bluuebunny/crossref_metadata_2025_split huggingface dataset:
A case of red‐cell adenosine deaminase overproduction associated with hereditary hemolytic anemia found in Japan
Authors: Miwa Shiro , Fujii Hisaichi , Matsumoto Noboru , Nakatsuji Tadako , Oda Susumu , Miura Yukari , Asano Hiroyuki , Asano Seiichi 
Published: February 2007

AbstractA case of red cell adenosine deaminase (ADA) overproduction associated with hereditary hemolytic anemia is reported here. This appears to be the second report. Proband is a 38‐year‐old Japanese male who had hemoglobin, 15.8 g/100 ml; reticulocyte count, 4.5%; serum indirect bilirubin, 4.9 mg/100 ml; 51 Cr‐labeled red cell half‐life, 12 days; red cells showed moderate stomatocytosis. His red cell ADA activity showed 40‐fold increase while that of the mother showed 4‐fold increase. The mother was hematologically normal. The father had a normal enzyme activity. The proband and the mother showed slightly high serum uric acid levels. The proband's red cell showed: ATP, 628 nmoles/ml (normal, 1,010‐1,550); adenine nucleotide pool, 46% of the normal mean; 2,3‐diphosphoglycerate content, 3,782 nmoles/ml (normal 4,170‐5,300); increased oxygen affinity of hemoglobin, P50 of intact crythrocytes being 21.8 mmHg (normal, 24.1‐26.1). Red cell glycolytic intermediates in the proband were low in general, and the rate of lactate production was low. Kinetic studies using crude hemolysate revealed a normal Km for adenosine, normal electrophoretic mobility but slightly abnormal pH curve and slightly low utilization of 2‐deoxyadenosine. The ADA activity of lymphocytes was nearly normal.
Some observations on propranolol on platelet aggregation and release
Authors: Leon Rafael , Tiarks Cheryl Y. , Pechet Liberto 
Published: February 2007

AbstractPlatelet function was investigated in four normal volunteers, one patient with a mild form of von Willebrand disease, and one with a thrombocytopathy, all taking propranolol. No effect on platelet function attributable to this drug could be demonstrated in any of these subjects. It is suggested that propranolol administered in conventional doses does not impair platelet hemostatic function.
B‐Cell leukemia‐lymphoma with striking resemblance to burkitt lymphoma in a 70‐year‐old woman
Authors: Shaw Michael T. , Palmer Charles , Yuhas John M. , Rembe Armin , Robertson Gerald , Bankhurst Arthur D. 
Published: February 2007

AbstractA 70‐year‐old woman developed acute leukemia and a serum IgM spike. She entered complete remission with an adriamycin, vincristine, cytosine arabinoside, and prednisone combination. Bone marrow remission was maintained with intermittent cytosine arabinoside; however, she developed large skin nodules which partly remitted following adriamycin, vincristine, cyclophosphamide, and prednisone combination. They very rapidly recurred, and she died soon after. Autopsy revealed extensive tumor in the abdomen, pelvis, and thoracic cavity, but no bone marrow involvement. Histology revealed a “starry sky” appearance. Cytology showed undifferentiated cells with vacuolated cytoplasm resembling Burkitt lymphoma cells. Peroxidase and esterase stains were negative. There was strong pyroninophilia and the periodic acid‐Schiff reaction showed granular activity in the cytoplasm. Electron microscopic appearances also resembled Burkitt lymphoma. Cytogenetic studies were normal, with no Ph1 chromosome. Immunofluorescence demonstrated surface IgM and a little IgA. 3H‐thymidine incorporation was high, indicating rapid growth. Dibutyral cyclic adenosine monophosphate (cAMP) stimulated growth, which was further evidence of the lymphoid origin of the tumor. The close resemblance of this tumor to Burkitt lymphoma emphasizes the difficulties in systematically classifying the lymphomas.
New leukemia in the course of therapy of acute lymphoblastic leukemia
Authors: Ravindranath Yaddanapudi , Inoue Susumu , Considine Basil , Lusher Jeanne , Zuelzer Wolf W. 
Published: February 2007

