bluuebunny/crossref_metadata_2025_split huggingface dataset:
Correlation of serum IgD level with clinical and histologic parameters in Hodgkin disease
Authors: Corte G , Ferraris AM , Rees JK , Bargellesi A , Hayhoe FG 
Published: October 2019

Abstract
The concentration of serum IgD was measured in 100 patients with Hodgkin disease; 65 had 3--50-fold increases in IgD levels. Several parameters were found to influence serum IgD concentration: IgD level in older patients (greater than 50 yr) was significantly lower than in the younger patients (P less than 18 yr). IgD concentration was significantly higher in splenectomized than in nonsplenectomized patients (p less than 0.005). Therapy was found to depress IgD concentration, which fell to a value significantly lower than in untreated patients (p less than 0.05). An interesting correlation was found between IgD levels and histologic type of the disease, lower levels being preferentially associated with the lymphocyte predominance type and higher with the lymphocyte dipletion type. The logarithms of the means of these two groups were significantly different from the overall mean of the disease (p less than 0.05 and p less than 0.0005, respectively). Mixed cellularity and nodular sclerosing showed intermediate values.
Hereditary dyserythropoiesis with abnormal membrane folate transport
Authors: Howe RB , Branda RF , Douglas SD , Brunning RD 
Published: October 2019

Abstract
Dyserythropoiesis, which morphologically and serologically resembles congenital dyserythropoietic anemia type III but is not accompanied by anemia, is described in a young man. In addition to striking gigantism and multinuclearity of erythroid precursors, electron microscopy revealed widening of nuclear pores, nuclear clefts, and cytoplasmic inclusions. Membrane transport of 5-methyltetrahydrofolate by the patient's red cells was markedly reduced; total uptake, uptake velocity, and maximal velocity of uptake were all significantly less than in controls. In contrast, red cell uptake of pteroylglutamic acid was normal. Bone marrow cells in culture also showed decreased 5- methyltetrahydrofolate uptake, as well as very low thymidine incorporation. Because folate uptake by mitogen-stimulated lymphocytes was normal, the defect apparently does not involve all cell lines. These results suggest that a specific membrane defect, affecting the carrier system for reduced folate compounds, is present in this patient's erythrocytes, and perhaps, their bone marrow precursors.
Mucosal iron transport by rat intestine
Authors: Savin MA , Cook JD 
Published: October 2019

Abstract
Using highly sensitive 2-site immunoradiometric assays, we examined the relationship between iron absorption from closed intestinal loops and transferrin and ferritin concentrations in isolated duodenal mucosal cells. As in prior studies, mucosal ferritin correlates inversely with iron absorption and directly with body iron stores as measured by the concentration of nonheme iron in liver. Mucosal transferrin, on the other hand, varies directly with both the total mucosal uptake of radioiron and the proportion of this radioiron transferred from the mucosa to the carcass. The highest correlation with iron absorption was observed with the transferrin-ferritin ratio in isolated mucosal cells. These results suggest that there are two functionally distinct iron- binding compartments in the duodenal mucosa. One is a strong compartment, ferritin, and the other is a transport compartment, transferrin. Control of iron absorption by the intestinal mucosa is closely tied to the balance between these two intracellular iron compartments.
The mouse pale ear pigment mutant as a possible animal model for human platelet storage pool deficiency
Authors: Novak EK , Hui SW , Swank RT 
Published: October 2019

Abstract
The mouse pigment mutant pale ear, ep/ep, which has a defect in kidney lysosomal enzyme secretion, had prolonged bleeding on experimental injury. Platelet counts and platelet protein did not differ from normal. There was, however, a deficiency in the platelet dense granule contents, serotonin, ATP, and ADP. Furthermore, a marked reduction of platelet dense granules was observed by electron microscopy. The results suggest that pale ear is a useful animal model in the study of platelet storage pool disease. Studies on this mutant and other pigment mutants have established that one gene can regulate at least three subcellular organelles, including the melanosome, the lysosome, and the platelet dense granule.
Enhancement of iron chelation by desferrioxamine entrapped in red blood cell ghosts
Authors: Green R , Miller J , Crosby W 
Published: October 2019

