bluuebunny/crossref_metadata_2025_split huggingface dataset:
Erythroid colony studies on sickle cell anemia in hypoproliferative crisis
Authors: Lutton J. D. , Schmalzer E. A. , Rao A. N. , Rao S. P. , Levere R. D. 
Published: February 2007

AbstractBone marrow cells, peripheral blood lymphocytes, and sera from patients with sickle‐cell anemia in hypoproliferative crisis were studied in the plasma clot culture system in the presence or absence of erythropoietin (Epo). Bone marrow cells from five patients demonstrated a marked ability to form erythroid colonies in the presence of Epo. These studies also suggested that bone marrow cells from some patients may have an increased sensitivity to Epo. The most outstanding observation in the present study was the marked erythroid colony inhibition by serum taken from one patient during crisis. Serum taken from the same patient two months after hypoproliferative crisis had no suppressive effect on erythroid colony formation. Lymphocytes taken from three patients in crisis had a stimulatory effect on erythroid colony formation when included in culture. The conclusion is that the defect of erythropoiesis in sickle‐cell anemia during hypoproliferative crisis is not due to the absence of erythroid precursor cells or to the presence of suppressor lymphocytes, but may in some cases be associated with a circulating inhibitor of erythroid maturation.
Studies on hemolytic anemia in pregnancy with evidence for autoimmunization in a patient with a negative direct antiglobulin (coombs') test
Authors: Yam Priscilla , Wilkinson Lee , Petz L. D. , Garratty George 
Published: February 2007

AbstractThree patients were studied who had acquired hemolytic anemia during pregnancy. One patient had a relapsing hemolytic anemia of pregnancy with a negative direct antiglobulin test. Previously reported cases have been presumed to be antibody‐mediated because of rapid destruction of transfused blood, transient hemolysis in the newborn, and a favorable response to corticosteroid therapy. Our findings with the complement‐fixation antibody consumption (CFAC) test offer support for an immune pathogenesis, since we documented abnormal concentrations of IgG on the patient's red cells during pregnancy and also on a sample of cord blood. The hemolytic anemia responded partially to prednisone during pregnancy and resolved postpartum. A repeat CFAC test postpartum revealed a marked reduction in the number of IgG molecules per red cell on the mother's cells, and IgG was no longer detectable on the infant's red cells.The other patients had serologic abnormalities characteristic of an autoimmune hemolytic anemia with an IgG warm autoantibody. The patients were followed closely during pregnancy because of previous reports of life‐threatening morbidity in mothers, as well as stillbirths, neonatal death, and seriously affected infants. An amniocentesis was performed in one patient because of persistent hemolysis in spite of prednisone therapy. The mothers and their infants did well, but serologic abnormalities and mild hemolytic anemia persisted in both mothers. Therefore, an elective splenectomy was performed with significant improvement in both instances.
Functional characteristics of red cells of sickle cell anemia patients with and without α‐thalassemia‐2
Authors: Abraham E. C. , Huisman T. H. J. 
Published: February 2007

AbstractOxygen equilibrium curves, red cell indices, 2,3‐DPG levels, and the percentages of ISC and Hb F were determined for red cell fractions isolated by Dextran 40 density gradient centrifugation from blood of a sickle cell anemia (SS) patient, an SS patient with an additional homozygosity for α‐thalassemia‐2, and a normal control. In the SS patient, the MCHC and P50 values increased and 2,3‐DPG levels decreased with an increase in red cell density; the P50 values were directly related to the MCHC values and inversely related to the 2,3‐DPG levels. The bottom fraction contained about 80% ISC, and had the highest MCHC and P50 values and the lowest 2,3‐DPG level. In the SS α‐thalassemia‐2 patient, a decrease in the P50 value corresponded with a decrease in 2,3‐DPG levels because no change in MCHC value was observed. The bottom fraction contained about 10% ISC and had the lowest P50 value. These data confirm that both MCHC and 2,3‐DPG levels influence the oxygen affinity of SS red cells; an increase in MCHC decreases the oxygen affinity to a great extent while a concomitant decrease in 2,3‐DPG level partially abolishes this effect.
Imbalance of T cell subpopulations in peripheral blood of children with non‐Hodgkin malignant lymphoma
Authors: Beck J. D. , Wollner N. , Miller D. R. , Good R. A. , Gupta S. 
Published: February 2007

