bluuebunny/crossref_metadata_2025_split huggingface dataset:
Increased prevalence of polycythemia vera in parents of patients on polycythemia vera study group protocols
Authors: Brubaker Leonard H. , Wasserman Louis R. , Goldberg Judith D. , Pisciotta Anthony V. , McIntyre O. Ross , Kaplan Manuel E. , Modan Baruch , Flannery John , Harp Rollie 
Published: February 2007

AbstractAn investigation of relatives of 652 patients entered on studies of the Polycythemia Vera Study Group yielded five documented cases of the disease among the parents of patients. When compared with expected values based on the Connecticut Tumor Registry and other population studies a significant increase was found in the lifetime incidence of polycythemia vera in parents of these patients.
The relationship between fetal hemoglobin level and glycosylation in sickle cell disease
Authors: Elseweidy M. M. , Abraham E. C. 
Published: February 2007

AbstractGlycosylated hemoglobin (glyco Hb) was determined by affinity chromatography and Hb S1 and Hb F by Bio‐Rex 70 chromatography in patients with sickle cell anemia, SC disease, Sβ+‐thalassemia, and Sββ‐thalassemia. SC and Sβ‐thalassemia patients had normal levels of glyco Hb whereas SS patients had significantly lower levels. Within each group of patients a direct correlation existed between Hb F and glyco Hb or Hb S1 levels. A similar relationship was noticed when glyco Hb and Hb F levels were compared in red cell populations of various densities (ages). Hb F seems to influence glycosylation through its effect on red cell survival.
Peripheral blood lymphocytes with receptors for a determinant common to B‐lymphoblastoid cell lines and acute myelogenous leukemia blasts
Authors: Lichtenstein Alan , Naiem Faramarz , Zighelboim Jacob 
Published: February 2007

AbstractWe investigated whether the rosetting of B‐lymphoblastoid cell lines (B‐LCL) by peripheral blood lymphocytes (PBLs) reflected possible interactions between lymphoid cells and immature cells of the hematopoietic system. Rosette formation could be blocked by the addition of soluble antigen extracted from B‐LCL or blasts obtained from patients with acute myelogenous leukemia (AML). This inhibition was specific for AML blasts (similarly extracted material from melanoma lines had no inhibitory effect) and for the B‐LCL receptor (leukemic extracts had no effect on surface receptors for sheep red blood cells (E) or antibody‐sensitized red blood cells (EA)). The B‐LCL receptor is present on leukemic Sezary T‐cells as well as normal T‐cells and its sensitivity to various enzymatic treatments is markedly different from that of E and EA receptors. In addition, B‐LCLs derived from in vitro EB‐viral infection of a normal donor's B lymphocytes were significantly rosetted by that donor's autologous PBLs. These data suggest the B‐LCL receptor, present on mature T‐cells, can recognize self determinants on myeloblasts and B‐LCL. Further investigation will determine whether this interaction can affect the function of rosetted target cells.
Pancytopenia with hypercellular bone marrow—a possible paraneoplastic syndrome in carcinoma of the lung: A report of three cases
Authors: Raz Itamar , Shinar Eilat , Polliack Aaron 
Published: February 2007

AbstractThree patients with idiopathic pancytopenia and hypercellular bone marrow who developed carcinoma of the lung within two years of diagnosis are reported. All three patients had macrocytic anemia associated with a megaloblastic marrow in the presence of normal serum vitamin B12 and folic acid levels. Neutropenia with monocytosis, elevated serum muramidase and LAP scores, and increased fetal hemoglobin levels were also found. In all cases Ham's tests were negative with a normal bone marrow karyotype. In all three patients, pancytopenia due to myelodysplasia, a probable preleukemic state, was diagnosed initially prior to the appearance of carcinoma of the lung.One of the patients showed improved leukocyte and platelet counts during chemotherapy, while the other two died before chemotherapy could be administered. In the light of the above findings we suggest that carcinoma of the lung may be the cause of a paraneoplastic syndrome with pancytopenia, particularly in patients with a hypercellular marrow with a normal karyotype.
Functional diversity within the suppressor phenotype as defined by monoclonal antibody in T‐cell prolymphocytic leukemia
Authors: Zamkoff Kenneth W. , Poiesz Bernard J. , Ruscetti Francis W. , Moore Janet L. , Davey Frederick R. , Planas Antonio T. , Lamberson Harold 
Published: February 2007