Abstract
We have exploited the physiologic mechanism for removal of red cells from the circulation to target the iron chelator desferrioxamine to reticuloendothelial iron stores. Compared with free desferrioxamine injected intravenously, the same dose of desferrioxamine entrapped in resealed red blood cell ghosts resulted in a fourfold to fivefold increase in excretion of radioiron in rats with a selective 59Fe radiolabel of reticuloendothelial iron stores. Desferrioxamine in red cell ghosts did not enhance excretion in rats with selective radiolabeling of parenchymal iron stores. In rats with uniformly radiolabeled iron stores, desferrioxamine in red cell ghosts produced an eightfold to ninefold greater loss of iron in the urine free desferrioxamine intravenously or by slow subcutaneous infusion. Desferrioxamine in red cell ghosts resulted in significantly greater fecal excretion of iron than intravenous desferrioxamine, but desferrioxamine in red cell ghosts and subcutaneous desferrioxamine infusion resulted in similar fecal iron excretion. Clinical application of the red cell ghost method for administration of desferrioxamine and other iron chelators may ber useful for improvement of iron chelation efficiency.
Malignant monoblasts can function as effector cells in natural killer cell and antibody-dependent cellular cytotoxicity assays
Authors: Hokland P , Hokland M , Ellegaard J 
Published: October 2019

Abstract
This is the first report describing natural killer (NK) and antibody- dependent cellular cytotoxicity (ADCC) of malignant monoblasts. Pure acute monoblastic leukemia was diagnosed in bone marrow aspirations from two patients by use of conventional cytochemical methods as well as multiple immunologic techniques including detection of ALL antigens and terminal transferase. The malignant cells were subsequently found to be potent effectors in NK and ADCC assays. Addition of partially purified alpha-interferon to the in vitro cultures was found to have an enhancing effect on NK activity, whereas no modulation was seen in ADCC. These findings are discussed in the light of our present knowledge of lymphoid NK cells.
Acquired von Willebrand's disease in association with Wilm's tumor: regression following treatment
Authors: Scott JP , Montgomery RR , Tubergen DG , Hays T 
Published: October 2019

Abstract
A 9-yr-old female presented with a Wilm's tumor and a coagulopathy consistent with von Willebrand's disease. Factor VIII procoagulant activity (VIII C), factor VIII related antigen (VIIIR:Ag), and von Willebrand factor activity (VIII:vWf) were decreased. There was no evidence for a circulating inhibitor of the factor VIII molecular complex. von Willebrand's antigen II (vW AgII), which is deficient in hereditary von Willebrand's disease, was decreased below detectable levels in this patient. The coagulation studies, VIIIR:Ag, and vW AgII levels returned to normal following therapy of the Wilm's tumor. Wilm's tumor must be included as one of the malignancies associated with acquired von Willebrand's disease. Immunofluorescent studies of the tumor specimen showed normal endothelial staining of VIIIR:Ag by semiquantitative techniques and a lack of specific tumor adsorption of VIIIR:Ag The presence of normal amounts of tissue VIIIR:Ag has not previously been demonstrated in acquired von Willebrand's disease. Since we failed to demonstrate an inhibitor in the plasma in this patient, the etiology of the acquired von Willebrand's disease in this patient appears to differ from other cases of acquired von Willebrand's disease. The finding that vW AgII is decreased in this patient, similar to that reported in hereditary von Willebrand's disease, supports the close association of vW AgII to VIIIR:Ag, even though they are immunologically and biochemically distinct.
Human trophoblasts: cellular source of colony-stimulating activity in placental tissue
Authors: Ruscetti FW , Chou JY , Gallo RC 
Published: October 2019

Abstract
Colony-stimulating activity (CSA) is a class of protein factors that stimulates the in vitro growth and differentiation of hematopoietic committed stem cells into mature granulocytes and macrophages. In man, production of CSA is mediated by monocytes-macrophages, lymphocyte, and endothelial cells. One of the best studied and most potent sources of CSA is conditioned media derived from cultured human placenta. The cellular source of this placental CSA was studied using cloned term placental cell lines induced by a simian virus 40 (SV-40) Wild-Type (wt) or temperature sensitive (tsA) mutants. Conditioned media from SV- 40 wt-transformed placental cells grown at 37 degrees C and tsA- transformed placental cells grown at 33 degrees C (the temperature at which the cells exhibit the transformed phenotype) and at 40 degrees C (temperature at which the cells express a normal differentiated phenotype) contained high levels of CSA. At the restricted temperature (40 degrees C), these cell lines expressed normal trophoblastic characteristics in that they produced human chorionic gonadotropin, pregnancy-specific-beta1-glyco protein, and placental alkaline phosphatase, indicating that the SV-40 transformation has not interfered with normal cellular functions of these trophoblasts. The CSA released by these cell lines resemble those of CM from fresh placental cells, in that the level of activity is high and that formation of both granulocyte/macrophage and eosinophil colonies is stimulated.
Hb Bart's level in cord blood and deletions of alpha-globin genes
Authors: Lie-Injo LE , Solai A , Herrera AR , Nicolaisen L , Kan YW , Wan WP , Hasan K 
Published: October 2019

