bluuebunny/crossref_metadata_2025_split huggingface dataset:
Determination of glycosylated hemoglobins in neonatal blood by isoelectric focusing
Authors: Fitzgerald M. D. , Cauchi M. N. 
Published: February 2007

AbstractIsoelectric focusing (IEF) with suitable „spacers”︁ can be used to distinguish glycosylated hemoglobins from HbA and HbF in adults and infants, respectively. Using this technique we have shown that there is a good correlation between glycosylated hemoglobins measured by IEF and column chromatography (r = 0.778, P < 0.001). There was no significant difference in the rate of glycosylation between adult and cord blood hemolysates following incubation with glucose in vitro: In both instances there was an increase from the normal levels of about 5% to 26% through 31% after 18 hours of incubation. The levels of glycosylated hemoglobins in infants of varying gestation, neonates, and normal adults was remarkably constant, varying from 4.7 ± 1.1% to 6.1 ± 1.6%.These results show that IEF can be useful in the quantitation of glycosylated hemoglobins, particularly in the presence of hemoglobins (eg, HbF) where standard chromatographic techniques cannot be used. IEF has the added advantage of using small quantities of blood, and allows a number of samples to be tested at the same time, thus facilitating the identification of abnormal hemoglobins.
Childhood CML in blastic stage: An analysis of cell markers and cell kinetics
Authors: Beck J. D. , Andreeff M. , Mertelsmann R. , Haghbin M. , Tan C. , Miller D. R. , Good R. A. , Gupta S. 
Published: February 2007

AbstractThe clinical course of Ph+ CML and their terminal blastic stage is described in the following case histories. During blastic phase, cell surface markers, terminal deoxynucleotidyl transferase (TdT) activity, and flow cytometric measurements were used for determination of the blast cell phenotype which was undifferentiated by morphological and cytochemical criteria. A high proportion of blast cells expressed Fc receptors for IgG, TdT activity was normal in both children, and RNA measurements of single cells by flow cytometry showed a high RNA content in the majority of blasts. These findings are compatible with the phenotype seen in myelomonocytic leukemias. Flow cytometry appears to be a useful adjunct to immunological methods and TdT activity for the rapid characterization of CML in blastic phase.
A clinicopathologic analysis of chronic lymphocytic leukemia
Authors: van Scoy‐Mosher Michael B. , Bick Miriam , Capostagno Vince , Walford Roy L. , Gatti Richard A. 
Published: February 2007

AbstractSixty consecutive patients with chronic lymphocytic leukemia (CLL) were studied from both a clinical and laboratory standpoint. Hypoimmunoglobulinemia was found in 45% of patients; many of these patients suffered from severe bacterial infections. Second primary malignancies were diagnosed in 20% of patient's; most of these predated the diagnosis of CLL. HLA‐B17 typing was found in unexpectedly high frequency in a small group of Coombs'‐positive patients. The Rai‐staging system was found to be generally useful for determining prognosis of groups of patients, although less useful in any individual patient. Findings relating to surface membrane immunoglobulin‐positive (B) and E‐rosetting (T) lymphocytes are described. A patient with null‐cell CLL is described. All patients with proliferation of gamma heavy‐chain‐bearing cells were diagnosed in early Rai stages suggesting that this marker may identify a subset of patients who present early and have a good prognosis. These data suggest that lymphocyte marker studies augment the Rai criteria in evaluating prognosis and may eventually be of value to the clinician in evaluating stage of disease and response to treatment.
The immunological and cytochemical characterization of T‐cell lymphoproliferative diseases
Authors: Spira T. J. , Ades E. W. , Gordon D. S. 
Published: February 2007

AbstractSurface markers, terminal transferase, cytochemical staining, and function of peripheral blood lymphocytes were examined in nine patients with T‐cell lymphoproliferative disease. Anti‐T‐cell antiserum proved useful in characterizing the phenotype of the abnormal cells when only a minority of the abnormal cells formed E‐rosettes. Anti‐B‐cell antiserum was consistently unreactive with the abnormal cells. Terminal transferase was elevated in T‐cell ALL but low in T‐cell CLL, lymphoma‐leukemia, or Sézary syndrome. Lymphocyte proliferative response to nonspecific mitogens was generally depressed as was the response to allogeneic cells. The cells also failed to stimulate in mixed lymphocyte culture, implying a lack of Ia antigens on their surface. Acid phosphatase stain was positive in the three patients tested. Peroxidase and esterase stains were negative, whereas PAS was positive in five of eight patients tested. Prognosis in general, regardless of the clinical picture, (ie, acute, chronic, or lymphoma‐leukemia), was poor with seven of the nine patients dying within 14 months of diagnosis. Therefore, the use of anti‐T‐cell antisera and the E‐rosette surface marker should be used to characterize the phenotype of the malignant cells in lymphoproliferative disease to enable more vigorous treatment of those that are of T‐cell origin and that therefore carry a poor prognosis.
Late relapses in hodgkin disease
Authors: Kanofsky Jeffrey R. , Golomb Harvey M. , Vardiman James E. , Sweet Donald L. , Ultmann John E. 
Published: February 2007

