bluuebunny/crossref_metadata_2025_split huggingface dataset:
Natural history of acquired immunodeficiency syndrome in hemophilic patients
Authors: Kim Hugh C. , Nahum Kenneth , Raska Karel , Gocke David J. , Kosmin Martin , Karp George I. , Saidi Parvin 
Published: February 2007

AbstractDuring the 5‐year period from 1981 to 1985, we have observed 8 cases of acquired immunodeficiency syndrome (AIDS) among our 85 patients with hemophilia A. Thus, the prevalence of AIDS with hemophilia A is 9.4% in our patient population. By utilizing stored serum or plasma samples dating back to 1978, antibody against HTLV‐III was detected in all 8 cases with AIDS. Based on the time interval from the appearance of antibody to HTLV‐III to the diagnosis of AIDS in these patients, the incubation period ranged from 27 months to 60 months, with a median of 36 months. Before the diagnosis of full‐blown AIDS, all patients exhibited a variety of prodromal manifestations of non‐specific nature, including weight loss, oral candidiasis, unexplained non‐productive chronic cough, generalized lymphadenopathy, and thrombocytopenia lasting several months to several years. Serial T‐lymphocyte subset studies were available in some patients during the HTLV‐III seropositive period and showed progressive lymphopenia, depletion of T4 cells with an average absolute count of 94 ± 128 per mm3 (mean ± 1 S. D.), and a markedly reversed T4/T8 ratio of 0.26 ± 0.19 (mean ± 1 S. D.). These findings suggest that the incubation period of AIDS is considerably long and that prospective study of serial immunologic markers and HTLV‐III markers may be warranted in hemophilic patients at risk.
Immunogold staining of microtubules in resting and activated platelets
Authors: Escolar Gines , Sauk John , Bravo Maria Luisa , Krumwiede Marlys , White James G. 
Published: February 2007

AbstractA circumferential microtubule is known to support the discoid form of resting platelets, but its fate following exposure of the cells to aggregating agents is uncertain. The present study has employed an immunocytochemical approach to follow the fate of the circumferential microtubule in activated platelets. Monoclonal antibodies to tubulin and to vinculin and a polyclonal antibody to actin were incubated with isolated microtubule coils and stained with staphylococcal protein A coupled to immunogold in order to test their specificity. Thin sections of glycolmethacrylate embedded platelets before and after exposure to thrombin for 15, 30 and 60 s were stained with antibodies to tubulin and actin. Immunogold particles showed a high specificity for isolated MT coils stained for tubulin, modest intensity for actin, and none for vinculin. Gold particles were randomly distributed in thin sections of resting and activated platelets stained for actin. Immunogold was limited to the circumferential microtubule in resting platelets and constricted coils in thrombin‐activated cells. The number of gold particles in areas of cytoplasm away from microtubules in platelets stained with antitubulin antibody increased slightly following thrombin activation, but the change was not significant. Results support the concept that microtubule coils supporting the discoid form of resting platelets do not dissolve following exposure of the cells to potent agonists.
Discordance in a pair of identical twin carriers of factor IX deficiency
Authors: Kitchens Craig S. 
Published: February 2007

AbstractFemale twin daughters of a man with hemophilia B were determined to be identical but widely discordant with respect to their degree of factor IX deficiency. The proposita had a marked deficiency (3%) of factor IX with clinical bleeding. She became pregnant. Delivery was managed with infusion of factor IX concentrates. These twins represent the second reported case of identical twins with discordant factor IX levels.
Combination of three α‐globin gene loci deletions and hemoglobin new york results in a severe hemoglobin H syndrome
Authors: Chan Vivian , Chan T. K. , Tso S. C. , Todd D. 
Published: February 2007

AbstractA Chinese patient with hemoglobin (Hb) H and Hb New York is described. Unlike classical Hb H disease, the additional βNew York chain defect resulted in a more marked α β 3‐globin chain imbalance in this patient with only one α‐gene, accounting for her severe anemia (3.4‐6.8 g/dl) during the steady state.
Acute lymphocytic leukemia: Correlation of clinical features with immunocytochemical classification
Authors: Twu Bair‐Her , Li Chin‐Yang , Smithson William A. , Clark Hoagland H. , Dewald Gordon W. 
Published: February 2007