Abstract
The white blood cell DNA of 36 cord blood samples with Hb Bart's in the red blood cells was studied for alpha-globin gene deletions by hybridization of DNA fragments digested by the restriction endonucleases Eco RI, Hpa I, Bam HI, and Bgl II. All 16 DNA samples from cord blood with Hb Bart's below 3% and no other abnormal hemoglobin had one alpha-globin gene deletion (alpha thal2), except one which had two alpha-globin gene deletions (alpha thal1). Most of the alpha thal2 were of the rightward deletion alpha thal2 genotype. Two new types of alpha thal2 variation was found, probably due to a polymorphism somewhere in an area outside the alpha-globin gene. All 14 cases with Hb Bart's between 3.5% and 8.5% and no other abnormal hemoglobin had two alpha-globin gene deletions (alpha thal1), except one that did not have any alpha-globin gene deletion and one that had one alpha-globin gene deletion. Three DNA samples of cord blood with Hb Bart's accompanied by Hb CoSp did not have any alpha-globin gene deletion. Sixty-five DNA samples from cord blood without Hb Bart's or other abnormal hemoglobin had no alpha-globin gene deletions, except one that had one alpha-globin gene deletion (alpha thal2). Two of the 65 DNA samples were found to have triplicated alpha-globin gene loci.
Acute nonlymphocytic leukemia with basophilic differentiation
Authors: Wick MR , Li CY , Pierre RV 
Published: October 2019

Abstract
Four cases of acute nonlymphocytic leukemia with primitive basophilic differentiation are presented. In all four cases, study revealed Philadelphia chromosome negativity, and in none were there clinical findings of chronic granulocytic leukemia. In each case, the leukemic blasts contained granules that failed to stain for peroxidase content but stained positively with toluidine blue. The former result could have led to the misclassification of the cases as lymphoid leukemias. Three of the four patients had physical findings that may have been due to circulating histamine excess. The histochemical and clinical features of these cases suggest that certain examples of leukemia with basophilic differentiation represent a distinctive variant of acute nonlymphocytic leukemia.
Analysis of T-cell differentiation antigens in acute lymphatic leukemia using monoclonal antibodies
Authors: Koziner B , Gebhard D , Denny T , McKenzie S , Clarkson BD , Miller DA , Evans RL 
Published: October 2019

Abstract
Leukemic cells in 134 patients with ALL were analyzed by a panel of mouse monoclonal antibodies. Two antibodies are reactive with all peripheral blood T cells but define different surface antigens (Leu-1 and Leu-4). Two other antibodies react with antigens that are restricted to suppressor/cytotoxic T cells (Leu-2) and to helper T cells (Leu-3). We also used antibodies to the receptor for sheep red blood cells (SRBC) (Leu-5) and to a human “TL-like” antigen that is found on most thymocytes but not in peripheral T cells (Leu-6). An antibody to the human p29.34 “Ia-like” molecule was also tested. Of the 134 ALL patients, 17 had a predominance of SRBC-rosetting (Leu-5+) lymphoblasts (“T” ALL), expressing different surface phenotypes defined by this panel of monoclonal antibodies. These phenotypes were not readily classifiable according to a scheme of sequential stages of normal differentiation proposed. Moreover, the lymphoblasts in 8 of 113 patients not expressing conventional B- to T-cell markers (“null” ALL) reacted with the monoclonal anti-T-cell antibodies. This study suggests that the classification of lymphoblasts in ALL based on the reactivities observed with this panel of mouse monoclonal antibodies is not easily reconciled with current models of normal T-cell differentiation. However, it should be emphasized that the precise sequence of antigenic expression by cells undergoing thymic differentiation is still not fully known, and further phenotypic analysis of ALL cells might contribute to an improved understanding of this malignancy.
Activation of human factor VII by activated factors IX and X
Authors: Masys DR , Bajaj SP , Rapaport SI 
Published: October 2019

