bluuebunny/crossref_metadata_2025_split huggingface dataset:
Factor VII Padua 2: another factor VII abnormality with defective ox brain thromboplastin activation and a complex hereditary pattern
Authors: Girolami A , Cattarozzi G , Dal Bo Zanon R , Toffanin F 
Published: October 2019

Abstract
A new factor VII abnormality is presented. The propositus was a 9-yr- old child who presented a mild bleeding tendency characterized by epistaxis and easy bruising. The parents were not consanguineous, but they came from the same area. The laboratory features were mild prolongation of prothrombin time and P.P. test and normal partial thromboplastin and Stypven cephalin clotting times. The Thrombotest was moderately prolonged. Factor VII was 40%-50% of normal using rabbit or human brain thromboplastin, but only 13%-24% using ox brain thromboplastin. Factor VII cross-reacting material (CRM) was about 50% of normal. The father, a paternal aunt, and a paternal cousin showed similar clinical and laboratory findings. The brother of the propositus, the mother, and other members of her family showed about 50% factor VII activity and CRM and were considered to be heterozygotes for true factor VII deficiency. Similar findings were also present in the father and in the brother of the affected cousin. The defect in the propositus seems to consist of a double heterozygosity between abnormal factor VII and heterozygous factor VII true deficiency. The factor VII abnormality appears to consist of abnormal reactivity toward ox brain tissue thromboplastins and appears to be different from previously described factor VII abnormalities. The name factor VII Paudua2 is proposed for this condition.
Shifts in expression of cell membrane phenotypes in childhood lymphoid malignancies at relapse
Authors: Borella L , Casper JT , Lauer SJ 
Published: October 2019

Abstract
To determine if cell membrane phenotypes change under the selective pressures of therapy we have conducted a prospective study of 54 children with lymphoid malignancies of T-like, B-like, common, and null cell types. Membrane phenotypes were determined at diagnosis in all patients and again 1–24 mo later in 18 children who either failed induction therapy or had one or more relapses. In 7 patients the cells tested were from relapse sites different than those of the original diagnoses. The data indicate that at relapse most children with lymphoid neoplasias had the same cell membrane phenotype as established at diagnosis, and suggest that the site of relapse did not affect the expression of cell surface markers. However, there were three exceptions: (1) a child initially diagnosed as having null cell acute lymphocytic leukemia had 90% T-antigen-positive blasts in her second- relapse bone marrow; (2) only membrane IgM was present on relapse blasts from a B-cell lymphoma that had both membrane IgM and IgD before initiation of treatment; (3) at diagnosis, bone marrow blasts from a child with T-like leukemia expressed both T antigen and E receptors, but at relapse, bone marrow and pleural fluid cells expressed only T antigens. We postulate that these phenotype shifts may be due to selective effects of therapy on cells at different stages of differentiattion.
Surface immunoglobulin density on human peripheral blood mononuclear cells
Authors: Slease RB , Wistar R Jr , Scher I 
Published: October 2019

Abstract
The densities of surface immunoglobulin (slg) on peripheral blood mononuclear cells (PBM) of normals and patients with chronic lymphocytic leukemia (CLL), chronic lymphosarcoma cell leukemia (LCL), and hairy cell leukemia (HCL) were analyzed using the fluorescence- activated cell sorter (FACS). PBM were labeled with fluorescein conjugates of F(ab')2 fragments of affinity chromatography-purified anti-Fab or class-specific anti-mu, anti-delta, anti-gamma, or anti- alpha. Histograms of relative cell fluorescence, rems of relative cell fluorescence, reflecting slg density, were prepared with the FACS. Anti- Fab-labeled normal PBM demonstrated a homogeneous low-density peak that when separated by the FACS and analyzed cytochemically consisted predominantly of monocytes, whereas brighter-staining cells were predominantly lymphocytes. Anti-mu and anti-delta labeled 9.0% and 8.5% of normal PBM, respectively, the slg+ cells being virtually all lymphocytes. Cells labeled by anti-gamma exhibited low homogeneous slg density and consisted of more than 80% monocytes. No normal or leukemic PBM were labeled by anti-alpha. All slg-positive cells (less than 5% monocytes) from 12 of 13 patients with CLL had very low homogeneous densities of slg and bore slgM, Whereas cells from 9 of 13 and 2 of 13 patients bore slgD and slgG, respectively. Similarly, PBM from 2 patients with HCL exhibited low and homogeneous densities of algM, slgD, and slgG, whereas those from a third patient bore only slgG. By contrast, the density of slgM and PBM derived from 3 patients with LCL was very high; slgD and slgG densities varied from very high to undetectable in these patients. The different homogeneous densities of slg on peripheral blood lymphocytes from patients with CLL, HCL, and LCL suggest that these diseases represent malignant transformation of different B-lymphocyte subpopulations.
Release of platelet fibronectin (cold-insoluble globulin) from alpha granules induced by thrombin or collagen; lack of requirement for plasma fibronectin in ADP-induced platelet aggregation
Authors: Zucker MB , Mosesson MW , Broekman MJ , Kaplan KL 
Published: October 2019

