bluuebunny/crossref_metadata_2025_split huggingface dataset:
Platelet calcium‐dependent proteins: Identification and localization of the calcium‐dependent regulator, calmodulin, in platelets
Authors: White Gilbert C. , Levine Steven N. , Steiner Alton N. 
Published: February 2007

AbstractThe calcium‐dependent regulator protein, calmodulin, is a 17,000 molecular weight polypeptide which binds calcium and has been shown to confer calcium sensitivity on contractile and other proteins. In the present study, we have examined the presence and subcellular distribution of this protein in preparations of human platelets. Calmodulin was quantified using a two‐stage phosphodiesterase assay. Whole platelets contained 1.33 ± 0.06 units calmodulin per 106 platelets or 26.5 ± 3.4 fg calmodulin per platelet. The distribution of calmodulin in the platelet was predominantly soluble with over 80 percent of calmodulin activity in the soluble fraction of the cell. There was no apparent difference in the distribution of calmodulin between soluble and particulate compartments in recalcified platelet homogenates compared to homogenates in EDTA. Indirect immunofluorescent studies with monospecific antisera to dinitrophenylated calmodulin showed intense staining of platelets in a diffuse pattern. The identification of calmodulin in platelets raises the possibility that this protein may participate in calcium‐dependent reactions important in platelet aggregation and release.
pH dependency of potassium efflux from sickled red cells
Authors: Roth Eugene F. , Nagel Ronald L. , Bookchin Robert M. 
Published: February 2007

AbstractPotassium efflux from deoxygenated, hemoglobin S‐containing red cells is often used as an “objective” in vitro measure of red cell sickling, particularly during tests with antisickling agents. Since varying pH is known to affect both the extent of sickling and passive K+‐flux across the red cell membrane, in opposite directions, we measured the sickling‐related K+‐efflux in sickle cell anemia (SS) and sickle cell trait (AS) red cells as a function of extracellular and intracellular pH. The sickling‐related K+‐efflux was found to show the same direction of pH dependence as normal red cells, so that as the extracellular pH was reduced below 7.6, sickling and K+‐efflux were increasingly dissociated. A similar dissociation was observed between sickling and K+‐efflux when the intracellular pH was lowered by increasing red cell organic phosphate levels. The sickling‐related K+‐efflux from osmotically shrunken AS cells (whose sickling tendency resembles that of SS cells) was similar in magnitude and pH dependency to that of the SS cells. The findings suggest that measurement of K+‐efflux may be an accurate estimate of the extent of intracellular polymerization in sickled red cells, provided that both the intracellular and extracellular pH levels are carefully controlled, and the experimental conditions produce no independent effects on K+ permeability.
Hepatocellular enzyme patterns and hepatitis b virus exposure in multitransfused young and very young hemophilia patients
Authors: Gomperts E. D. , Lazerson J. , Berg D. , Lockhart D. , Sergis‐Deavenport E. 
Published: February 2007

AbstractThirty‐eight children with severe hemophilia A, 11 years of age and under, were evaluated by initial and follow‐up liver function tests (LFTs) in relation to age of onset of transfusion therapy. Each child had at least two complete evaluations within one year for a follow‐up period of at least one year. The mean number of exposure days was 36 with a mean of 275 units of factor VIII per exposure day prior to initial LFTs. At initial testing, 30% of patients demonstrated antibody to HBsAg and 39–51% at least one abnormal serum enzyme level (AST, ALT, LDH). During an average follow‐up period of 34.8 months, two children developed HBsAg‐positive icteric hepatitis. Of those initially serologically negative for HBsAg or antibody, 44% became antibody‐positive. Intermittent abnormalities of at least one serum enzyme were observed in 79% of the patient group, with 13% and 8% being persistently normal and abnormal. Eleven children born after January 1976, receiving only third‐generation RIA‐tested products for HBsAg, constituted a subgroup. Although only one child at first assessment had evidence of hepatitis B virus exposure, 55% had elevated ALTs, indicating considerable frequency of non‐A, non‐B hepatitis in this very young group.
Serum concentrations of erythropoietin measured by radioimmunoassay in hematologic disorders and chronic renal failure
Authors: Zaroulis Charles G. , Hoffman Beth J. , Kourides Lone A. 
Published: February 2007