AbstractAmong a cohort of 142 patients with Hodgkin disease (HD), pathologic stages (PS) IA through IVB, 84 remained in complete remission (CR) at least 3 years after the completion of initial protocol therapy. Eight of these patients subsequently developed a recurrence of HD and were defined as having a late relapse. The disease‐free interval ranged from 37 to 76 months (median 53 months). Six of the relapse patterns were those of dissemination. All patients were salvaged and are in CR from 14 to 50 months after relapse. The actuarial survival, measured from the time of relapse, of the eight late‐relapsing patients was superior to the actuarial survival of 38 patients with early relapse (disease‐free interval less than 36 months), but as yet this difference is not statistically significant (P = 0.07, Gehan).
Effects of dexamethasone on fetal hemoglobin synthesis in peripheral blood erythroid burst‐forming units
Authors: Steinberg Martin H. , Coleman Mary B. , Garver Fred A. , Grenett Hernan E. , Pressley Annette , Adams Junius G. 
Published: February 2007

AbstractColonies derived from erythroid burst‐forming units (BFU‐E) synthesize fetal hemoglobin (HbF) in amounts that far exceed in vivo levels. There is some evidence that HbF synthesis is controlled at the level of a primitive erythroid precursor cell. Dexamethasone may potentiate the development of BFU‐E. Since a means of augmenting HbF production in sickle cell anemia or severe β‐thalassemia would be of great therapeutic value, we studied the effects of dexamethasone on HbF and γ‐globin chain synthesis in BFU‐E from patients with sickle cell anemia and controls. HbF was measured by radioimmunoassay of BFU‐E lysate and γ‐chain synthesis by the incorporation of 3H‐leucine into globin, which was then purified by gel filtration and column chromatography. Dexamethasone (10−9 M) produced an increase in the number of BFU‐E in 16 of 19 subjects when compared with numbers of BFU‐E cultured with only erythropoietin. The individual BFU‐E were larger and contained more subcolonies. Dexamethasone did not increase HbF or γ‐chain synthesis, and there was no relationship between increased proliferation of BFU‐E and augmented HbF production. Thus, although dexamethasone augmented the development of erythroid bursts, there was no increment in HbF.
Human thymus antigen: Characterization and its expression on human leukemias
Authors: Fujimoto Junichiro , Ishii Yoshifumi , Koshiba Hirofumi , Matsuura Akihiro , Ogasawara Minoru , Uede Toshimitsu , Kikuchi Kokichi 
Published: February 2007

AbstractSpecific anti‐human thymus xenoantiserum (ATS) was utilized for characterizing a human thymus antigen (HTA) preferentially expressed on human thymocytes. Binding of ATS with different cell types was studied by immunofluorescence and immunoperoxidase techniques, as well as by radioimmunoprecipitation (RIP) followed by SDS‐polyacrylamide gel electrophoresis (SDS‐PAGE). ATS reacted selectively with the majority (87%) of human thymocytes among normal lymphoid cell populations tested. In the thymus, HTA was expressed largely on cortical thymocytes but not on those cells located in the thymic medulla. When 20 cases of different types of lymphatic leukemias were studied with ATS, HTA was found on acute lymphoblastic leukemia cells rosetting with sheep erythrocytes (T‐ALL) but not on other lymphatic leukemia cases, including chronic lymphocytic leukemia cells rosetting with sheep red blood cells (T‐CLL). SDS‐PAGE analysis of immunoprecipitates formed between ATS and radiolabeled cell‐surface components of human thymocytes demonstrated that ATS bound a 48,000‐molecular‐weight component that could be adsorbed to Sepharose 4B coupled with Lens culinaris hemagglutinin and could be labeled by periodate‐tritiated borohydride, indicating that HTA is a sialoglycoprotein. Similar antigen molecules were also precipitated by ATS from T‐ALL cells but not from other lymphatic leukemia cells. Five T‐ALL‐derived cell lines were tested with ATS by immunofluorescence and by RIP and SDS‐PAGE, but only two cell lines (MOLT‐3 and P12/Ich) possessed HTA molecules on their cell membrane.
A defect in the formation of uropod‐bearing lymphocytes (hand‐mirror cells) in patients with the wiskott‐aldrich syndrome
Authors: McGraw Thomas P. , Folds James D. , Whisnant John K. , Phillips Terry M. , Stass Sanford A. 
Published: February 2007

