple means for quickly screening large numbers of potential platelet donors at one time.
The association of sickle cell anemia with heterozygous and homozygous α‐thalassemia‐2: In vitro HB chain synthesis
Authors: Felice A. E. , Webber B. , Miller A. , Mayson S. M. , Harris H. F. , Henson J. B. , Gravely M. E. , Huisman T. H. J. 
Published: February 2007

AbstractThe in vitro synthesis of hemoglobin chains was investigated in 34 scikle cell anemia (SS) patients and five patients with Hb S‐βo‐thalassemia. Incubations were made for 30 minutes and for 120 minutes. Hematological and family data were also obtained. Although the 30‐minute α/non‐α total activity ratios were more widely distributed than the ratios at 120 minutes, a distinct classification of SS patients into groups without an α‐thalassemia, with a heterozygous α‐thal‐2, or with a homozygous α‐thal‐2 could not be made. Family studies indicated that four patients who had 30‐minute α/non‐α ratios below 0.82 and mean corpuscular volume (MCV) values below 70 fl had a homozygosity for both α‐thal‐2 and Hb S. They had mild hematological features of SS disease. Many SS patients with 30‐minute α/non‐α ratios between 0.8 and 1.0 and MCV values above 70 fl had an associated α‐thal‐2 heterozygosity. Their hematological features were similar to those of SS patients with four active α chain genes. It appears that an α‐thal‐2 heterozygosity (−α/αα; βS/βS) does not alter the hematological expression of SS disease. An α‐thal‐2 homozygosity −α/−α;βS/βS results in a microcytosis similar to that seen in Hb S‐βo‐thalassemia patients. The diagnosis of these α chain deficiencies in association with SS disease (by in vitro chain synthesis analyses) leaves several uncertainties.
Acquired aplastic anemia: Antibody‐mediated hematopoietic failure
Authors: Freedman Melvin H. , Gelfand Erwin W. , Saunders E. Fred 
Published: February 2007

AbstractThe hematopoietic failure in aplastic anemia was studied in six patients by assessing marrow erythroid stem cell (CFU‐E) and granulocytic stem cell (CFU‐C) colony growth in vitro. CFU‐E and CFU‐C were absent or reduced in five patients, and their serum did not inhibit CFU‐E or CFU‐C growth from control marrows. In contrast, marrow from one patient yielded 80 CFU‐E/105 and 16 CFU‐C/105 (control CFU‐E: 197/105 cells plated, range 110–387; control CFU‐C: 48/105, range 15–106). His serum decreased CFU‐E (122 to 9/105) and CFU‐C (71 to 32/105) from control marrow, and CFU‐E from his own marrow (80 to 37/105). His peripheral blood lymphocytes cocultured with autologous or control marrow similarly decreased CFU‐C. The inhibition was more marked in cocultures using a B‐cell enriched lymphocyte fraction obtained by albumin density gradient centrifugation. IgG separated from the patient's serum demonstrated a dose‐responsive suppression of CFU‐E (102 to 8/105), and of CFU‐C (58 to 28/105) from control marrow. We conclude that of the six patients, one demonstrated an antibody‐mediated hematopoietic failure. It is recommended that all patients with acquired aplastic anemia be studied for this, since in vitro testing can detect those in whom immunosuppression and plasmapheresis may be more appropriate therapy than marrow transplantation.
Marked hypodiploidy in a case of acute undifferentiated leukemia
Authors: Como Ronald , Sarna Gregory , Sparkes Robert S. 
Published: February 2007

AbstractA 29‐year‐old white man with acute undifferentiated leukemia had bone marrow cells with predominantly 42 chromosomes including several rearrangements. Following a brief induced “complete” remission, during which normal chromosomes were observed, bone marrow cells with 42 chromosomes but a different karyotype than before remission were found. Evaluation of that karyotype indicates not only a reduced chromosome count but also a reduced quantity of chromosome material.
Immune thrombocytopenic purpura in Hodgkin disease
Authors: Hassidim Kamran , McMillan Robert , Conjalka Michael S. , Morrison John 
Published: February 2007