AbstractPeripheral blood lymphocytes from 13 children with newly diagnosed untreated non‐Hodgkin malignant lymphoma were investigated for the proportion of Tμ and Tγ cells. Tumor involvement was classified as stage II in two patients, stage III in five patients, and stage IV in six patients. Diffuse poorly differentiated lymphoblastic histological‐type lymphomas, as defined by the Rappaport classification, were found in eight patients. The proportion of Tγ cells was found to be increased and that of Tμ cells decreased in the patient group as compared to levels in healthy controls. A marked imbalance of the Tμ/Tγ ratio was observed in ten of 13 patients. There was no correlation between Tμ/Tγ ratios and stage of the disease. Imbalances of Tμ and Tγ cell proportions are discussed in relation to humoral and cellular immunodeficiencies observed in patients with non‐Hodgkin malignant lymphoma.
Preselection of donors to improve the quality of cryoprecipitate
Authors: Tomasulo Peter A. , Richards William , Bailey Marilyn , Gajewski Mieczyslaw , Aster Richard H. , Lazerson Jack 
Published: February 2007

AbstractIn theory, the potency of cryoprecipitated antihemophilic factor (cryo) can be increased by utilizing plasma only from donors with high levels of factor VIII procoagulant activity (fVIIIc). Previous reports have suggested that plasma of group O individuals has, on the average, less fVIIIc than plasma from individuals of blood groups A, B, and AB. We have investigated the feasibility of increasing the potency of cryo by preselecting plasma for cryo production on the basis of the blood group of the donor. FVIIIc levels of fresh plasma, fresh frozen plasma (FFP), and cryo (prepared both by air‐thaw and water‐thaw methods) were assayed. The plasma, FFP, and cryo from group O donors contained only 75% of the fVIIIc present in plasma from group A, group B, and group AB donors. Following infusion to patients with hemophilia, in vivo recovery and survival of fVIIIc activity from groups A, B, and AB cryo reflected the higher in vitro levels. The mean potency of bags of cryo can be increased, and the cost of hemophilia treatment with cryo can be reduced by excluding group O plasma from the production of cryoprecipitate.
Concomitant presentation of two chronic leukemias: Evidence for independent clonal evolution
Authors: Vilpo J. A. , Klemi P. , Lassila O. , De La Chapelle A. 
Published: February 2007

AbstractA case of chronic lymphocytic leukemia (CLL) occurring simultaneously with Philadelphia chromosome‐positive chronic granulocytic leukemia (CGL) in a 58‐year‐old man is described. The patient presented with typical CGL (all bone marrow mitoses having a Philadelphia chromosome), but during the course of busulfan therapy, CLL became more prominent, less than half of the bone marrow mitoses having a Philadelphia chromosome. The patient is in good clinical condition after a follow‐up of 3.5 years, and the clinical picture resembles that of typical CLL.The coexistence of CGL and CLL here may be explained by chance alone. Evidently the histogenesis of two chronic leukemias in our patient took place as an independent evolution of two malignant hematopoietic clones. In spite of the frequent association of chronic leukemias with secondary cancer, no well‐documented cases of two chronic leukemias with a simultaneous presentation have been described previously.
Factor II antigen in liver disease and warfarin‐induced vitamin K deficiency: Correlation with coagulant activity using echis venom
Authors: Corrigan James J. , Earnest David L. 
Published: February 2007

AbstractFour coagulation factors (II, VII, IX, X) are synthesized in the liver as precursor forms. Vitamin K is necessary for their conversion to functional factors. Factor II precursor is antigenically similar to factor II and can be activated to form thrombin by Echis carinatus venom. Forty‐one patients with liver disease and 30 patients with vitamin K deficiency (Warfarin treated), were compared with 51 normal patients. Rats with CCl4‐induced hepatic necrosis and animals given Warfarin were also studied. The following measurements were made: standard prothrombin time; Echis clotting time; factor II coagulant assay with thromboplastin; factor II assay with Echis venom; and factor II antigen (human) by electroimmunoassay. In animals and humans with liver disease, factor II was reduced, as measured by all techniques. With vitamin K deficiency functional factor II was reduced, but factor II antigen and Echis factor II activity were normal. The data suggest that the prothrombin time and Echis coagulation methods can be used to differentiate whether the coagulopathy in liver disease is due to reduced precursor levels of factor II, vitamin K deficiency (ie, impaired formation of functional from precursor factor II), or both.
Von willebrand disease with an increased ristocetin‐induced platelet aggregation and a qualitative abnormality of the factor VIII protein
Authors: Takahashi Hoyu , Sakuragawa Nobuo , Shibata Akira 
Published: February 2007