Abstract
Platelets lysed with Triton X-100 contain 3.44 +/- 1.27 (SD) microgram of fibronectin (cold-insoluble globulin) per 10(9) platelets. Fibronectin was partially released from washed whole platelets by collagen or thrombin, and its release by collagen was inhibited by aspirin. Analysis of subcellular fractions obtained by density-gradient centrifugation of disrupted platelets indicated that fibronectin was contained in the alpha granules. Fibrinogen depleted of fibronectin (less than 2 microgram/mg) supported ADP-induced aggregation as effectively as fibrinogen contaminated with this protein, thus reinforcing the generally held view that fibrinogen itself is the necessary protein cofactor in this reaction.
Two cases of carcinoma of the lung characterized by a bone marrow agar culture pattern resembling acute myeloid leukemia
Authors: McCarthy JH , Sullivan JR , Ungar B , Metcalf D 
Published: October 2019

Abstract
In vitro agar culture patterns of bone marrow cells in acute myeloid leukemia may show several growth patterns, including cultures where no colonies or clusters develop, cultures with varying numbers of clusters and no colonies, or colony and cluster formation with an extremely high ratio of clusters to colonies. Twelve cases of carcinoma of the lung are described, of which two show an in vitro growth pattern of cluster formation alone, characteristic of that seen in acute myeloid leukemia. The remaining ten patients showed slightly reduced colony numbers compared to normal.
Clonal growth of metastatic carcinoma of bronchus in bone marrow culture
Authors: Francis GE , Broadbent JE , Johnson N , Hoffbrand AV 
Published: October 2019

Abstract
Bone marrow cells from a 35-yr-old male with small cell anaplastic carcinoma of the bronchus and bone marrow metastases were cultured in semisolid agar. Numerous small clones grew in the cultures, with a similar gross appearance to clones produced by cell from a proportion of patients with acute myeloid leukemia. Examination of the cells from these clones by electron microscopy revealed marked differences between these cells and cells grown in cultures from normal and acute myeloid leukemia marrow. This suggests that metastatic cells of the anaplastic carcinoma of the bronchus grew in clones in the agar culture system.
Lithium and granulocytopenia during induction therapy of acute myelogenous leukemia
Authors: Stein RS , Flexner JM , Graber SE 
Published: October 2019

Abstract
Twenty-seven patients receiving a standard cytosine arabinoside and daunorubicin regimen as induction of reinduction therapy of acute myelogenous leukemia were randomly assigned to receive lithium carbonate, 300 mg t.i.d., or no lithium. Treatment groups were comparable with respect to age and baseline granulocyte counts. All patients developed granulocyte nadirs below 100/cu mm. By actuarial analysis, the median duration of granulocytopenia, less than 1000/cu mm, was 16.0 days in the lithium group and 24.6 days in the no-lithium group, p = 0.013. The median duration of granulocytes less than 500/cu mm also favored the lithium group but only approached statistical significance: 14.0 days versus 20.5 days, p = 0.054. Lithium levels between 0.5 and 1.0 meq/liter were easily maintained in 11 of 12 patients receiving lithium, 300 mg t.i.d., and toxicity directly attributable to lithium was not observed. Despite the shortened duration of neutropenia, the incidence of infections and the rate of remission were not affected.
Membrane receptors and their redistribution in lymphoproliferative disorders
Authors: Naeim F , Bergmann K , Gatti RA 
Published: October 2019