AbstractMany immunologic studies of acute lymphocytic leukemia (ALL) during the past decade have demonstrated the close correlation of immunologic phenotypes of ALL subclasses with the clinical presenting features and prognosis. However, the clinical application of conventional immunologic techniques had been very limited because of the requirement of a fresh sample to prepare the mononuclear cell suspensions for study. We studied 81 cases of ALL using immunoperoxidase stain for nuclear terminal deoxynucleotidyl transferase (TdT) and immunoalkaline phosphatase stain for surface markers (using monoclonal antibody J5 for common ALL antigen [CALLA], Leu‐1 for pan‐T antigen, and B1 for pan‐B antigen) on air‐dried smears. The cases were classified as common ALL (TdT+, CALLA+, pan‐T−, and pan‐B−) (41 cases), null‐ALL (TdT+, CALLA−, pan‐T−, and pan‐B−) (19 cases), T‐ALL (TdT+, CALLA−, pan‐T+, and pan‐B−) (nine cases), B‐ALL (TdT−, CALLA−, pan‐T−, and pan‐B+) (six cases), pre‐B‐ALL (TdT®, CALLA+, pan‐T−, and pan‐B+) (four cases), or pre‐T‐ALL (TdT+, CALLA+, pan‐T+, and pan‐B−) (two cases). This subtyping of ALL correlated well with known clinical presenting features, prognosis, chromosome analysis in 35 cases with an abnormal clone, and conventional immunologic typing in 38 cases. The data suggest that these simple and practical immunocytochemical stains can be used for immunologic subclassification of ALL.
Corticosteroids therapy in paroxysmal nocturnal hemoglobinuria
Authors: Issaragrisil Surapol , Piankijagum Anong , Tang‐naitrisorana Yaowalak 
Published: February 2007

AbstractWe evaluated the efficacy of alternate day, high dose prednisolone for the treatment of paroxysmal nocturnal hemoglobinuria (PNH). Nineteen patients were included. Thirteen were men and six were women, aged between 13–56 years. Eleven patients improved, eight with good response and three with fair response. Eight patients were non‐responders. Responders had gradual improvement in the hemoglobin level, but none achieved a normal hemoglobin level. Age at diagnosis, sex, initial hemoglobin, white count, and percentage of a positive Ham's test had no apparent bearing on treatment outcome. A prolonged interval from diagnosis to prednisolone treatment decreased the chance of a favorable hematologic response to therapy. Age at the treatment in non‐responders was higher than responders. Responders had higher numbers of colonies derived from BFU‐E and CFU‐GM both in the blood and bone marrow than non‐responders although the differences did not achieve statistical significance. These data indicate that alternate day, high dose prednisolone therapy is effective in some patients with PNH.
Spontaneous antithrombin in a patient with benign paraprotein
Authors: Gabriel Don A. , Carr Marcus E. , Cook Linda , Roberts Harold R. 
Published: February 2007

AbstractA 66‐year‐old man with peptic ulcer desease developed a paraprotein that resulted in a spontaneously prolonged prothromoin time, activated partial thromboplastin time, and thrombin clotting time. Although the reptilase time was normal, the thrombin clotting time failed to correct with the addition of normal plasma, calcium, or protamine sulfate. The patient's purified fibrinogen was normal, but his serum contained an IgG that inhibited the clotting of normal plasma and purified fibrinogen in the presence of thrombin. In contrast to previously described paraproteins, this patient's IgG appeared to inhibit the activity of thrombin per se rather than to interfere with fibrinogen cleavage or fibrin polymerization. Although immunoprecipitation between thrombin and the paraprotein could not be demonstrated, the patient's purified IgG, in the presence of thrombin, decreased the thrombin activity on a chromogenic substrate. Further, increasing concentrations of thrombin overcame the inhibitory effect of the patient's paraprotein. Thus, the patient's paraprotein appeared to possess antithrombin activity.
Impaired synthesis of immunoglobulin in patients with chronic lymphocytic leukemia
Authors: Kurec Anthony S. , Davey Frederick R. 
Published: February 2007