AbstractThe formation of a uropod by lymphocytes (hand‐mirror cells) represents a morphologic stage of immune activation and motility in lymphocytes. Immune complexes have been previously shown to induce hand‐mirror‐cell (HMC) formation in human lymphocytes and have been associated with increased number of HMC in acute leukemia. We studied the ability of immune complexes to induce HMC in patients with Wiskott‐Aldrich syndrome (WAS), a disease of cellular immune deficiency. Our findings indicate that lymphocytes from patients with WAS adhere to immune complexes but have a defect in their ability to form HMC when compared with normal human lymphocytes. These results support the concept that immune complexes induce HMC formation and that the HMC is related to the normal cellular immune response.
In vitro synthesis of hemoglobin and hemoglobin chains in the BFUe‐derived colonies from persons with α‐ or β‐thalassemia
Authors: Huisman T. H. J. , Reese A. L. , Webber B. , Okonjo K. , Altay C. , Felice A. E. 
Published: February 2007

AbstractThe synthesis of α and non‐α chains of human hemoglobin (Hb) was studied in reticulocytes and in BFUe‐derived cell colonies from patients with α chain or β chain deficiencies. The subjects included normal adults (αα/αα) with or without a β chain variant (Hb S, Hb Leslie) or an α chain variant (Hb G‐Georgia); α‐thalassemia‐2 heterozygotes (α0α/αα) with an α chain variant (G‐Georgia or G‐Philadelphia); an α‐thalassemia‐1 heterozygote (α0α0/αα); α‐thalassemia‐2 homozygotes (α0α/α0α) with a β chain variant (Hb S), an α chain variant (G‐Philadelphia), a Hb S homozygosity with Hb G‐Philadelphia, or a Hb G‐Philadelphia homozygosity; and three black β+ ‐thalassemia homozygotes. Data from reticulocyte in vitro synthesis analyses showed the expected deficiencies. However, similar analyses of the Hb synthesized in cell colonies (even from the black β‐thalassemia homozygotes) gave (nearly) balanced ∑α/∑non‐α ratios. It is speculated that this balanced synthesis is due to a most effective proteolysis in the immature erythroblast which rapidly removes free α or β chains. The levels of Hb F and Hb A2 were considerably increased in these proerythroblasts; a two‐ to threefold increase in the synthesis of Hb A2 was observed over that seen in the reticulocytes.
Aspects of platelet formation and release
Authors: Scurfield G. , Radley J. M. 
Published: February 2007

AbstractThe surface architecture and location of megakaryocytes in the extravascular compartment of mouse bone marrow are considered. The finger‐like cytoplasmic processes that extend into sinuses pass through apertures, approximately 3.0 mμ in diameter, occurring singly or in groups. The cytoplasmic processes enlarge on first entering a sinus and form villi that seem to anchor them to the endothelium. The villi consist largely of microfilaments. The cytoplasmic processes extend, attenuate, and undergo irregular constriction along their lengths. The constrictions demarcate modicums of cytoplasm of platelet size one from another that are released when they break. Extravascular platelet release may also occur, and evidence is presented that points to the likelihood of the platelet's being engulfed and phagocytosed by macrophages before reaching the microcirculation.
Priapism in a non‐black with sickle cell anemia associated with α‐thalassemia
Authors: Fich Alexander , Rachmilewitz Eliezer A. 
Published: February 2007