Abstract
Lymphoid cells from 20 patients with lymphoproliferative disorders, including chronic lymphocytic leukemia, hairy cell leukemia, Sezary syndrome, lymphoma, and lymphadenitis, were studied for redistribution of surface membrane immunoglobulins (SmIg) and concanavalin A (Con-A) receptors. Fluorescein-labeled polyvalent goat anti-human immunoglobulin and fluoresceinated concanavalin A were used as ligands. Results were similar with both ligands. The highest percentage of capping of ligand-membrane receptors was noted in mononuclear cells from patients with “hairy” cell leukemia: from 24% to 90%. These cells showed moderate to marked fluorescein activity and were able to cap within 15 min at 4 degrees C. Chronic lymphocytic leukemia cells showed a weak fluorescein stain with a very low percentage of cells (0%--16%) capping. Lymph node cells from patients with lymphoma demonstrated moderate to strong fluorescein activity with only an average of 3% of the cells capping; while lymphoid cells from patients with lymphaedenitis showed an average of 27.5% capping and moderate fluorescein activity. Capping of Con-A receptors in mononuclear cells from patients with Sezary syndrome was poor (0%--14%) with moderate fluorescein intensity. This report demonstrates difference in density and mobility of binding sites for SmIg and Con-A on the surface membrane of lymphoid cells from various subclasses of lymphoproliferative disorders. These differences may assist in the differential diagnosis and classification of these conditions.
Fetal erythropoiesis and hemoglobin ontogeny in tail-short (Ts/+) mutant mice
Authors: Brotherton TW , Chui DH , McFarland EC , Russell ES 
Published: October 2019

Abstract
Mutant Ts/+ fetuses are developmentally retarded as compared to normal +/+ littermates. Mutant fetuses have less total hemoglobin than do normal fetuses of the same gestational age. However, when compared to +/+ fetuses of similar body weight, Ts/+ fetuses have the appropriate amount of total hemoglobin, suggesting that the apparent anemia observed in mutant fetuses is most likely the result of delay in growth and development. Changes in proportions of embryonic hemoglobins during fetal development are similar in Ts/+ and +/+ fetuses at day 12 and later of gestation. Moreover, adult hemoglobin is detected in circulating primitive nucleated erythrocytes in the developmentally retarded Ts/+ mutant fetuses at about the same chronologic age as their +/+ normal littermates.
Megakaryocyte colony formation from human bone marrow precursors
Authors: Vainchenker W , Bouguet J , Guichard J , Breton-Gorius J 
Published: October 2019

Abstract
We report the growth in plasma clot culture of megakaryocyte colonies from adult bone marrow cells with the use of four different sources of erythropoietin (Ep) as the stimulating factor. A major proportion of the megakaryocyte colonies (75%) were pure, while the others were mixed, involving erythroblasts and megakaryocytes. Ultrastructural studies have shown that the maturation of megakaryocytes was essentially normal and that platelet shedding occurred. Megakaryocyte colony formation required a large number of plated cells (greater than 3 X 10(5)/ml). In the absence of erythropoietin, rare spontaneous megakaryocyte colonies could be observed, while no erythroid colonies were present. However, erythropoietin induced a fivefold increase in the total number of colonies. These data suggest that erythropoietin is involved in the differentiation of human megakaryocytes, but that it does not act alone, since another factor related to the number of seeded cells appears essential for the formation of human megakaryocyte colonies.
Premature chromosome condensation studies in human leukemia. I. Pretreatment characteristics
Authors: Hittelman WN , Broussard LC , McCredie K 
Published: October 2019

Abstract
The phenomenon of premature chromosome condensation (PCC) was used to compare the bone marrow proliferation characteristics of 163 patients with various forms of leukemia prior to the initiation of new therapy. The proliferative potential index (PPI, or fraction of G1 cells in late G1 phase) and the fraction of cells in S phase was determined and compared to the type of disease and the bone marrow blast infiltrate for each patient. Previously untreated patients with acute leukemia exhibited an average PPI value three times that of normal bone marrow (37.5% for acute myeloblastic leukemia [AML], acute monomyeloblastic leukemia [AMML], or acute promyelocytic leukemia [APML] and 42% for acute lymphocytic leukemia [ALL] or acute undifferentiated leukemia [AUL]). Untreated chronic myelogenous leukemia (CML) patients showed intermediate PPI values (25.2%), whereas CML patients with controlled disease exhibited nearly normal PPI values (14.6%). On the other hand, blastic-phase CML patients exhibited PPI values closer to that observed in patients with acute leukemia (35.4%). Seven patients with chronic lymphocytic leukemia (CLL) exhibited even higher PPI values. No correlations were observed between PPI values, fraction of cells in S phase, and marrow blast infiltrate. For untreated acute disease patients, PPI values were prognostic for response only at low and high PPI values. These results suggest that the PCC-determined proliferative potential is a biologic reflection of the degree of malignancy within the bone marrow.
Comparison of fibrinogen association with normal and thrombasthenic platelets on exposure to ADP or chymotrypsin
Authors: Mustard JF , Kinlough-Rathbone RL , Packham MA , Perry DW , Harfenist EJ , Pai KR 
Published: October 2019