AbstractThe cause of hypogammaglobulinemia in patients with chronic lymphocytic leukemia (CLL) is unknown. Experiments were performed to determine if sera, monocytes, or non‐T cells from patients with CLL suppress the proliferative response and synthesis of immunoglobulin (Ig) following incubation with pokeweed mitogen (PWM) in cocultures with lymphocytes from normal individuals. The data indicate that sera and monocytes from patients with CLL did not suppress the proliferative response or synthesis of Ig by normal non‐T cells. When various numbers of normal non‐T cells and CLL non‐T cells were cocultured with a constant number of normal T cells, the proliferative response and the concentration of supernatant Ig decreased as the proportion of CLL non‐T cells increased. Since similar results were obtained when irradiated non‐T cells from normal individuals were substituted for non‐T cells from patients with CLL, we believe that the decrease in proliferative response and diminished synthesis of Ig is not the result of the suppressor non‐T cells but is related to the dilution of normal B cells by inert non‐ T cells. We conclude that these experiments serve as an in vitro model for patients with CLL and suggest that the hypogammaglobulinemia observed in this disease is related to the diluting out of normal B cells by the accumulation of neoplastic B cells in the peripheral blood, bone marrow, and lymphoid tissue of these patients.
No evidence for bradykinin hydrolysis in human erythrocyte suspensions: H NMR studies
Authors: Hyslop Serena J. , King Glenn F. , Kuchel Philip W. 
Published: February 2007

AbstractIn view of their permeability to small peptides, it has been postulated that human erythrocytes may play a role in terminating the action of some circulating peptide hormones. Work using classical paper chromatographic techniques for detecting free amino acids indicated that the octapeptide, des‐(Arg9)‐bradykinin, enters these cells and its amino‐terminal arginine residue is released by cytosolic aminopeptidase‐P. We have used 1H NMR to monitor directly the release of arginine from bradykinin. The hydrolytic reaction rate in hemolysates, with an initial peptide concentration of 13.0 mmol l−1 was 6.5 mmol (1 packed red cell)−1h−1. But no reaction was evident after a 4.5‐h incubation with intact cells, thus contradicting the earlier suggestion that erythrocytes are involved in the primary inactivation of this hormone. This is consistent with our previous findings that the pentapeptide leu‐enkephalin fails to enter human erythrocytes but that its lower‐order degradation products may do so.
Differentiation between essential thrombocythemia and polycythemia vera with marked thrombocytosis
Authors: Iland Harry J. , Laszlo John , Case Delvyn C. , Murphy Scott , Reichert Thomas A. , Tso C. Y. , Wasserman Louis R. 
Published: February 2007

AbstractAccurate distinction between essential thrombocythemia and thrombocytotic polycythemia vera requires determination of the red cell mass in the presence of adequate iron stores, but this is not always possible. We therefore compared the clinical and laboratory features at the time of presentation of 50 patients with unequivocal essential thrombocythemia and 27 patients with thrombocytotic polycythemia vera. Univariate analysis failed to identify any single parameter capable of reliably separating the groups. A logistic regression algorithm incorporating hematocrit, white cell count, and spleen size markedly increased the diagnostic accuracy (92 %) compared with predictions based on the hematocrit alone (52 %). The algorithm's usefulness for patients with intermediate hematocrits was confirmed by analysis of independent samples of essential thrombocythemia and thrombocytotic polycythemia vera patients, and also by analysis of patients with probable essential thrombocythemia in whom the diagnosis could not be confirmed because of inadequate exclusion of polycythemia vera. Furthermore, comparison of survival data suggests that differentiating these disorders is prognostically important. The algorithm is recommended as an alternate method for differentiating essential thrombocythemia from thrombocytotic polycythemia vera whenever the red cell mass is unavailable or iron deficiency cannot be excluded.
Measurement of terminal deoxynucleotidyl transferase mrna in clinical samples: A new parameter in analysis of leukemia cells
Authors: Wolf Susan C. , Steinherz Peter G. , Landau Nathaniel R. , Silverstone Allen E. 
Published: February 2007

AbstractA 1750 base pair cDNA to human terminal deoxynucleotidyl transferase (TdT) has been cloned. This cDNA detects a dominant 2200 base pair messenger RNA species in normal and leukemic cells synthesizing the enzyme. A quantitative dot blot assay was utilized to survey a number of clinical samples from patients with TdT positive and negative leukemias as well as cells from normal volunteers. A linear relationship was detected between the amount of TdT mRNA and the amount of enzyme activity in bone marrow cells. The assay is sensitive enough to detect normal TdT levels in bone marrow, and distinguish these levels from the lack of such mRNA in peripheral blood and bone marrow of patients with myeloid leukemia. Elevated levels of mRNA were found in two cases of patients in clinical remission. The prognostic significance of these observations must await further study.
Two murine monoclonal antibodies to peripheral blood monocyte differentiation antigens discriminate within M5 acute non‐lymphoid leukemia (ANLL) cells
Authors: Lopez M. , De Rossi G. , Santoro L. , Mandelli F. , Alescio T. , Annino L. , Pasqualetti D. , Siccardi A. G. , Mottolese M. , Natali P. G. , Cuomo M. 
Published: February 2007