AbstractBioassays for human erythropoietin are cumbersome, time‐consuming, and insensitive. The purification of human erythropoietin (EP) has provided small quantities of highly bioactive EP (∼70,000 U/mg) required for the development of an EP radioimmunoassay (RIA). The RIA for EP described in this investigation, can detect 5 mU/ml of EP in the assay tube; the serum concentration of EP in normal individuals ranged from <18 to 81 mU/ml with a mean value of 29 mU/ml. In contrast, nine patients with severe aplastic anemia had markedly elevated serum EP concentrations with a mean value of 3,487 mU/ml, range 984–6,434 mU/ml. In this RIA, patients with Polycythemia vera had consistently undetectable EP concentrations, <18 mU/ml. Eleven patients with chronic renal failure had a higher mean serum EP concentration (40.5 mU/ml) than normal individuals, but the range (<18–115 mU/ml) overlapped that of normals. By immunologic and gel chromatographic criteria, EP measured in serum was similar to standard urinary EP. The EP immunoassay that we have developed has sufficient sensitivity and specificity not only to quantitate the elevated serum EP levels found in aplastic anemia but also to discriminate decreased from normal serum concentrations of EP in most circumstances. This simple, reliable RIA has provided the necessary framework upon which to increase our understanding of the importance of EP in hematopoiesis.
Double light‐chain production by leukemic cells of common clonal origin: A case report with review of pertinent literature
Authors: Choi Young Ja , Wong Martha S. 
Published: February 2007

AbstractWe are reporting the first case of acute lymphoblastic leukemia whose individual leukemic cells produced both K and λ light chains, without any heavy chains, as demonstrated by double immunofluorescent staining. This case together with cases of lympho‐plasmacytic neoplasia reported in the literature indicates that cells of a single clone are capable of producing more than a single class of light and/or heavy chains.
Hypereosinophilia in a monosomy 7 myeloproliferative disorder in childhood
Authors: Humphrey M. J. , Hutter J. J. , Tom W. W. 
Published: February 2007

AbstractHyperosinophilia in association with a monosomy 7 myeloproliferative disorder is described in an eight‐year‐old boy. Antileukemic treatment resulted in a decrease in the eosinophilia, but did not appear to affect the blast cell population or the presence of monosomy 7 cells in the bone marrow. These findings suggest that bone marrow karyotyping may be useful in differentiating pre‐malignant causes of eosinophilia from those with a more benign etiology.
Hemoglobin connecticut (β21(b3) Asp→Gly): A hemoglobin variant with low oxygen affinity
Authors: Moo‐Penn W. F. , McPhedran P. , Bobrow S. , Johnson M. H. , Jue D. L. , Olsen K. W. 
Published: February 2007

AbstractElectrophoretic analysis of a hemolysate from a young man undergoing a routine physical examination revealed an abnormal hemoglobin with a mobility similar to Hb S on cellulose acetate (pH, 8.4). This new variant, designated Hb Connecticut, was found in three generations of a family of Polish descent. Several individuals possessing the variant exhibited mild anemia. Structural analysis of the abnormal β‐chain indicated that the amino acid substitution was at position 21(B3), and involved the replacement of aspartic acid with glycine. Oxygen dissociation studies revealed low oxygen affinity. The alkaline Bohr effect and the degree of cooperativity were unchanged. Analysis of the crystal structure of the variant suggested that the low oxygen affinity was due to the possible disruption of salt bridges between aspartic acid 21(B3) and lysines 61(E5) and 65(E9), changes that could lead to steric interference in oxygen binding.
The relationship between lymphocyte nuclear morphology and cell cycle stage in lymphoid neoplasia
Authors: Diamond Lawrence W. , Weisenburger Dennis D. , Rappaport Henry 
Published: February 2007

AbstractA significant percentage of lymphoid cells isolated from three lymphoid neoplasms (nodular lymphoma mixed cell type, mycosis fungoides, and Sézary syndrome) had tetraploid or near‐tetraploid DNA content, as determined by flow cytometric analysis. In each case, a morphologically distinct population of cells with large, irregular nuclei was present, the percentage of which corresponded to the percentage of tetraploid cells. In a fourth case (diffuse “histiocytic” lymphoma), cells were sorted on the basis of DNA content from the G0‐G1 compartment and compared morphologically to cells sorted from the S‐G2(M) compartment. The G0‐G1 cells had clumped nuclear chromatin, lacked prominent nucleoli, and had a mean nuclear diameter of 10.18 ± 1.22 μm. The sorted cells with S‐G2(M) DNA content had one to several prominent nucleoli and less clumped nuclear chromatin, and they were significantly larger, with a mean nuclear diameter of 13.76 ± 1.59 μm (P < 0.00001). These preliminary results suggest that, in lymphoid malignancies, certain morphologic characteristics of individual lymphoid cells are related to their DNA content (level of ploidy) and/or the stage of the cell within its division cycle.
Successful allogeneic bone marrow transplantation after reversion to chronic phase of blast crisis in chronic myeloid leukemia
Authors: Vernant J. P. , Rodet M. , Rochant H. , Feuilhade F. , Thuran A. , Bierling P. , Lesc M. C. , Mannoni P. , Dreyfus B. 
Published: February 2007