Abstract
Factor VII clotting activity increases about five-fold when blood is clotted in glass. Prior studies suggested that this results from activation induced by activated factor IX (IXa). However, in purified systems containing phospholipid and calcium, activated factor X (Xa) is known to activate factor VII rapidly. Therefore, we studied activation of factor VII by IXa and X, in systems using purified human factors. Concentrations of IXa and Xa were calculated from total activated protein concentrations rather than from active site concentrations. In the presence of phospolipid and calcium, both IXa and Xa activated factor VII 25-fold; however, Xa was roughly 800 times more efficient than IXa. Without added phospholipid, activation of factor VII by both Xa and IXa was markedly slowed, and Xa was roughly 20 times more efficient than IXa. When both phospholipid and calcium were omitted, activation of factor VII by either enzyme was negligible. Adding normal prothrombin, but not decarboxylated prothrombin, substantially slowed activation of factor VII by both Xa and IXa. Adding thrombin-activated factor VIII and antithrombin-III did not change rates of factor VII activation by either enzyme. These results from purified systems do not provide an explanation for the prior data from plasma systems.
Two new acute lymphoblastic leukemia cell lines with early B-cell phenotypes
Authors: Findley HW Jr , Cooper MD , Kim TH , Alvarado C , Ragab AH 
Published: October 2019

Abstract
Two leukemic cell lines (697 and 207) were established from bone marrow cells obtained from children with ALL in relapse. These cell lines were positive for the common-ALL antigen (CALLA), the HLA-DR (i.e., Ia-like) antigen, and for cytoplasmic and surface IgM heavy chains. The lines were negative for other immunoglobulin heavy chains and light chains. The lines had elevated levels of terminal deoxynucleotidyl transferase enzyme and expressed surface antigens found on normal myeloid- macrophage cells (MMA) and on natural killer cells (HNK-1). A minority of cells in line 207 expressed the T-1, T-6, and Leu-1 antigens as detected by monoclonal antibodies. Line 697 was positive for Epstein- Barr virus (EBV), while line 207 did not possess EBV. Line 697 carried a marker chromosome (identified as a translocation between chromosomes 7 and 19), which was also patient's fresh leukemic cells. The leukemic origin of the cell lines was further indicated by their morphological, cytochemical, and immunologic similarity to the patients' leukemic cells. Phenotypically, both cell lines appear to be arrested in a transitional stage of development between pre-B and B cells and express surface antigens usually found on normal and fresh leukemic cells of non-B-cell lineages.
The use and safety of Ibuprofen in the hemophiliac
Authors: Inwood MJ , Killackey B , Startup SJ 
Published: October 2019

Abstract
After demonstrating initial safety of Ibuprofen administered to hemophiliacs, a 16-wk double-blind individual crossover trial was designed to test the safety and, to a more limited extent, the efficacy of 1600 mg of Ibuprofen or placebo given daily to 20 hemophiliacs with hemophiliac arthropathy. The trial was completed with no evidence of increased frequency or severity of hemophiliac bleeding episodes or clinical or laboratory evidence of bleeding secondary to Ibuprofen. There were five treatment failures, none associated with hemorrhage or lack of compliance. A benefit was obtained in reduction of early morning stiffness and pain. Ibuprofen should be considered as a safe and potentially beneficial antiinflammatory agent in the treatment of carefully monitored hemophiliacs eligible for such therapy.
Ascorbic acid, vitamin A, folic acid, and amino acids in blood of patients with hemophilia
Authors: Toy L , Young EA , Longenecker JB 
Published: October 2019

Abstract
Blood levels of ascorbic acid, vitamin A, folic acid, and amino acids were studied in patients at the South Texas Comprehensive Hemophilia Center, San Antonio, TX. The mean plasma ascorbic acid level in hemophiliacs was significantly lower than controls (p less than 0.0001). This was observed despite a dietary ascorbic acid intake in excess of 66% of the Recommended Dietary Allowances (RDA). However, those subjects receiving specific factor replacement therapy at home and consuming at least 66% RDA of ascorbic acid maintained a mean plasma ascorbic acid level not significantly less than controls. Hemophilic subjects not on home therapy, on the other hand, had a mean plasma ascorbic acid level significantly below that of controls while receiving optimal dietary ascorbic acid. With prompt adequate medical care of bleeding episodes and with optimal nutrition, the demand for ascorbic acid needed for tissue repair in hemophilic patients may be lessened. Hemophiliacs had mean serum vitamin A, mean serum folate, and mean red cell folate levels that were not significantly different from controls. Significantly higher mean plasma arginine and lower, but not significantly lower, mean plasma ornithine levels were found in hemophilic subjects, suggesting altered arginase activity.
Fibrin-associated plasminogen activation in alpha 2-plasmin inhibitor deficiency
Authors: Aoki N , Sakata Y , Ichinose A 
Published: October 2019