AbstractWe have recently observed the development of second leukemia of a morphologically different type in three patients with acute lymphoblastic leukemia (ALL) while on therapy. The “second” leukemia occurred while on therapy at 23, 27, and 32 months of initial remission. All three were receiving systemic chemotherapy (CT) and prophylactic fractional irradiation to the central nervous system (CNS). The second leukemias in these three cases were one case of juvenile chronic myelogenous leukemia (JCML) and two cases of acute leukemia of the myeloblastic type by the usual morphologic criteria including the presence of Auer rods in one. In two cases a cytogenetically new clone was detected in the remission marrow 10 and 12 months preceding the overt change in clinical status.These three cases demonstrate that second leukemia occurs in patients with ALL and that some late “relapses” fall into this category. The possible etiologic role of modern intensive treatment regimens in the development of second leukemia is discussed.
A new chromosome abnormality in idiopathic sideroblastic anemia: 46, XY, del11q23
Authors: Hyder Dan M. , Bottomley Sylvia S. , Bottomley Richard H. 
Published: February 2007

AbstractA new marker chromosome, deletion 11q23, was observed with the Giemsa banding technique in the bone marrow of a patient with idiopathic sideroblastic anemia. The abnormality was not detectable in the peripheral blood or with nonbanded chromosome studies. Nineteen of 40 cases of this disorder studied and reported to date had chromosomal aberrations, although the majority had only nonbanded karyotypes performed. This apparently high incidence of chromosomal defects and the finding in the present case indicate that more banded‐karyotype analyses are needed to assess the presence of possible nonrandom cytogenetic changes in idiopathic sideroblastic anemia.
Antibody‐induced von willebrand syndrome: Inhibition of VIII VWF and VIII AGN with sparing of VIII AHF by the autoanti body
Authors: Gazengel C. , Jacques C. , Nedellec J. , Josso F. , Buriot D. , Prieur A. M. 
Published: February 2007

AbstractAcquired von Willebrand syndrome is reported in a 16‐year‐old girl with systemic lupus erythematosus. Routine coagulation studies showed a normal platelet count, prolonged bleeding time, and abnormal glass bead retention. Factor VIII molecular complex respective activities were 8% for VIII AHF and undetectable for VIII VWF (ristocetin aggregation of washed platelets) and VIII AGN (electro‐immunodiffusion).In vitro, the patient's plasma exhibited an inhibitory activity against exogenous VIII AGN and VIII VWF but did not neutralize VIII AHF activity of control plasma, even after a two‐hour incubation at 37°C. This inhibitory activity was supported by the purified plasma IgG fraction. In vivo, following cryoprecipitate administration (20 units VIII AHF/kg), only 50% of the infused VIII AHF activity was recovered after 15 mn, and the original level was reached four hours later. Only a transient peak of VIII VWF activity was observed, and VIII AGN level did not increase at all after the infusion.After the start of prednisone therapy the three factor VIII related activities returned to normal levels in the following order: VIII AHF and VIII VWF (9‐12 days); VIII AGN (3 weeks).These findings could be explained by the formation of a short‐lived circulating immune complex between the antibody and the factor VIII molecular complex. In such an hypothesis the autoantibody would respect the site of VIII AHF activity and would mask the site reacting with anti VIII AGN heteroantibodies.
Control factors of granulopoiesis in human serum
Authors: Bolin Rex W. , Robinson William A. 
Published: February 2007

AbstractThe role of serum factors in the modulation of production of colony‐stimulating activity (CSA) has been investigated. A factor has been described, and partially characterized, in human serum that has the capacity to stimulate increased synthesis and release of CSA by human mononuclear cells (MNC). MNC RNA and protein synthesis are required to demonstrate this effect of serum, but DNA synthesis and mitotic division are not required. The factor in serum resulting in this effect is a heat‐labile protein with a molecular weight slightly greater than that of CSA.
Studies on hematopoietic stem cells: XI. Lack of erythroid burst‐forming units (BFU‐E) in patients with aplastic anemia
Authors: Moriyama Yoshiaki , Sato Masatsugu , Kinoshita Yasutami 
Published: February 2007

AbstractThe marrow concentration of erythropoietic precursors was examined in normal donors and patients with idiopathic aplastic anemia using a plasma clot culture system. On time course observations the heterogeneity of human erythroid precursors assayable in culture was demonstrated. To evaluate human erythropoiesis in vitro, the benzidine‐positive colonies were divided into three groups: small colony, containing 8–50 cells; medium‐sized colony, containing 50–500 cells; and large colony, containing more than 500 cells. The majority of the large colonies assumed the morphology of erythropoietic bursts (BFU‐E) consisted of several subcolonies. The small colonies were counted as CFU‐E1, the medium‐sized as CFU‐E2, and the large as BFU‐E to evaluate the erythroid precursor cell compartment in aplastic anemia.The marrow concentration of CFU‐E1 and CFU‐E2 was shown to be quantitatively diminished in aplastic anemia. In addition, there was no ability of the marrow cells from aplastic patients to grow BFU‐E in vitro even in the presence of a large dose of erythropoietin. This lack of BFU‐E colony growth may play an important role in the mechanism of the erythropoietic deficiency in aplastic anemia.
The periodic acid‐schiff reaction and prognosis in children with acute lymphoblastic leukemia
Authors: Raney R. Beverly , Festa Robert S. , Waldman Maria T. G. , Manson David , Hann Hie‐Won L. 
Published: February 2007