AbstractTwo cases of von Willebrand disease (VWD) that revealed an increased ristocetin‐induced platelet aggregation (RIPA) and a qualitative abnormality of the factor VIII protein are reported. The threshold concentration of ristocetin giving a 30% increase in light transmission was 0.5 mg/ml in the proband and 0.4 mg/ml in her father (normal: 1.16 ± SD 0.18 mg/ml) although both patients showed reduced plasma von Willebrand factor activity (VIIIR:WF). In both patients, the amount of factor VIII related antigen (VIIIR:AG) in their platelets were normal, but an increased binding affinity of platelets to plasma factor VIII was demonstrated. The qualitative abnormality of the factor VIII protein was characterized by an increased anodal migration of VIIIR:AG in crossed immunoelectrophoresis (CIE), a delayed elution pattern as demonstrated by gel filtration on Sepharose 2B, and a decreased precipitation with concanavalin A (Con A). The response to DDAVP was also investigated.
Diarrhea in streptococcus pneumoniae bacteremia
Authors: Seeler Ruth Andrea , Jacobs Norman M. 
Published: February 2007

AbstractDiarrhea with fever was a significant complaint in 26 of 280 (9.3%) of non‐SS children with S. pneumoniae bacteremia and four of 30 (13%) children with sickle syndromes and S. pneumoniae bacteremia. Two additional children with sickle‐cell disease and S. pneumoniae bacteremia developed diarrhea within hours of hospital admission; four of the six died. The mortality rate among 24 additional SS patients with S. pneumoniae bacteremia without diarrhea was 12.5% (3/24). Only one of 26 non‐SS patients with diarrhea and S. penumoniae bacteremia died; she was surgically asplenic and had fulminant 9‐hour course. The seasonality of the diarrhea cases was similar to our overall experience, and the serotypes associated with the diarrhea cases were also representative of our overall experience. Although predominantly seen in infants, the oldest non‐SS patient was 12 years, and the oldest SS patient was nine years.
Sickle red cell calcium metabolism: Studies on Ca2+‐Mg2+ATPase and ca‐binding properties of sickle red cell membranes
Authors: Litosch Irene , Lee Kwang Soo 
Published: February 2007

AbstractSickle (Hb SS) red cells, preloaded with 45Ca by reversal of hemolysis, exhibit an incomplete 45Ca extrusion, retaining approximately four times more 45Ca than normal cells. Studies indicated that neither the reduction in Hb SS cell Ca2+‐Mg2+ ATPase activity (84% of normal) nor the activation of Ca2+‐Mg2+ ATPase by calmodulin was sufficiently different from normal cells to attribute a major role to the calcium pump in 45Ca retention. These results suggested that 45Ca retention may reflect an alteration in the calcium‐binding properties of Hb SS cell membranes. Low‐affinity calcium‐ binding (freely dissociable) was similar in normal and Hb SS cell membranes. However, the total calcium bound with high‐affinity (tightly bound) was four‐to‐five times greater in Hb SS cell membranes than in normal membranes. These results are compatible with the hypothesis that Hb SS cell 45 Ca retention reflects an exchange of a fraction of the total 45Ca with a tightly bound calcium pool, larger in Hb SS cell membranes than in normal membranes.A comparable degree of red cell 45Ca retention, which did not correlate with the reticulocyte population, was observed in other chronic anemic states. These findings suggest that the increased high‐affinity calcium binding by the membrane may be a consequence of cellular changes induced by the anemic condition.
An unusual factor‐X inhibitor in leprosy
Authors: Ness P. M. , Hymas P. G. , Gesme D. , Perkins H. A. 
Published: February 2007

AbstractTwo cases of Hansen disease demonstrating a lupus‐like inhibitor directed against the activation of coagulation factor X are described. Each case showed factor‐X activity markedly depressed by an inhibitor when measured in one assay system; yet normal levels were measured by three alternate factor‐X assay systems.
Intrinsic neutrophil defects in a child with impaired neutrophil chemotaxis and immunodeficiency
Authors: Morosawa Hironori , Yamazaki Munehiro , Sugita Kenichi , Saitoh Hiroharu , Komiyama Atsushi , Akabane Taro 
Published: February 2007

AbstractA boy with recurrent pyogenic infection was found to have occasional neutropenia, defective neutrophil chemotaxis, hypogammaglobulinemia with increased IgM, and impaired cellular immunity. The T and B lymphocytes were defective in IgG production in vitro. Ultrastructure of the neutrophils was normal. The marrow cells formed normal numbers of granulocytic colonies in culture, but the colonies were apparently small in size. The levels of colony‐stimulating activity were normal. The lymphocytes did not impair granulopoiesis of control marrow cells. These data indicate that the neutropenia and defective neutrophil chemotaxis are due to the intrinsic neutrophil defects and are not secondary to T and/or B lymphocyte dysfunctions in the patient.
Cutis verticis gyrata: Unusual chloromatous disease in acute myelogenous leukemia
Authors: Cheson Bruce D. , Christiansen Robert M. 
Published: February 2007