Abstract
Although 125I-fibrinogen becomes associated with washed platelets from normal human subjects during ADP-induced shape change and aggregation, 125I-fibrinogen did not become associated with washed plateletes from a thrombasthenic subject during ADP-induced shape change and the platelets did not aggregate. Platelets from control and thrombasthenic subjects were treated with chymotrypsin, which is known to degrade platelet membrane glycoproteins. More 125I-fibrinogen became associated with chymotrypsin-pretreated platelets from normal subejcts than with untreated platelets, and fibrinogen caused the enzyme-treated platelets to aggregate. 125I-fibrinogen did not become associated with chymotrypsin-pretreated thromobasthenic platelets, and fibrinogen did not aggregate them. Thus, there appears to be a defect in thrombasthenic platelets that prevents the association of fibrinogen with them.
Thrombocytosis-induced suppression of small acetylcholinesterase- positive cells in bone marrow of rats
Authors: Long MW , Henry RL 
Published: October 2019

Abstract
Transfusion of platelet concentrates was used to establish a thrombocytosis of approximately three times normal platelet levels in male rats. This thrombocytosis resulted in a rebound thrombocytopenia to 60% of normal counts. Examination of the small acetylcholinesterase (ACh-E) positive cells of the marrow at this time showed a reduction to 50% of normal levels without significant changes in control animals. A second group of experiments indicated that this suppression developed as early as the third day posttransfusion, persisted until day 7, and returned to baseline levels by day 9. Incorporation of 75SeM indicated that the reduction in platelet count was due to decreased platelet production. Little or no changes were observed in the hematocrit or WBC. This evidence supports the hypothesis that these cells are early cells in the megakaryocytic series. They are the earliest cells of the series seen to be affected by thrombocytosis. Feedback control by platelets or platelet extracts of this cell population may represent one level of regulation of megakaryopoiesis.
Effect of prothrombin complex concentrates on factor VIII inhibitor levels
Authors: Kasper CK 
Published: October 2019

Abstract
Factor VIII inhibitor levels were measured on 261 occasions in 76 hemophilia-A inhibitor patients before and after prothrombin complex concentrate infusion at 13 treatment centers. A rise in inhibitor level to at least twice the pretreatment value occurred in 35 treatment episodes (13.5%), in 27 patients (36%). In 16 patients (21%), such an anamnestic immune response occurred with the first treatment. Factors predisposing to anamnestic responses may include patient idiosyncrasy, low pretreatment inhibitor levels, and exposure to concentrate over several days.
Molecular basis of hemoglobin-H disease in the Mediterranean population
Authors: Kan YW , Dozy AM , Stamatoyannopoulos G , Hadjiminas MG , Zachariades Z , Furbetta M , Cao A 
Published: October 2019

Abstract
We investigated the molecular basis of hemoglobin-H disease by hybridization and restriction endonuclease mapping of the DNA in the Mediterranean populations. Of the 12 patients studied from Cyprus and Sardinia, 8 had the typical deletion defect with a single remaining alpha-globin gene. The nondeletion type of alpha-thalassemia was found in 3, and a “dysfunctional” gene in one. We conclude that the predominant cause of alpha-thalassemia in these populations is gene deletion.
Dual B and T markers in acute and chronic lymphocytic leukemia
Authors: Foon KA , Billing RJ , Terasaki PI 
Published: October 2019

Abstract
Leukemic cells from 20 patients with chronic lymphocytic leukemia (CLL) and 60 patients with acute lymphocytic leukemia (ALL) were studied for T- and B-lymphocyte cell surface membrane markers. B-cell markers included surface membrane immunoglobulin, erythrocyte-antibody complement rosette formation, and B-cell (a-like or HLA-DR) antigens detected by a B-cell antiserum. T-cell markers included spontaneous sheep red blood cell rosette formation and a cytotoxic reaction to a specific T-cell antiserum. Seven patients with CLL and two with ALL had dual B and T markers. We propose that dual B- and T-cell markers are more common in CLL and ALL patients than previously reported. With newer and more sensitive tests for identification of B and T cells, this observation may be recognized more frequently.
Platelet production after administration of antiplatelet serum and 5- fluorouracil
Authors: Radley JM , Hodgson GS , Levin J 
Published: October 2019