AbstractA patient with T‐cell prolymphocytic leukemia (T‐PLL) is described. The malignant T‐cells from the patient were predominantly Leu‐2‐positive, indicating a suppressor phenotype. The cells were then tested to determine their functional capabilities. The patient's Leu‐2‐positive cells initially suppressed B‐cell proliferation, as predicted by their phenotype but later functioned as T helper cells in the pokeweed mitogen system without a change in phenotype. The cells also responded inadequately to alloantigen and mitogen despite addition of exogenous T‐cell growth factor (TCGF). Leu‐2‐positive prolymphocytes from the spleen of the patient were constitutive producers of TCGF. Surface phenotype using monoclonal antibody was inadequate to predict T‐cell function of the cells from this patient with T‐PLL. In addition, these data suggest there may be functional subpopulations within the OKT8 + phenotype. Constitutive TCGF production by malignant post‐thymic T‐cells may represent a mechanism by which these cells sustain their own growth.
Increase in density and accumulation of serotonin by human aging platelets
Authors: Mezzano Diego , Aranda Eduardo , Rodríguez Soledad , Foradori Arnaldo , Lira Pablo 
Published: February 2007

Abstract51Cr‐labeled autologous platelets were infused into splenectomized subjects and the specific radioactivities of high‐density (HD) and low‐density (LD) platelet subpopulations were determined sequentially in postinfusion samples. A rapid decrease in the specific radioactivity of LD cohorts (T 1/2 = 2.5 days) was observed, but the specific radioactivity of HD platelets remained constant or increased slightly during the first 4 days and then gradually declined for the next 5 days. No experimental artifacts during the platelet‐labeling steps that could account for these results were demonstrated. These findings confirm previous observations in eusplenic individuals and support the hypothesis that human LD platelets are, on the average, younger than HD platelets.LD platelets contain 33.8 ± 13.5 ng serotonin (5HT)/108 platelets and HD platelets 76.8 ± 9.5 ng 5HT/108 platelets (P < 0.001). Sequential measurements of 5HT in PRP platelets were performed during the recovery phase of thrombocytopenia following splenectomy in patients with idiopathic thrombocytopenic purpura (ITP), a condition associated with aging of platelets in circulation. Presplenectomy platelet 5HT was 17.7 ng/108 platelets and on days 1, 6, and 12 after surgery it increased to 18.1, 37.8, and 61.0 ng/108 platelets (n = 7). When three healthy volunteers were given aspirin (500 mg/day) for up to 15 days, no significant change in the 5HT content of circulating platelets was observed. If aspirin blocks, at least partially, the secretory process in vivo without interfering the 5HT uptake by the platelets, this finding stands against the possibility that a net depletion of 5HT occurs during the life‐span of normal human platelets.The observation that human HD platelets, enriched with older cells, contain more 5HT than LD platelets taken together with the parallel increase in platelet 5HT and age during the recovery from thrombocytopenia in ITP patients and the lack of effect of aspirin on platelet 5HT content, provides initial evidence that human platelets accumulate 5HT during their life‐span in circulation.
Kinetics and distribution of platelets in man
Authors: Tsan Min‐Fu 
Published: February 2007

Abstract51Cr sodium‐chromate, though having been widely used in the last two decades for labeling platelets, suffers from several serious drawbacks, ie, low labeling efficiency, long physical half‐life, and low gamma photon yields. 111In‐oxine and 111In‐tropolone overcome these shortcomings and have the potential of precise determination of platelet kinetics as well as visualization and in vivo quantification of the temporal and spatial distribution of platelets in man. Computer analysis of platelet kinetics reveals that the multiple‐hit model fits the survival curve better than the linear or the exponential model. The multiple‐hit model provides not only the mean platelet survival time but also information on the initial recovery and the shape of the survival curve. The application of these techniques in normal and disease states should greatly enhance our understanding of the physiology and pathophysiology of platelets.
Familial hereditary thrombocytopenia and HLA
Authors: Naparstek Yaakov , Abrahamov Ayala , Cohen Tirza , Brautbar Chaim 
Published: February 2007

AbstractThree generations of a Jewish family with hereditary thrombocytopenia (HT) are described. The disease was manifested clinically by mild bleeding tendency since infancy. Circumcision, however, did not result in excessive bleeding. HLA study in this family indicated that the HT locus is not linked to HLA.
Disappearance of microspherocytes in peripheral circulation and normalization of decreased lipids in plasma and in red cells of patients with hereditary spherocytosis after splenectomy
Authors: Sugihara Takashi , Miyashima Kosuke , Yawata Yoshihito 
Published: February 2007