AbstractImmune thrombocytopenic purpura is rarely seen in Hodgkin disease and the presence of platelet‐associated antibody has not been previously reported in these patients. A patient with Hodgkin disease is described who developed a destructive thrombocytopenia demonstrated by shortened platelet survival. In conjunction with his thrombocytopenia, he had marked elevation of platelet‐associated immunoglobulin G levels (nanograms IgG per 109 platelets: 15,187 prior to splenectomy and 71,130 and 81,900 after surgery). Mean values (± SD) of control subjects averaged 1,975 ± 381 and four patients with Hodgkin disease and normal platelet counts had levels ranging from 1,581 to 4,011.We suggest that this patient had immune‐mediated thrombocytopenia; whether the increase in platelet‐associated immunoglobulin G was due to antiplatelet antibody or to adsorbed or phagocytosed immune complexes cannot be demonstrated by these studies. The platelet‐associated immunoglobulin G test may be useful in evaluating these patients.
Chromosome changes in a patient achieving complete remission in the acute phase of chronic myelogenous leukemia
Authors: Stern Ronald , Sorenson Julie , Wurster‐Hill Doris H. , Cornwell Gibbons G. , McIntyre O. Ross 
Published: February 2007

AbstractA patient with chronic myelogenous leukemia (CML) had a Philadelphia chromosome (Ph′) [t(9q+;22q–)] in all cells and trisomy C in 13% of cells (later determined to be trisomy 8) at the time of diagnosis. After 21 months of intermittent treatment with busulfan, the acute phase developed with the appearance of an additional abnormality (trisomy ?19). During a complete remission and reconversion to the chronic phase, trisomies 8 and ?19 disappeared, although the Ph′ remained. Following a period of marked thrombocytosis, a second relapse occurred with the reappearance of both marker chromosomes.
The distribution of a receptor for IgM (μFcR) on haemic cells
Authors: Burns G. F. , Cawley J. C. , Worman C. P. , Barker C. R. , Hayhoe F. G. J. 
Published: February 2007

AbstractA range of normal and malignant haemic cells is examined for expression of receptors of the Fc of IgM (μFcR) in order to establish the distribution and possible nosological significance of this receptor. It is shown that μFcR expression by freshly isolated cells is restricted to a small population of normal lymphocytes and to the pathological B cells of most of 45 cases of chronic lymphocytic leukaemia (CLL) and all 23 cases of hairy‐cell leukaemia (HCL) examined. The pathological cells of acute lymphoblastic leukaemia (eight cases), prolymphocytic leukaemia (two cases), a variety of non‐Hodgkin lymphomata (eight cases) and immunoproliferative disorders (25 cases), Hodgkin disease (three cases), and a range of myeloid and histiocytic proliferations (22 cases) were all negative for the μFcR. The pathological cells of the Sezary syndrome were also shown to possess a μFcR, but this required a period of in vitro incubation in RPMI medium containing foetal calf serum for its demonstration. This and other differences between μFcR expression by T‐ and B‐cells are discussed. The possible immunological, nosological, and diagnostic significance of the observed restriction of μFcR expression is considered.
A comparison of methods for the rapid quantitation of unsaturated transcobalamin II and the r‐binding proteins
Authors: Fernandes‐Costa Francisco , Metz Jack 
Published: February 2007

AbstractMethods for the rapid quantitation of unsaturated transcobalamin II (TC II) and R‐binders in serum following separation of these binders using (a) adsorption by uncoated charcoal, (b) adsorption by Quso G32, (c) precipitation with ammonium sulphate, and (d) acidification of serum, have been compared with the standard gel filtration technique. In serum from healthy subjects and patients with elevated R‐binders, the serum acidification and Quso methods yielded results in closest agreement with those obtained by gel filtration. When TC II was markedly elevated, the recommended concentration of Quso failed to remove all TC II from serum. Both the serum acidification and Quso methods are suitable for routine use, providing that with the latter method the concentration of Quso is increased when elevated levels of TC II are anticipated, as, eg, in severe jaundice. All the rapid methods tested yielded clinically significant results in patients with myeloproliferative disorders.