Abstract
Although 5-fluorouracil induces relatively minimal thrombocytopenia in mice compared with antiplatelet serum, the ensuing rebound thrombocytosis is much greater. It also occurs several days later. Administration of 5-fluorouracil 24 hr after antiplatelet serum suppresses the increase in platelets produced by antiplatelet serum while the subsequent thrombocytosis is indistinguishable from that produced by 5-fluorouracil alone. It is concluded that the thrombocytosis that occurs after 5-fluorouracil originates from a class of primitive stem cells, or early megakaryocyte precursors, which are not killed by the drug. Thrombocytosis occurring after antiplatelet serum is derived from more mature precursor cells, which are sensitive to 5-fluorouracil.
Immunologic characterization of a granulocytic leukemia of inbred strain 13 guinea pigs: presence of Ia-positive myeloblasts
Authors: Clement LT , Hu C , Shevach EM , Green I 
Published: October 2019

Abstract
Membrane markers expressed by a transplantable granulocytic leukemia induced in inbred strain 13 guinea pigs by N-nitroso-N-butylurea have been investigated by serologic and immunochemical methods. Although the leukemic myeloblasts have no detectable surface immunoglobulin or Fc receptors, they have been found to synthesize and express membrane antigens encoded for by the I-region of the major histocompatibility complex. Despite the presence of Ia antigens, these cells do not stimulate allogeneic T lymphocytes in the mixed lymphocyte reaction nor are they able to present soluble antigen to immune T cells. The presence of Ia antigens on immature myeloid cells suggests that these proteins may be important in cellular functions beyond those identified in the cellular immune system.
Thymidine as a chemotherapeutic agent: sensitivity of normal human marrow, peripheral blood T cells, and acute nonlymphocytic leukemia
Authors: Howell SB , Taetle R , Mendelsohn J 
Published: October 2019

Abstract
Normal marrow granulocyte (CFU-GM) and peripheral blood T-lymphocyte (CFU-TL) colony-forming cells were studied for their sensitivity to high concentrations of thymidine (dThd) and compared to leukemic CFU from patients with acute nonlymphocytic leukemia (ANLL). The sensitivity of two ANLL cell lines was also assessed. dThd was toxic to both CFU-GM and CFU-TL at concentrations above 10(-5) M when cultured under conditions where dThd exposure was analogous to that used in clinical trials. There was little variation in the fractional colony survival between marrow samples, and the sensitivity of CFU-GM closely approximated that of CFU-TL. Thymine was not toxic at up to 10(-3) M. In liquid culture, T cells in G0 at the start of exposure were able to proliferate in the presence of 10(-3) M dThd, whereas T cells already proliferating in response to phytohemagglutinin (PHA) at the start of dThd exposure were killed. Leukemic CFU demonstrated marked variability in dThd sensitivity; blasts from some patients were resistant to dThd, while others were greater than 100-fold more sensitive than normal CFU- GM.
Fatal graft-versus-host disease following blood transfusion in Hodgkin's disease documented by HLA typing
Authors: Dinsmore RE , Straus DJ , Pollack MS , Woodruff JM , Garrett TJ , Young CW , Clarkson BD , Dupont B 
Published: October 2019

Abstract
Fatal graft-versus-host disease (GVHD) developed in a patient with Hodgkin's disease treated with combined chemotherapy and radiotherapy following the transfusion of 2 U of packed red blood cells. Clinical features of the GVHD included the development of exfoliative dermatitis, progressive hepatic dysfunction, aplastic anemia, and finally progressive fatal pneumonia. GVHD was documented by skin biopsy and chimerism by HLA typing. The HLA phenotype of the patient's skin fibroblasts [A3, Bw44 (w4)/A2, B15 (w4)] was appropriate for parental haplotypes and probably represented her true HLA phenotype. Lymphocytes from the patient (peripheral blood and lymph node biopsy) were of a different HLA phenotype (A3; Bw35, w38, w4, w6; Cw4), which was inappropriate for parental HLA haplotypes but identical to the HLA phenotype of one of the blood donors. The HLA-DR typing of the patient's family and of the blood donor demonstrated that the patient and the donor probably were HLA-DR identical (DRw5/DRw6), although no B lymphocytes could be obtained from the patient for direct DR typing. We are currently irradiating all blood products administered to patients with Hodgkin's disease receiving intensive treatment. Further observations will be necessary to determine whether transfusions to other cancer patients with immunodeficiency states should be restricted to irradiated blood products.