AbstractTwo murine monoclonal antibodies (MoAbs), LAM3 and LAM7 of the IgG1 isotype, which were produced by immunization with normal peripheral blood monocytes (PBM), were assayed in their specificity by indirect immunofluorescence against a panel of normal as well as leukemic cells.Both LAM3 and LAM7 were reactive with PBM while LAM3 also recognized platelets. Neither MoAb showed reactivity with erythrocytes, granulocytes, or resting and mitogen activated B and T lymphocytes. The reactivity with bone marrow cells correlated with the degree of monocyte contamination.Among the 62 cases of leukemia tested, which included three cases of B‐CLL, 19 cases of ALL, and 40 cases of ANLL, both MoAbs reacted highly homogenously only with M5b ANLL cells. These findings indicate that the two MoAbs, which recognize two distinct epitopes, represent useful markers in the differential diagnosis of M5b ANLL.
Apotransferrin receptors and the delivery of iron from cultured human blood monocytes
Authors: Baynes R. D. , Bukofzer G. , Bothwell T. H. , Bezwoda W. R. 
Published: February 2007

AbstractA study was done to find out whether apotransferrin receptors are involved in the release of iron from reticuloendothelial cells. To this end, human macrophages which had been obtained by culturing blood monocytes for 7 days were incubated with either diferric or apotransferrin at the physiological pH of 7.4 or at an acidic pH (6.0). While specific diferric transferrin receptors (Kb 1.3 × 10−8M) were demonstrated at pH 7.4, no apotransferrin receptors were found. In contrast, both diferric receptors (Kd 2.1 × 10−8M) and apotransferrin receptors (Kd 2.8 × 10−9M) were found at pH 6.0. The finding of specific apotransferrin binding at acidic pH fits in with the current understanding of iron uptake by cells, in which the iron‐transferrin complex is endocytosed and the iron is released at acidic pH. The present results suggest that the apotransferrin remains attached to its receptor in the endocytosed vesicle at this acidic pH but that it becomes detached at the cell surface where the pH is neutral. No evidence was found to indicate that iron is transported out of macrophages via apotransferrin receptors at the physiological pH.
Cryptococcal tonsillitis in a patient with chronic lymphocytic leukemia: An unusual manifestation of cryptococcal disease
Authors: Korvick Joyce , Yu Victor L. 
Published: February 2007

AbstractWe present a case of chronic tonsillitis in a patient with chronic lymphocytic leukemia. Despite empiric radiation and antibiotic therapy, the patient's sore throat and tonsillar enlargement persisted. Excisional biopsy of the involved tonsil revealed the presence of Cryptococcus neoformans. Blood cultures also yielded cryptococcus. Specific antifungal therapy resulted in cure. To knowledge our this is the first case report of cryptococcal infection of the tonsil. This case illustrates the pitfalls of empiric therapy in an immunosuppressed host.
Systemic necrotizing vasculitis in sickle cell disease
Authors: Manci Elizabeth A. , Maisel Deborah A. , Conrad Marcel E. 
Published: February 2007

AbstractA patient with sickle cell disease and systemic necrotizing vasculitis is reported. Although there are a number of immune defects in patients with sickle cell disorders, this association has not been previously reported. We suspect that immunologic disorders frequently are unrecognized in these patients because the symptoms are attributed to complications of sickle cell disease.
Osteonecrosis in a patient with immune thrombocytopenic purpura
Authors: Smith Roy E. , Chelmowski Mark K. , Anderson Tom 
Published: February 2007

AbstractWe report a case of osteonecrosis in a 26‐year‐old woman treated with prednisone for immune thrombocytopenic purpura. The unusual complication occurred after a much shorter course of steroids of a considerably smaller cumulative dose than in previously reported cases.
Acquired von willebrand disease in patients with polycythemia rubra vera
Authors: Mohri Hiroshi 
Published: February 2007