AbstractA possible mechanism of the formation of microspherocytosis in unsplenectomized patients with hereditary spherocytosis (HS) was investigated in relation to lipid metabolism in plasma and in red cells of these patients. Plasma lipids (total cholesterol, free cholesterol, high density lipoprotein cholesterol, free fatty acids, and phospholipids) were markedly reduced in unsplenectomized HS patients with microspherocytosis. Red cell membrane lipids (free cholesterol and phospholipids such as phosphatidyl ethanolamine, sphingomyelin, phosphatidyl choline, and lysophosphatidyl choline) were also decreased in these unsplenectomized HS patients. After splenectomy, microspherocytosis disappeared concomitant to substantial normalization of plasma and red cell membrane lipids. These observations suggest that plasma lipid decrement in the unsplenectomized HS patients is at least one of the causative factors in pathogenesis of the formation of microspherocytosis. So‐called “splenic conditioning” may not imply a physical “loss” of membrane by fragmentation or pitting of the once‐formed HS red cells in the peripheral circulation. Instead, the decreased plasma lipids in the presence of the spleen may affect the de novo synthesis of red cell membrane lipids, resulting in the formation of microspherocytosis.
Platelet‐activating factor is a weak platelet agonist: Evidence from normal human platelets and platelets with congenital secretion defects
Authors: Rao A. Koneti , Willis Janet , Hassell Barbara , Dangelmaier Carol , Holmsen Holm , Smith J. Bryan 
Published: February 2007

AbstractWe have examined the effects of a novel platelet agonist, platelet activating factor (PAF), on human platelets. Irreversible aggregation and14C‐serotonin secretion in response to PAF (10−5M) was found to be dependent on both thromboxane production and secreted adenosine diphosphate (ADP). Liberation of arachidonic acid (AA) from membrane‐bound phospholipids is a prerequisite step in platelet thromboxane production. Studies with3H‐AA‐labeled platelets revealed that PAF (10−5M) was a weak stimulus for the mobilization of AA. In addition, PAF (10−5M) was found to be a weak inducer of thromboxane synthesis (mean = 6 pmol/108platelets) as compared to thrombin 5 U/ml (mean = 177 pmol/108platelets), measured using a radioimmunoassay for thromboxane B2. Formation of phosphatidic acid is an early step in stimulus‐response coupling in platelets. Our studies indicate that PAF is a weak stimulus for phosphatidic acid formation as well. To obtain further insights into its action, we examined the effect of PAF on platelets from three groups of patients with congenital secretion defects: patients with the storage pool deficiency, those with impaired thromboxane synthesis due to impaired liberation of AA from phospholipids, and those with impaired secretion despite normal granule stores and thromboxane production. The response to PAF was impaired in all patients, providing further evidence that PAF‐induced platelet activation is dependent on secreted ADP and thromboxane A2synthesis, and occurs by mechanisms common to a number of agonists. Overall, these studies indicate that PAF is a weak platelet agonist.
Exaggeration of defective platelet function by methyldopa in hypothyroidism
Authors: Zeigler Zella , Hasiba Ute , Lewis Jessica , Vagnucci Anthony , West Valerie , Bezek Elizabeth 
Published: February 2007

AbstractPlatelet function was analyzed in two hypothyroid patients who were taking methyldopa. Results from these two patients are compared to those obtained in seven others with hypothyroidism on no medications. Bleeding times in the two drug patients were 33 and 26 minutes compared to normal values (4.0 to 8.0 minutes) in the other hypothyroid individuals. In addition, platelet aggregation responses to epinephrine, collagen, and ristocetin were abnormal in these two patients; these were normal in the others with hypothyroidism. Repeat studies were done in the first drug patient when she was euthyroid and still receiving methyldopa. Her bleeding time decreased to 11 minutes, platelet retention increased from 31% to 75%, and abnormal aggregation responses corrected to normal. Factor VIII coagulant activity, VIII antigenic activity, VIII ristocetin cofactor activity, and two‐dimensional immunoelectrophoresis of VIII antigen were normal. These findings suggest that methyldopa can potentiate the mild platelet function defect seen in patients with hypothyroidism.
Erythrocytic pyruvate kinase deficiency and hemolytic anemia inherited as a dominant trait
Authors: Etiemble Jeanne , Picat Christiane , Dhermy Didier , Buc Hélène A. , Morin M. , Boivin Pierre 
Published: February 2007