AbstractWe evaluated the intensity of the periodic acid‐Schiff (PAS) stain reaction by examining 200 lymphoblasts from the initial marrow aspirate specimen of 38 children with acute lymphoblastic leukemia (ALL). The patients were separated into low, intermediate, and high groups according to mean PAS score. The groups were then compared in terms of age, sex, initial hematologic values, T‐ and B‐lymphocyte markers, percentage of patients in remission six months after diagnosis, and percentage surviving. Significant differences were found between the low group and the other two groups with respect to sex, height of initial white blood cell (WBC) count, outlook for continued remission at six months, and overall survival (P < 0.05). The low‐score patients were predominantly males, whereas the other PAS‐score groups contained approximately equal numbers of males and females. The low‐score patients had a higher median initial WBC than the others, but all three groups had a similar proportion of patients with initial WBC under 20,000/cu mm. Low‐score patients were less likely to be in remission six months from diagnosis, and they had shorter survival than the intermediate‐ and high‐score patients (P < 0.05). The results suggest that children with ALL whose lymphoblasts stain weakly with PAS have a worse prognosis than those with intermediate or high PAS reactivity. PAS reactivity may vary independently of the WBC at diagnosis in children with ALL, because children with a low PAS score have a poor outlook even with initial WBC under 20,000/cu mm.
Acute leukemia and preleukemia in eight males in a family: An X‐linked disorder?
Authors: Li Frederick P. , Marchetto David J. , Vawter Gordon F. 
Published: February 2007

AbstractEight males in a family died of acute leukemia or a potentially preleukemic blood disease. Three patients were descendants of a man through his first wife, and the other five through his second wife. The pattern of disease in the family suggests X‐linked inheritance through the two wives, although other mechanisms cannot be excluded. No detectable action of environmental leukemogens was observed in this family disorder.
Reduced ferrochelatase activity in fibroblasts from patients with porphyria variegata
Authors: Viljoen D. J. , Cayanis E. , Becker D. M. , Kramer S. , Dawson B. , Bernstein R. 
Published: February 2007

AbstractFerrochelatase deficiency has been shown in both porphyria variegata (PV) and erythropoietic protoporphyria (EPP). It has been suggested that in PV there is a decrease in the enzyme, whereas in EPP the enzyme is unstable. In the present study ferrochelatase activity was measured in skin fibroblasts from three patients with PV and three normal subjects. The enzymatic activity in the patients with PV (17.5 ± 4.5 pmoles heme formed per 107 fibroblasts per hour) was 50% of that of the control group (31.0 ± 3.2 pmoles heme formed per 107 fibroblasts per hour). This supports the contention that the enzyme is deficient in PV and that an inactive ferrochelatase is the primary deficiency in this type of porphyria.
Chromosomes and causation of human cancer and leukemia. XXXIII. 5q– in a case of acute lymphoblastic leukemia (ALL)
Authors: Abe S. , Kohno S. , Kubonishi I. , Minowada J. , Sandberg A. A. 
Published: February 2007

AbstractThe case of a 4‐year‐old boy with ALL, possibly developing subsequent to a lymphoma involving the throacic cavity, which was shown to be of the T‐cell type, is presented. The leukemic cells had a 5q– anomaly, which had previously been described only in cases of refractory anemia and/or AML. The 5q– abnormality was invariably accompanied by a 9p– chromosome in the leukemic cells. The interstitial deletion leading to the 5q– chromosome was shown with banding techniques to be similar to that described previously in myeloproliferative disorders. Some aspects of the 5q– anomaly in ALL and its relation to previous experience with this karyotypic change are discussed.
Antisickling agents: Effects of carbamyl phosphate or cyanate on survival, erythrocytes, and leucocytes in the mouse
Authors: Kraus Lorraine M. , Jernigan Howard M. , Schrank Gordon D. , Kraus Alfred P. 
Published: February 2007