AbstractChloromas are an unusual finding in patients with acute or chronic granulocytic leukemia. The present report describes a man with acute myelogenous leukemia (AML) whose course was complicated by cutis verticis gyrata (CVG), a mediastinal mass, neuropathies, and hepatosplenomegaly as a result of visceral involvement. Leukemic tumors and organ infiltration developed independent of his hematologic status and resulted in significant morbidity and, eventually, mortality from gastrointestinal hemorrhage. Nevertheless, at least temporary control over these complications was achieved when they were managed as leukemic “sanctuaries” and treated with local irradiation.
Fibrinogen chapel hill: Hypodysfibrinogenemia with a tertiary polymerization defect
Authors: McDonagh R. P. , Carrell N. A. , Roberts H. R. , Blatt P. M. , McDonagh Jan 
Published: February 2007

AbstractA patient with a functionally defective fibrinogen (fibrinogen Chapel Hill) has been investigated. Fibrinogen Chapel Hill is characterized by hypofibrinogenemia, with a plasma concentration about one third of normal, as measured both functionally and immunochemically. Fibronogen survival is normal; so also is fibrinopeptide release. A polymerization defect in this fibrinogen results in the delay of fibrin fibrils in solution to form a normal three‐dimensional gel. This defect is not associated with end‐to‐end aggregation or with lateral associations in solution. Delayed gelation results from an abnormality in a tertiary contact site involved in network branching, which is associated with the hydrophilic, carboxy‐terminal segment of the α chain. Fibrinogen Chapel Hill exhibits two additional abnormal responses, which are also associated with the same region. The early plasmin cleavages of fibrinogen and fragment X are delayed, and there is a concomitant delay in the appearance of fragments Y, D, and E. This fibrinogen also has an unusual sensitivity to Ancrod proteolysis, whereby Ancrod cleaves a large carboxy‐terminal segment of the α chain more rapidly than in normal fibrinogen. The abnormalities in fibrinogen Chapel Hill can be explained by a structural abnormality which is functionally related to an α chain associated polymerization domain.
Von willebrand factor (VIIIVWF) in lyophilized factor VIII concentrates
Authors: McCue Miles J. , Brossoit Arthur D. , Marmer Daniel J. , Head David R. 
Published: February 2007

AbstractSeveral (14) commercially prepared, lyophilized Factor VIII concentrates were assayed for VIIIAHF, VIIIAg, and VIIIVWF. Significant levels of VIIIAg (immunologic) and VIIIVWF (von Willebrand Factor) were present in addition to VIIIAHF (Functional VIII, Antihemophilic Factor, Factor VIII coagulant activity). An average of 0.95 units of VIIIVWF (range 0.47–1.7) were present for every 1.0 unit of VIIIAHF (as stated on the manufacturer's label). We suggest that the various lyophilized preparations of Factor VIII concentrate available could be assayed for VIIIVWF and should be tested therapeutically in patients with von Willebrand disease.
Acute lymphoblastic leukemia in idiopathic refractory sideroblastic anemia: Evidence for a common lymphoid and myeloid progenitor cell
Authors: Barton James C. , Conrad Marcel E. , Parmley Richard T. 
Published: February 2007

AbstractAcute leukemia of myeloblastic or erythroblastic morphology occasionally occurs as a complication of idiopathic refractory sideroblastic anemia, but the development of acute lymphoblastic leukemia has not been previously reported in these cases. A patient with idiopathic refractory sideroblastic anemia is described in whom acute lymphoblastic leukemia occurred. The leukemic cells were characterized by typical lymphoblastic morphology on Wright's stain, periodic acid‐Schiff‐positive cytoplasmic clumps, elevated levels of deoxynucleotidyl transferase (143 units/108 cells), high numbers of specific glucocorticoid binding sites (16,845 sites/cell, Kd = 5.40 × 10−9 M), non‐B, non‐T cell immunologic characteristics and clinical responsiveness to therapy with vincristine, prednisone, and methotrexate. Ultrastructural studies of the lymphoblasts identified ferruginous material in lysosomes and occasional mitochondria similar to but less abundant than that seen in abnormal sideroblasts. The concurrence of these two disorders supports the theory that in humans both lymphoid and myeloid cell lines arise from a common pluripotent stem cell.
Provoked priapism in sickle cell anemia
Authors: Conrad Marcel E. , Perrine George M. , Barton James C. , Durant John R. 
Published: February 2007