AbstractA female with chronic myeloid leukemia (XX Ph 1 +) in blast crisis (localized to pleura and lymph nodes) was treated by polychemotherapy. After reversion to the chronic phase, an allogeneic bone marrow transplantation (BMT) was performed. Sixteen months after BMT, no sign of the disease was present (XY Ph 1 ‐).
Complement‐fixing platelet autoantibodies in autoimmune thrombocytopenia
Authors: Kayser W. , Mueller‐Eckhardt C. , Budde U. , Schmidt R. E. 
Published: February 2007

AbstractA 16‐year‐old male patient is described with chronic autoimmune thrombo‐cytopenic purpura and, after two years, “warm” autoimmune hemolytic anemia (Evans syndrome) who transiently developed complement‐fixing platelet autoantibodies. The autoreactivity of these antibodies was established by quantitative complement fixation as well as by absorption and elution studies using autologous platelets. We believe this to be the first documented case with this very rare and peculiar type of platelet autoantibody.
Prednisone‐responsive aplastic anemia associated with T‐lymphocyte proliferation
Authors: Bodner Sara , Cohen Harvey Jay 
Published: February 2007

AbstractIn some cases, bone marrow aplasia has been thought to result from immunologic abnormalities. Our patient had severe transfusion‐dependent aplastic anemia, which responded to treatment with prednisone on two occasions. The exacerbations of aplastic anemia were associated with lymphocytic proliferation which on one occasion had the characteristics of T‐cell chronic lymphocytic leukemia. Though he had had mild lymphoproliferation for a number of years, he ultimately died with progressive diffuse lymphoid infiltration of the bone marrow and other organs. The simultaneous occurrence of the T‐cell lymphoproliferation and aplastic anemia and their simultaneous response to therapy suggests that this may be a clinical example of T‐lymphocyte mediated suppression of erythropoiesis.
Opsonic activity of myeloma immuno‐globulins (mm‐lg)
Authors: Cheson Bruce D. 
Published: February 2007

AbstractPatients with MM are at an increased risk for life‐threatening bacterial infections, primarily by organisms that require opsonization for interaction with granulocytes. In the present study we used a neutrophil chemiluminescence (CL) assay of opsonization to explore the opsonic activity of MM‐lg for zymosan particles. Particles were treated with serum lacking in Ig to explore the contribution of Ig to zymosan opsonization. This serum was found to have 69 ± 2.6% (X̄ ± SEM) of the opsonic activity of normal serum (P < .001). Normal serum concentrations of normal IgG, MM‐IgG, and MM‐IgA all lacked opsonic activity for zymosan. However, when particles were treated with Nl‐IgG, MM‐IgG, or MM‐IgA as well as complement, normal opsonic activity was generated. Increasing the concentration of the Ig to stimulate MM serum did not inhibit this opsonic activity. Thus both MM‐IgG and MM‐IgA can function as normal, nonspecific opsonins.
A neutrophil lysozyme leak in patients with familial mediterranean fever
Authors: Bar‐Eli Menashe , Territo Mary C. , Peters Robert S. , Schwabe Arthur D. 
Published: February 2007

AbstractPolymorphonuclear cells derived from the peripheral blood of patients with Familial Mediterranean Fever release more lysozyme in response to high temperatures (42°, 46°C) than do control cells. No differences between the FMF and control cells were observed in the release of acid phosphatase, β‐glucuronidase, or lactoferrin. Colchicine treatment had no effect on the measurable release of the enzyme from PMNs derived from FMF patients. The increased release of lysozyme in response to high temperatures appears to be specific to FMF neutrophils, and was not found in PMNs from non‐FMF patients with febrile or inflammatory diseases, nor was it seen in monocytes derived from the FMF patients. It is suggested that the increased release of lysozyme from the neutrophils may be of importance in the pathogenesis of FMF.
Auer rod positive dysmyelopoietic syndrome
Authors: Weisdorf Daniel J. , Oken Martin M. , Johnson Gerhard J. , Rydell Robert E. 
Published: February 2007