AbstractEqual mole doses of the anions of disodium carbamyl phosphate (carbamyl P) or sodium cyanate, antisickling agents, have been compared in C57B1 mice. Using 15 mice per group, two groups were given the equivalent ip dose of carbamyl P or cyanate anion (7 mmoles/kg/day) in a divided dose, in the morning and six hours later, for 17–18 days. The control group received sodium chloride (13.8 mmoles of Na+ or C1−/kg/day). Surviving mice per group were sodium chloride, 15/15; disodium carbamyl P, 14/15; and sodium cyanate, 0/15, all mice died by day 2. Surviving mice appeared normal throughout the study, and no abnormalities were seen at necropsy. The hematologic measurements were the same for sodium chloride or disodium carbamyl P, including hemoglobin, packed cell volume, erythrocyte counts, leucocyte counts, and differential counts. The mean hemoglobin carbamylation was 1.24 (±0.06 SE) moles of valine hydantoin/mole of hemoglobin tetramer in mice receiving disodium carbamyl P for 18 days, sufficient for antisickling activity. The enzymatic degradation of carbamyl P to NH3, CO2, and Pi was measured in serial blood samples in additional C57B1 and DBA/2J mice following ip injections of carbamyl P or cyanate. Both NH3 and Pi increased immediately after giving carbamyl P, but no increase occurred after cyanate administration. Thus enzymatic degradation of carbamyl P occurs in vivo and appears to be an important detoxification mechanism. When equivalent mole doses of anion are administered, disodium carbamyl P is less toxic than sodium cyanate in mice.
The kinetics of immunoglobulin synthesis by pokeweed mitogen stimulated peripheral blood lymphocytes in multiple myeloma
Authors: George Edward R. , Cohen Harvey J. 
Published: February 2007

AbstractAs a means of investigating the immunoglobulin (Ig) deficiency in multiple myeloma, the effects of pokeweed mitogen (PWM) on morphologic transformation and concomitant immunoglobulin synthesis and secretion kinetics were studied in peripheral blood lymphocytes (PBL) of normals and patients with myeloma. Lymphocyte Ig production kinetics, both in the unstimulated state and after five days of culture with PWM, were evaluated by 3H‐leucine incorporation. B‐lymphocytes were found to be decreased by EC3 rosetting in the myeloma patients studied. In addition their lymphocytes had a mean of 30% transformation compared to 53% for normals (P < 0.005). After five days of mitogen stimulation in culture, myeloma PBL failed to show the rise in intracellular Ig production seen in normal PBL under similar conditions. Thus, myeloma patients were found to have decreased numbers of EC3 rosetting B‐cells, decreased morphologic transformation rate in response to mitogenic stimulation, and failure to increase intracellular Ig after mitogenic stimulation. Possible etiologic mechanisms for the decreased Ig production are discussed.
Ferritin synthesis and Iron uptake in developing erythroid cells
Authors: Konijn A. M. , Hershko C. , Izak G. 
Published: February 2007

AbstractThe effect of maturation on ferritin synthesis and iron uptake by erythroid precursor cells was studied in vitro in rabbit bone marrow cells synchronized by actinomycin D. Early mononuclear precursors were compared with basophilic normoblasts and reticulocytes. Apoferritin synthesis was measured by following the incorporation of 3H‐amino acids into purified ferritin, and this was correlated with total protein synthesis and with radioactive iron uptake by whole cells and radioactive iron incorporation into heme and ferritin. With increasing cellular maturation, a progressive reduction in both apoferritin and total protein synthesis has been observed, undifferentiated cells synthesizing 12 times more protein and 20 times more ferritin than reticulocytes. The rate of ferritin synthesis was inversely related to the rate of radioactive iron uptake by whole cells and radioactive iron incorporation into heme; lowest rates of radioactive uptake and heme synthesis were found in undifferentiated cells, and highest rates in reticulocytes. Radioactive iron incorporation into ferritin was higher in basophilic normoblasts than in undifferentiated cells but was very low in reticulocytes. This sequence of events indicates that in developing erythroid cells the phase of maximal ferritin synthesis precedes the phase of maximal iron uptake. The accumulation of newly formed apoferritin is followed by an increase in cellular iron uptake and an increased retention of iron in ferritin. Finally, with continuing cellular maturation, ferritin iron and iron derived directly from transferrin are utilized for hemoglobin synthesis.
Marrow culture in diffusion chambers in rabbits: III. Effect of endotoxin and leukocyte products on cell production
Authors: Willemze Roel , Walker Richard I. , Herion John C. , Palmer Jeffress G. 
Published: February 2007