AbstractTwo patients with sickle cell anemia are reported who developed relative impotence after repeated episodes of priapism. They learned that excessive ethanol ingestion produced an erection and utilized this mechanism to satisfy sexual partners. Hospitalization and transfusion therapy were required for repeated episodes of priapism. Extensive interviews were required in order to learn the sequence of events. This synergistic relationship between ethanol and priapism should be sought in patients with repeated hospitalizations for this complication of sickle cell anemia because it is preventable with appropriate counseling.
The synthesis of the Gγ and Aγ chains of human fetal hemoglobin in erythroid colonies cultured from peripheral blood BFUe's of normal adults and newborn and of subjects with an Aγ or a Gγ chain abnormal fetal hemoglobin
Authors: Huisman T. H. J. , Reese A. L. , Gravely M. E. , Harris H. , Wilson J. B. 
Published: February 2007

AbstractPeripheral blood mononuclear cells from normal adults, normal newborn infants, and from newborn and adult subjects with one of three γ chain variants (GγF‐Malta I, GγF‐Port Royal, AγF‐Hull) were cultured in vitro with erythropoietin. The 35S‐methionine‐labelled hemoglobin from 13‐ to 15‐day‐old BFUe‐derived colonies was studied by chromatography on columns of DEAE‐cellulose and the quantities of Hbs A2, Fx, and F0 determined. The percentages of Gγ and Aγ chains in isolated Hb Fx and Hb F0 were determined using high‐performance liquid chromatography (HPLC) of the tryptic peptides of these proteins. Calculation of these percentages was based on the total activities of the GγT‐15 and AγT‐15 peptides which contain one (35S‐labelled) methionyl residue each and can be separated by the HPLC procedure.The data show an increased synthesis of Hb F in the „adult”︁ colonies and a decreased synthesis in the „newborn”︁ colonies. The Gγ to Aγ ratio of the Hb F from adult colonies varied greatly. The percentages of Gγ and Aγ chains in the Hb F from adult colonies correlated with the percentages in the Hb F isolated from the Hb F of circulating red blood cells. The Gγ to Aγ ratio in the Hb F from newborn colonies was high as in the Hb F from cord blood samples.Gγ and Aγ chain abnormal Hb F variants were readily detectable in colonies from both adults and newborn. The Gγ to Aγ ratio in the Hb F of colonies of adult Hb F‐Malta I and Hb F‐Hull heterozygotes approached 1, but that of adult Hb F‐Port Royal heterozygotes remained about as high as in colonies from newborn heterozygotes. The percent Hb F‐Port Royal in the Hb F of adult colonies was twice that in the Hb F of newborn colonies.These results are discussed in the light of information from recent detailed studies of genomic DNA assuming controlling functions for the segments of DNA that are interspersed between the various structural genes.
Erythrocytes as carriers of chemotherapeutic agents for targeting the reticuloendothelial system
Authors: Lynch William E. , Sartiano George P. , Ghaffar Abdul 
Published: February 2007

AbstractThe object of this work was to define a model using hypotonically loaded erythrocytes as a vehicle to target drugs to the reticuloendothelial system (RES). The optimum hemolytic event was found to occur at 100 mOsm/kg using a 0.5‐min exposure at 0°C. Approximately one third of the total volume of the cells could be replaced with hypotonic drug solutions under these conditions. Although cytosine‐β‐D‐arabinofuranoside, ara C, is membrane permeable and could not be entrapped in the erythrocytes, phosphorylation of this nucleoside antimetabolite enabled it to be loaded efficiently. Actinomycin D could be loaded and retained within the cells at 0°C, but 90% of this loaded drug leaked out of the erythrocytes in 1 min at 37°C. Actinomycin D‐DNA complexes, however, could be loaded and retained for longer periods. In this case, 50% of the DNA‐bound drug was retained in the cells for one hour at 37°C. It was found that the glycopeptide antitumor antibiotic, bleomycin, could be entrapped and retained in the cells without appreciable leakage. It was possible to load a human therapeutic dose of this drug in 1–2 ml of packed cells. Furthermore, it was demonstrated that bleomycin entrapped in erythrocytes was significantly more effective than the same dose of free drug in suppressing the phagocytic function of the RES in Balb/C and C3H mice. The rationale is discussed for the possible use of these drugs, entrapped in erythrocytes, for the production of RES blockade in the treatment of disorders in man.