AbstractTwenty‐five patients with refractory anemia with excess blasts (RAEB) were studied. Five of these patients showed Auer rods in their myeloblasts, but met other criteria for RAEB. Median survival of the Auer rod‐positive group was 14 months (range 2–27) from diagnosis with survival of 7 months after Auer rods were first observed. Median survival for the Auer rod‐negative group was 12 months. Two patients developed overt acute leukemia, both from the Auer rod‐negative group. The clinical course of Auer rod‐positive RAEB, like that of Auer rod‐negative RAEB, was one of progressive bone marrow failure complicated by infection, serious bleeding and the development of absolute transfusion requirement. These findings suggest that Auer rod‐positive RAEB is a morphologic variant dysmyelopoietic syndrome that may pursue a similar clinical course to Auer rod‐negative disease. Formulation of a separate treatment approach for those RAEB patients who possess Auer rods would appear ill advised.
Intrasplenic pH in normal and phenylhydrazine‐induced anemic rats
Authors: Chen L. T. , Chiang P. K. 
Published: February 2007

AbstractDirect measurement of the intrasplenic pH with a microcombination glass pH probe was performed in rats. The pH probe was inserted into the splenic pulp during measurements. In the normal rats, the pH was 7.21. However, in rats with hemolytic anemia induced by phenylhydrazine treatment, the pH decreased to 7.01. The level of lactic acid in the spleen was also determined. The lactic acid content in the spleen of anemic rats was found to be higher (1.20–1.44 μmoles/gm spleen) than that of normal rats (0.70–0.74 μmoles/gm spleen). The results suggest that the lower pH in the spleens of anemic rats may result from an increase in the lactic acid level in the spleen.
Methods of processing marrow samples may affect the frequency of detectable aneuploid cells
Authors: Waghray Manjula , Eques Celida , Rowley Janet D. , Martin Paulette , Testa Joseph R. 
Published: February 2007

AbstractKaryotypes were analyzed from 14 patients with various myeloproliferative disorders who had marrow samples processed directly and cultured in various ways. Eleven patients had acute nonlymphocytic leukemia (ANLL) (seven patients) or a dysmyelopoietic syndrome (four patients). All of these patients were known to have an abnormal karyotype, and in each case, two samples of a bone marrow aspirate were available: one processed directly and another cultured for 24 hr. Five of the 11 patients had essentially the same proportion of abnormal cells in the direct and 24‐hr samples; in five other patients, the percentage of aneuploid cells in the sample cultured for 24 hr was higher than that in the direct preparation. A higher percentage of aneuploid cells was observed in the direct preparation in only one case.In three other cases of ANLL, a marrow aspirate was cultured with methotrexate in addition to samples processed directly and after 24‐hr culture; only two of these had an abnormal clone in any sample. The results of karyotype analysis differed in these two patients. The proportion of aneuploid cells was substantially higher in the methotrexate culture than in the 24‐hr culture in one patient; in the other patinet, the 24‐hr culture contained aneuploid cells, whereas the methotrexate culture showed none.Three of the 13 aneuploid patients would have been incorrectly classified as being karyotypically normal on the basis of the initial analysis of the direct preparation, since only a single abnormal cell was detected in each case.The karyotypic pattern in untreated patients with ANLL has prognostic significance; therefore, the method of processing marrow aspirates may substantially influence the degree of correlation between the karyotype and survival reported by different laboratories.
Plasma fucosyltransferase as an indicator of imminent blastic crisis
Authors: Shah‐Reddy Ila , Kessel David H. , Chou Ta‐Hsu , Mirchandani Ila , Khilanani Urmilla 
Published: February 2007

AbstractPlasma fucosyltransferase activity was evaluated as an indicator of an impending blastic transformation in 25 patients with chronic granulocytic leukemia (CGL). Fifteen age‐and sex‐matched controls were also studied. The level of enzyme activity was significantly higher in the plasma of patients with blastic transformation (1,630 ± 570 units) compared with steady state chronic granulocytic leukemia (509 ± 110 units) and normal controls (354 ± 57 units). In three patients with CGL, a rise in fucosyltransferase activity preceded any other clinical or laboratory parameter of blastic transformation by 16–20 weeks.
Human leukocyte interferon‐mediated granulopoietic differentiation arrest and its abrogation by lithium carbonate
Authors: Verma Dharmvir S. , Spitzer Gary , Gutterman Jordan U. , Beran Miloslav , Zander Axel R. , McCredie Kenneth B. 
Published: February 2007