AbstractSoluble leukocyte products harvested from incubated peritoneal exudate leukocytes, injected intravenously or intramuscularly, increased production of granulocytes and macrophages in marrow in diffusion chambers implanted into the peritoneal cavity of rabbits. Red cell production in the chambers was not consistently affected. Endotoxin increased production of all cell types. Endotoxin tolerance induced by daily injection of endotoxin to host rabbits abolished granulopoietic stimulation by endotoxin given during the culture period but did not diminish the granulopoietic stimulation produced by injected leukocyte products. Attempts to induce tolerance to leukocyte products by daily injections did not reduce the granulopoietic stimulation produced by either endotoxin or leukocyte products injected during the culture period. Intraperitoneally administered leukocytes products markedly inhibited production of all cell types.Endotoxin or leukocyte products given to normal rabbits increased plasma colony stimulating activity (CSA); the increase occurred sooner after leukocyte products than after endotoxin. Endotoxin‐tolerant animals showed no rise in CSA after endotoxin, but their response to leukocyte products was normal. Leukocyte products added to agar cultures neither supported nor inhibited colony growth but augmented CSA‐stimulated colony production. Endotoxin, leukocyte products, and CSA are different and may interact in regulating granulopoiesis.
The aplastic anemia‐paroxysmal nocturnal hemoglobinuria syndrome
Authors: Conrad Marcel E. , Barton James C. 
Published: February 2007

AbstractOccasionally it is difficult to differentiate paroxysmal nocturnal hemoglobinuria (PNH) from idiopathic aplastic anemia in patients who present with pancytopenia and an aplastic bone marrow. Patients with PNH may not have an abnormal acid hemolysis test, and patients with aplastic anemia may present with evidence of abnormal sucrose lysis, acid hemolysis, and antibody‐mediated complement hemolysis. Demonstration of a population of red blood cells which are highly susceptible to antibody‐mediated complement lysis makes a diagnosis of PNH probable. Donor red blood cell survival studies, which distinguish intracorpuscular from extracorpuscular hemolytic disorders, permit differentiation of the two disorders.
Iron chelation therapy in sickle cell anemia
Authors: Cohen Alan , Schwartz Elias 
Published: February 2007

AbstractIron stores, ascorbic acid levels, and urinary excretion of iron in response to deferoxamine (DFO) were studied in nine patients with sickle cell anemia who had received 8–55 liters of red blood cells. Ferritin levels ranged from 2, 168 to 6,300 ng/ml and were elevated even in patients who had normal serum iron concentrations. Leukocyte ascorbic acid levels were low in three patients. Urinary iron excretion in response to 2.0 gm DFO administered as a 12‐hour subcutaneous infusion after ascorbic acid supplementation was 13–39 mg/24 hours. With this method of DFO therapy daily, urinary iron excretion would exceed transfusional iron accumulation in eight of nine patients. Following the intramuscular injection of 0.75 gm DFO, urinary iron excretion was 5–16 mg/24 hours. Iron excretion would exceed iron accumulation in only one of nine patients with this method of DFO therapy. Urinary iron excretion in response to 1.5 gm DFO infused intravenously in 18 hours after ascorbic acid supplementation was 11–38 mg/24 hours. In six regularly transfused but nonchelated patients iron excretion in response to intravenous DFO had increased 46–107%, in comparison with previous studies 15–22 months before. These data indicate that negative iron balance can be obtained in regularly transfused patients with sickle cell anemia by the use of overnight subcutaneous infusions of DFO. Urinary iron excretion in response to DFO should be evaluated periodically in appropriate patients with sickle cell anemia to determine the proper time for the institution of chelation therapy.
Bleeding time in Uremia: A useful test to assess clinical bleeding
Authors: Steiner R. W. , Coggins Cecil , Carvalho Angelina C. A. 
Published: February 2007

AbstractModified Ivy bleeding time (template) and platelet aggregation to ADP, epinephrine, and collagen were studied in 26 uremic patients who had not recently ingested anti‐platelet drugs. Regardless of the aggregating agent used, the abnormalities in platelet aggregation were often mild, even with advanced uremia, and frequently less severe than the effects of common anti‐platelet drugs. The inhibition of collagen‐induced aggregation was significantly correlated with both increased bleeding time and blood urea nitrogen. Platelet aggregation was not discriminative between clinically bleeding and non‐bleeding groups of patients, but the bleeding time was helpful in this regard. In certain cases, the aggregometric patterns differed between drug‐induced and uremic thrombocytopathies. Platelet aggregometry appears to be of little help clinically in assessing the severity of the uremic bleeding diathesis.