AbstractA nonspherocytic hemolytic anemia, associated with a pyruvate kinase (PK) deficiency apparently inherited as a dominant trait has been identified in a family. In the affected members, the residual PK activities were 20% that of normal controls, an unusually low level for heterozygous subjects. The anemia was mild except in the proband, a 2‐year‐old boy who suffered from a severe anemia.The PKs of the proband and of his both parents have been characterized as kinetically and electrophoretically normal enzymes. Immunoprecipitation tests indicated a large amount of L‐type inactive protein in the proband and his father. Moreover, an M2 type PK was present in the father's hemolysate, suggesting the existence of a compensatory process that derepressed the corresponding structural gene. We suggest that the presence of one or more mutated subunits in the tetrameric forms of L‐type PK leads to the inactivation of these tetramers. This hypothesis accounts for the low residual activity in affected heterozygous members of this family. The severity of the hematological symptoms in the proband, in comparison with the mild hemolysis observed in the other heterozygous members of the family points to the existence of a large spectrum of pathologic expression for an identical PK defect present in a family.
Kinin cleavage by human erythrocytes
Authors: Sidorowicz W. , Canizaro Peter C. , Běhal Francis J. 
Published: February 2007

AbstractAn aminopeptidase‐P has been purified 230‐fold from human erythrocytes. The purified enzyme cleaved arginine from des‐(Arg9)‐bradykinin (Arg‐Pro‐Pro‐Gly‐Phe‐Ser‐Pro‐Phe) had a molecular weight in nondenaturing buffers of 155,000 ± 6,900 daltons, was not inactivated by chelating agents, had a pH optimum of 7.2, and was stimulated by manganous ions. The aminopeptidase‐P was stable in intact erythrocytes for at least 21 days. Extensively washed and intact human erythrocytes cleaved arginine from exogenously supplied des‐(Arg9)‐bradykinin; arginine was the earliest‐appearing reaction product. Purified aminopeptidase‐P also cleaved a group of X‐proline dipeptides including leucyl‐proline, methionyl‐proline, phenylalanyl‐proline, arginyl‐proline, and alanyl‐proline. The total intra‐erythrocytic aminopeptidase‐P activity of the “average 70‐kg man” was 2,600 units, approximately five times the amount of activity in the total lung mass. The human erythrocyte aminopeptidase‐P activity was not tightly bound to the erythrocyte membrane. Intact erythrocytes also exhibited some kinin‐converting enzyme activity.
Procainamide‐induced agranulocytosis with reversible myeloid sensitivity
Authors: Nelson John C. , Lutton John D. , Fass Arthur E. 
Published: February 2007

AbstractA 56‐year‐old man developed procainamide‐induced agranulocytosis. Bone marrow aspiration showed the absence of myeloid elements beyond the promyelocyte stage. Procainamide at therapeutic concentrations in vitro depressed bone marrow granulocyte‐macrophage colonies (CFUc) while CFUc growth was normal in the absence of drug. Following the patient's recovery, CFUc growth was no longer suppressed by procainamide in vitro. The observations in this patient showing reversible sensitivity to drug contrast with those on other patients with drug‐induced agranulocytosis that show persistent marrow injury or drug sensitivity despite clinical recovery.
Lymphoma of host origin in a marrow transplant recipient in remission of acute myeloid leukemia and receiving cyclosporin A
Authors: Bloom Robert E. , Brennan James K. , Sullivan John L. , Chiganti R. S. K. , Dinsmore Robert , O'Reilly Richard 
Published: February 2007

AbstractLymphomas are an uncommon complication of solid organ transplantation and rarely occur after marrow transplantation. When post‐marrow transplant lymphomas have occurred, they have been of donor cell origin and when sought, Epstein‐Barr virus DNA has been found in the tumor. A 21‐year‐old woman developed a poorly differentiated lymphocytic lymphoma 6 months after bone marrow transplantation for acute myeloid leukemia in remission. Cyclosporin A had been used as an immunosuppressant. A chromosomal polymorphism demonstrated that the tumor was of host origin and contained a monoclonal tumor marker, 46, XX INV 4 (p16q12). The tumor did not contain the DNA of the Epstein‐Barr virus.
Relationship between patients' age, bone marrow karyotype, and outcome of induction therapy in acute myelogenous leukemia
Authors: Bernard Ph. , Lacombe F. , Reiffers J. , David B. , Marit G. , Bourdeau M. J. , Broustet A. 
Published: February 2007