Abstract
The clot formed from the plasma of a patient with congenital deficiency of alpha 2-plasmin inhibitor underwent a spontaneous extensive fibrinolysis. Radiolabeled fibrinogen was added to the plasma before clotting, and the whole process of the fibrinolysis was followed by measuring the release of radiolabels. Plasminogen activation was also followed by measuring the amidolytic activity that developed. There was an initial latent period, followed by an exponential increase of fibrinolytic activity. During the latent period, there was little or no release of radiolabels and no development of amidolytic activity. During the latent period, the clot was washed thoroughly to remove unbound proteins from fibrin and was incubated in buffered saline. The washed clot still underwent fibrinolysis, similar to the original plasma clot, suggesting that the plasminogen/plasminogen activators bound to fibrin during the initial latent period are responsible for fibrinolysis. The amount of plasminogen bound to fibrin during the latent period was close to the amount of plasminogen activated during the whole process of fibrinolysis. When the amount of plasminogen bound to fibrin was decreased by epsilon aminocaproic acid, the extent of fibrinolysis was decreased in parallel with the decrease of the amount of the bound plasminogen. This suggests that the amount of plasminogen bound to fibrin is one of the determinants of the rate of the fibrinolytic process.
Electrophoretic and cytochemical characterization of alpha-naphthyl acetate esterases in acute myeloid leukemia: relationships with membrane receptor and monocyte-specific antigen expression
Authors: Scott CS , Linch DC , Bynoe AG , Allen C , Hogg N , Ainley MJ , Hough D , Roberts BE 
Published: October 2019

Abstract
Alpha-naphthyl acetate esterases (ANAE) were examined by cytochemical and isoelectric focusing (IEF) techniques in 48 cases of acute myeloid leukemia that were classified by conventional morphological criteria. Four main types of ANAE isoenzyme patterns were found by IEF, and comparisons with the expression of membrane receptors (Fc-IgG and C3b) and monocyte-specific antigens (UCHM1, UCHALF, and E11) suggest relationships between ANAE isoenzyme synthesis and distinct myeloid maturational stages. The results further indicate that the blast cells of acute myelomonocytic leukemia (AMML) may represent an immature variant of monocytic leukemia (AMoL) and that morphological examination alone is inadequate in the assessment of monocytic differentiation in acute myeloid leukemias. Inhibition studies of cytochemical ANAE activity with sodium fluoride (NaF) show that the presence of NaF- sensitive or NaF-resistant ANAE enzymes is often unrelated to the diagnostic category of acute leukemia. The results of this study are examined in relation to current concepts of myeloid differentiation, and the application of these findings to the subclassification of acute myeloid leukemias is discussed.
Comparison of two long-term chemotherapy regimens, with or without agents to modify skeletal repair, in multiple myeloma
Authors: Cohen HJ , Silberman HR , Tornyos K , Bartolucci AA 
Published: October 2019

Abstract
A randomized controlled trial was initiated in 1972 to compare two chemotherapeutic regimens [1–3-bis (2-chloroethyl) 1-nitrosourea (BCNU), cyclophosphamide, and prednisone versus melphalan and prednisone], to determine whether the two regimens are cross-resistant, and to evaluate the effectiveness of sodium fluoride, vitamin D, calcium gluconate, and fluoxymesterone in the promotion of bone healing. Initial responses (50%) and survival (36 mo median) for patients treated with the two chemotherapeutic regimens were the same. Patients on either regimen who failed to respond after 6 mo had a very low response rate to the alternative regimen (approximately 10%). Initially responding patients were randomly assigned to either an active drug regimen (sodium fluoride, vitamin D, calcium gluconate, fluoxymesterone) or placebo tablets. There was no significant difference in the low percentage of patients demonstrating bone improvement. Thus, the BCNU, cyclophosphamide, prednisone regimen is as effective as melphalan and prednisone. Fluoride, calcium, vitamin D, and androgenic steroids should not be routinely recommended in myeloma, as they seem to add little to effective chemotherapy and may contribute to morbidity.