AbstractInterferon has been shown to inhibit erythropoietic and granulopoietic differentiation. Since lithium carbonate (Li) elevates granulocyte levels in a variety of neutropenic disorders, we investigated the effect of Li on human leukocyte interferon (HLIF)‐mediated inhibition of granulopoietic differentiation. Using an agar culture technique for cloning granulocyte‐macrophage progenitor cells (GM‐CFC), we demonstrated that Li blocks HLIF‐induced granulopoietic differentiation arrest in a dose‐dependent manner. Results of removal of T lymphocytes from marrow cells suggest that this Li effect is not mediated through marrow T lymphocytes.
A study of a female with congenital sideroblastic anemia
Authors: Manabe Yutaka , Seto Shiro , Furusho Kenshi , Aoki Yosuke 
Published: February 2007

AbstractA female infant with congenital refractory sideroblastic anemia is described. A market reduction of δ‐aminolevulinic acid (ALA) synthetase activity of erythroblasts was noticed with and without treatment of pyridoxal phosphate. Mitochondrial neutral protease activity of erythroblasts, which inactivates specifically the apo form of ALA synthetase, was normal and the sensitivity of apo form of ALA synthetase to the neutral protease was also normal. It was speculated that the reduction of ALA synthetase activity is not due to the high speed of destruction, but rather due to the impairment in production of ALA synthetase, which could explain the unresponsiveness to pyridoxine therapy in this case.
Burkitt cell leukemia following therapy for hodgkin disease
Authors: Nassar Victor H. , Jacobs Julian , Mirra Suzanne S. , Pandya Kishan J. , Schwartz Sheldon , Bennett John M. 
Published: February 2007

AbstractTwo adults with advanced Hodgkin disease — one treated with combination chemotherapy, and one with chemotherapy and radiation therapy — developed Burkitt cell leukemia four and seven years after diagnosis, proved by cytochemical and ultrastructural study. Acute lymphocytic leukemia must be considered in the evaluation of therapy‐related leukemias.
Recovery of splenic function after gvhd‐associated functional asplenia
Authors: Demetrakopoulos George E. V. , Tsokos George C. , Levine Arthur S. 
Published: February 2007

AbstractAn adolescent male patient developed functional asplenia in the course of graft versus host disease (GVHD) after successful allogeneic bone marrow transplantation (BMT) for aplastic anemia. Coincident with the onset of the asplenia, amelioration of the ongoing GVHD was observed. Unexpectedly, after 34 months of functional asplenia, the organ regained its function without evidence of graft rejection or reactivation of GVHD.
Thalassemia: Recent insights into molecular mechanisms
Authors: Steinberg Martin H. , Adams Junius G. 
Published: February 2007

AbstractRecent advances in defining the molecular basis for the thalassemia syndromes are discussed. We now realize that the causes of the thalassemia phenotype are diverse and include gene deletions, nuclear RNA processing defects, nonsense mutations, fusion genes, termination codon mutants, and unstable globin chains.
A human T‐cell antigen defined by xenoantiserum to sézary leukemic cells: Immunochemical and functional studies
Authors: Ogasawara Minoru , Ishii Yoshifumi , Kamiya Hirofumi , Kikuchi Kokichi 
Published: February 2007

AbstractXenoantiserum to Sézary cell leukemia cells (ASS) was developed by immunizing rabbits with those cells and was absorbed with human red cells, liver, tonsil B cells, and cultured Raji cells. This reagent reacted by immunofluorescence with virtually all human thymus and T cells. In the thymus, medullary cells reacted more strongly with ASS than did cortical thymocytes. When immunoprecipitates that formed between ASS and 125I‐labeled lymphocyte surface glycoproteins were analyzed by sodium dodecyl sulfate‐polyacrylamide gel electrophoresis, it was found that ASS precipitated a 72K molecular weight (MW) glycoprotein from T cells but not from B cells. On the one hand, it was shown by functional studies that T cells sensitive to the cytotoxic effect of ASS contained T cells that could aid the immunoglobulin synthesis of B cells induced by pokeweed mitogen. On the other hand, suppressor T cells induced by concanavalin A resided in those cells rather resistant to its cytotoxic effect. These data support the idea that the 72K MW glycoprotein on human thymus and T cells might be homologous to mouse Lyt‐1 antigens.