AbstractSickle cell anemia, the most prevalent type of hemoglobinopathy, appears almost exclusively in blacks. Since the first report of sickle cell anemia in a Caucasian by Cooley and Lee in 1929 [1], additional cases have been reported, mainly from Mediterranean countries, the Persian Gulf, and India [2]. Several cases were also found among Arabs in Israel with a relatively benign clinical course [3, 4].The present report describes a unique case of sickle cell anemia associated with α‐thalassemia in a non‐black young adult. The diagnosis was made at the age of 23 years. Severe priapism was almost the first manifestation of the disease and subsided following transfusion of five units of packed red blood cells.
Cellular kinetics in a patient with sezary syndrome
Authors: Chandra P. , Chanana A. D. , Chikkappa G. , Cronkite E. P. 
Published: February 2007

AbstractCellular kinetics and proliferation of Sezary cells (SC) were studied in a 48‐year‐old woman with Sezary syndrome (SS). In vitro flash labeling indices of peripheral blood (PB) SC were studied by labeling with tritiated thymidine (3HTdR) and tritiated cytidine (3HCdR). Intradermal SC were labeled in vivo by local injection of 3HTdR followed by skin biopsies of the injected sites. Traffic patterns of DNA labeled PB SC were studied by intravenous 3HTdR followed by sampling of PB, skin, lymph node (LN), and bone marrow (BM). Fluxes of PB SC in various tissues were investigated by autotransfusion of 3HCdR labeled PB SC followed by serial sampling of PB, LN, BM, and skin. In vitro response of PB SC to phytohemagglutinin (PHA), concanavalin A (Con A), and pokeweed (PWM) were also investigated. The results from these studies in this patient indicate that 1) proliferation of SC was primarily in the skin, 2) there was a negligible flux of SC from blood into skin, LN, and BM, and 3) the mitogenic response of PB mononuclear cells (mostly SC) to PHA, PWM, and Con A was poor.
Nonimmune interaction of leukocytes with platelets and megakaryocytes
Authors: Poon Man‐Chiu , Parmley Richard T. , Chang‐Poon Vivien Y‐H , Embry Joseph H. , Austin Ronald L. 
Published: February 2007

AbstractPlatelet–leukocyte interaction was observed in an asymptomatic woman. After incubation in the patient's EDTA‐plasma, autologous and allogeneic platelets adhered to the surfaces of neutrophils, monocytes, macrophages and, rarely, eosinophils. Monocytes, macrophages, and occasionally neutrophils phagocytosed platelets. Degranulation of peroxidase‐positive lysosomes into the platelet‐containing phagosome was demonstrated ultrastructurally. Bone marrow studies indicated that bands and earlier neutrophilic precursors did not participate in the reaction, and that neutrophils adhered to, and were rarely engulfed by megakaryocytes.Sequential exposure of the patient's EDTA‐plasma to platelets and leukocytes indicated that a nondialyzable factor(s) was first absorbed by platelets which then interacted with leukocytes. The reaction proceeded best in the presence of EDTA at 21°C, and was inhibited or dissociated by divalent cations or at 37°C. Metabolic integrity of both platelets and leukocytes was also essential for the reaction since each was inhibited by formalin fixation and partially inhibited by the metabolic inhibitor 2‐deoxyglucose. Formalin‐treated platelets continued to absorb the plasma factor(s). The plasma and the cell fractions were inactivated by periodate and nonspecific protease. Treatment of the platelets with trypsin or the leukocytes with neuraminidase diminished the interaction by 50%. The reaction was also interfered with by concanavalin A. Immunofluorescent and immunoabsorption studies failed to identify an immune component of this interaction. These findings indicate that the plasma factor(s) and the cell surface receptors are nonimmune glycoconjugates and consequently differ from previously documented cases of platelet‐leukocyte interaction.
Clinical use of β‐thromboglobulin levels in diagnosing and treating consumptive and immune thrombocytopenia
Authors: Rubenstein Martin D. , Wall Robert T. 
Published: February 2007

AbstractPlasma β‐thromboglobulin (β‐TG), a platelet‐specific protein, is a marker of intravascular platelet degranulation. We measured plasma β‐thromboglobulin by radioimmunoassay in 13 patients with thrombocytopenia of various etiologies to determine whether or not the test is clinically useful in the differential diagnosis of thrombocytopenia. Four patients with intravascular platelet consumption (three with thrombotic thrombocytopenic purpura and one with vasculitis) had significantly higher plasma β‐thromboglobulin levels than four patients with extravascular platelet destruction due to idiopathic thrombocytopenic purpura. Five patients with thrombocytopenia and decreased numbers of megakaryocytes in the bone marrow also had β‐thromboglobulin levels that were not elevated. Two patients with thrombotic thrombocytopenic purpura achieved clinical remission associated with a decrease in β‐TG level to the normal range. Plasma β‐thromboglobulin determination can be useful in determining the mechanism of thrombocytopenia when bone marrow examination demonstrates adequate megakaryocyte numbers.
Acute lymphocytic leukemic transformation of chronic lymphocytic leukemia: Substantiation by flow cytometry
Authors: Frenkel Eugene P. , Ligler Frances S. , Graham Mateel S. , Hernandez Jose A. , Kettman John R. , Smith R. Graham 
Published: February 2007