AbstractBone marrow karyotypes were performed in 88 cases of adult acute myelogenous leukemia (AML) at diagnosis and classified NN (normal), AA (abnormal), and AN (mixture of normal and abnormal metaphases). A clear relationship was found between karyotype and complete remission (CR) rate: 58% CR in (NN + AN) cases; 14% CR in AA cases (P < .009). This relationship was even stronger when only patients under 60 years of age were studied. Considering failures of induction treatment, no relationship was found between the NN/AN/AA classification and a drug resistance. In patients over 60, the worse prognosis could be explained by an inferior ability to tolerate intensive treatment.
Rapid accurate absolute granulocyte count determination during severe leukopenia using the flow cytometer
Authors: Johnson Karen L. , Dougherty Carol , Vaughan William P. 
Published: February 2007

AbstractThe absolute granulocyte count (AGC) in 125 blood samples from patients with total white blood cell counts of less than 1,000/μl was estimated using three different methods, which were then compared for efficiency and accuracy. The three methods were 25 cell differential counts using Wright's‐stained blood smears, granulocyte percentage estimates from WBC counting chambers, and combined narrow‐ and wide‐angle light‐scatter characteristics determined on a flow cytometer. A survey of clinical laboratories at University Hospital Cancer Centers revealed that the smear differential was the most‐often‐used method in those laboratories even when less than 25 cells could be counted. Consequently the data obtained from the counting chamber and flow cytometer methods were compared to the smear differential “standard” using linear regression, and outliers were identified. There was good correlation between AGC determined by smear differential and WBC counting chamber (correlation coefficient .911) and excellent correlation between the AGC determined by smear differential and the flow cytometer method (correlation coefficient .970). The flow cytometer method used in this investigation required minimal specimen preparation, and test results were available at a rate of 60 seconds/sample. The ease of sample preparation, speed, and statistical reliability of test results makes the flow cytometer an attractive alternate method of determining granulocyte counts on leukopenic patients as compared to the stained blood smear differential.
Lack of relationship between in vitro cell measurements and response to busulfan in chronic myelocytic leukemia
Authors: Kirshner Jeffrey , Preisler Harvey D. , Raza Azra 
Published: February 2007

AbstractTwenty‐three patients with chronic‐phase CML have been treated with intermittent courses of busulfan to determine if the duration of unmaintained remission becomes progressively shorter with successive courses of therapy and to determine whether there was a relationship between cluster and colony formation, suicide index of CFUc, labeling index, percentage of cells in S‐phase (as determined by cytofluoro‐graphic DNA histogram analysis), in vitro sensitivity of the CFUc to busulfan, and response to busulfan therapy. Serial studies in individual patients and the group as a whole revealed no relationship between changes in the cellular parameters described above and response to busulfan. The white blood cell (WBC) doubling time with serial courses of busulfan in individual patients did not always progressively decrease as has been previously reported. In vitro studies of CFUc in chronic‐phase CML appear to be of no value in predicting response to busulfan.
Extravascular hemolysis following the administration of cefamandole
Authors: Branch Donald R. , Berkowitz Lee R. , Becker Robert L. , Robinson Judy , Martin Marsha , Gallagher Michael T. , Petz Lawrence D. 
Published: February 2007

AbstractHemolytic anemia occurred in a 70‐year‐old female after a five‐day course of intravenous cefamandole. The patient's serum contained an IgG antibody which was reactive with red blood cells which had been coated in vitro with cefamandole but not with uncoated cells. An in vitro assay of allogeneic mononuclear phagocytosis of cefamandole‐coated red cells sensitized with the patient's anticefamandole indicated that the anticefamandole could induce significant phagocytosis. The anticefamandole was easily inhibited in vitro by cefamandole as well as by a variety of related cephalosporins indicating broad cross‐reactivity, with the antigenic site primarily the 7‐amino‐cephalosporanic acid nucleus. Penicillins could inhibit the anticefamandole but only when using concentrations 3‐10× those of cephalosporins. Eleven examples of antipenicillin tested failed to react with cefamandole‐coated red cells. Screening of 344 random sera from hospitalized patients found only five (1.5%) reactive with cefamandole‐coated red cells; three of these sera were also reactive with penicillin‐coated red cells. The patient's hemolysis subsided following cessation of the drug. This is the first report of anticefamandole‐induced hemolytic anemia.