AbstractAcute leukemia, during the course of chronic lymphocytic leukemia, is a rare event and only a small fraction of such cases have been shown to be true transformations to acute lymphocytic leukemia. In one previous case, evidence suggested this was transformation of a single neoplastic clone. The present report describes the clinical and laboratory findings in a case of chronic lymphocytic leukemia in which an acute leukemic pattern evolved. The fluorescence‐activated cell sorter provided the resolution needed to distinguish low numbers of surface immunoglobulins on the acute leukemic cells, verifying their identity as B lymphoblasts. Furthermore, a small population of large cells bearing the same light chain type as the acute leukemic cells were detected in the spleen 3½ months before clinical or laboratory evidence of the onset of the acute leukemia.
A case of lead intoxication: Clinical and biochemical studies
Authors: Miwa Shiro , Ishida Yoji , Takegawa Susumu , Urata Gumpei , Toyoda Toshio 
Published: February 2007

AbstractA 23‐year‐old Japanese male with severe lead intoxication accompanied by hemolytic anemia was studied. The patient had taken 12 g of lead in about a month. He had moderate hemolytic anemia (Hb 8.9 g/100 ml) with reticulo‐cytosis ranging from 2.5 to 11.7%. Peripheral blood smear showed nucleated red cells (42/200 white blood cells) and marked basophilic stippling in the red cells. Activities of erythrocyte enzymes were either normal or increased except for pyrimidine 5′‐nucleotidase (P5N) and δ‐aminolevulinic acid dehydratase (ALA‐D) both of which were found to be decreased, being 48.8% and 4.1% of the normal controls respectively. Erythrocyte reduced glutathione (GSH) was high (145.6 mg/100ml RBC). Erythrocyte pyrimidine nucleotides were accumulated up to 10.2% of total nucleotides. The level of lead in peripheral blood was 112 μg per 100 ml blood.Ca2+‐Na2‐EDTA was given to the patient as treatment. The level of lead in the blood decreased gradually and hemolytic anemia improved. Basophilic stippling in the red cells disappeared.These results confirmed the findings of Valentine et al [1] that lead‐induced deficiency of P5N resulted in basophilic stippling and hemolytic anemia just like hereditary hemolytic anemia due to P5N deficiency.
Effect of chemical modification of sulfhydryl groups of human erythrocyte enzymes
Authors: Valentine W. N. , Paglia D. E. 
Published: February 2007

AbstractErythrocyte lysate proteins prepared by five methods were chemically modified by methanethiolation with methyl methanethiolsulfonate (MMTS). This relatively newly studied agent delivers the small uncharged, non‐hydrogen‐bonding CH3S‐group to accessible ‐SH groups under mild conditions. Glycolytic and nonglycolytic red cell enzymes studied could be grouped into four categories based on degree of catalytic inhibition by MMTS, which ranged from 100% to nil. NADH (0.06 mM) protected against lactate dehydrogenase (LDH) inhibition nearly completely, and partial protection was evident at 1 μM concentrations. NAD was virtually ineffective at comparable molarities, and pyruvate failed to protect significantly. Glucose‐6‐phosphate dehydrogenase (G‐6‐PD) (but not 6‐PGD) was protected by NADP (but minimally if at all by NADPH) at low molarities. Substrate G‐6‐P failed to protect. Mg++ alone protected enolase to a major degree against MMTS inhibition. Residual activities of several enzymes were rendered highly unstable to thermal stress, and in the case of selected enzymes, alteration of pH activity curves and catalytic activity at diminished substrate concentration was demonstrable. The highly specific modification of sulfhydryl groups of cysteinyl residues under mild conditions preserving other aspects of enzyme structure provides insights into the role of these most reactive of all amino side‐chains in the enzyme proteins derived from human red cell lysates.