AbstractPlatelet function and factor VIII complex were evaluated in ten patients with polycythemia rubra vera. Seven patients showed abnormal epinephrine‐induced aggregation. The intracellular concentrations of adenosine diphosphate (ADP) were below normal, and the ratio of adenosine triphosphate (ATP)/ADP was greater than normal. In four of eight cases, there was a decrease in ristocetin cofactor activity and a reduction in the slowly migrating forms of vWF:Ag on crossed immunoelectrophoresis. Defect of large multimers of vWF:Ag was also observed. The ratio of vWF:Ag to ristocetin cofactor was elevated in these patients. Plasma from the patients had no effect on normal plasma except in one case, in which isolated IgG appeared to cause inactivation of ristocetin cofactor. Treatment with 1‐deamino‐8‐arginine vasopressin caused correction of the vWF abnormalities with rapid return of ristocetin cofactor to baseline in some patients. The present study shows that the alterations of multimeric structure of vWF occur in more than 50% of patients with polycythemia rubra vera and are in some part due to the inhibitor specific for vWF.
Surgery in acute leukemia: A review of 167 operations in thrombocytopenic patients
Authors: Bishop J. F. , Schiffer C. A. , Aisner J. , Matthews J. P. , Wiernik P. H. 
Published: February 2007

AbstractOf 435 consecutive patients with acute leukemia, 95 patients had 167 operations with a platelet count of < 100 × 109/L and 130 operations with platelet counts of < 50 × 109/L. Only 7% of operations had intraoperative blood loss of >500 ml, and 7% required >4 units of red cells transfused in the perioperative period. No patient died of bleeding attributable to surgery within 1 month of the operation. Granulocyte count was <0.5 × 109/L in 66% of operations with 57% febrile preoperatively. However, no patient developed infection within the surgical field in the postoperative month. Logistic regression analyses were used to assess preoperative factors, which identified those operations at risk for excessive bleeding or postoperative surgical complications. Major (group 1) operations, preoperative fever, and preoperative coagulation abnormalities were associated with operations with an intraoperative blood loss >500 ml and/or perioperative red blood cell RBC transfusions >4 units (P < .001). Surgery in cytopenic patients with acute leukemia is safe provided optimal supportive care is available.
Dissociation of neutrophil aggregation, adhesiveness, and Fc receptor activity
Authors: Spagnuolo Philip J. , Fain Mindy , Bass Steven N. 
Published: February 2007

AbstractNeutrophils that bear receptors for the Fc portion of immunoglobulin G have been demonstrated to be more active in assays of adherence, aggregation, and chemotaxis compared to Fc receptor‐negative cells. We examined the relationship of neutrophil Fc receptor activity and cell–cell adherence or aggregation induced by phorbol myristate acetate. In contrast to 1‐isoproterenol, isobutyl‐methyl‐xanthine, and dibutyryl cAMP, each of which inhibited Fc receptor activity and neutrophil aggregation, theophylline significantly impaired aggregation without affecting Fc receptor activity. The selective beta‐2 agonist, metaproterenol, and 8‐Bromo cAMP failed to inhibit Fc receptor activity or neutrophil aggregation. Three known inducers of neutrophil intracellular cyclic AMP, PGE1, PGE2, and cholera toxin, also did not inhibit Fc receptor activity. Inhibition of Fc receptor activity by 95% in the presence of purified Fc fragments did not affect neutrophil aggregation. Similarly suppression of Fc receptor activity by purified Fc fragments did not inhibit neutrophil adhesion to nylon fiber columns. These data demonstrate that the Fc receptor does not mediate phorbol myristate acetate‐induced cell–cell adhesion and is not necessary for optimal neutrophil adhesion to nylon fibers. Our results are consistent with the possibility that the reversible inhibitory activity of beta‐adrenergic agonists on rosette formation may be a steric effect rather than a metabolic effect. These data tend to dissociate Fc receptor activity, neutrophil aggregation, and adhesion and support the hypothesis that the Fc receptor may be a marker of neutrophil heterogeneity rather than a component necessary for optimal neutrophil aggregation or adhesion.
Heme metabolism and in vitro erythropoiesis in anemia associated with hypochromic microcytosis
Authors: Brown A. C. , Lutton J. D. , Pearson H. A. , Nelson J. C. , Levere R. D. , Abraham N. G. 
Published: February 2007

AbstractHeme metabolism and in vitro erythropoietic growth (CFU‐E, BFU‐E) were examined in bone marrow cells taken from two siblings with apparent familial hypochromic microcytic anemia. Bone marrow cells from both patients grew adequate numbers of CFU‐E and BFU‐E colonies in culture in the presence of erythropoietin. In addition, small numbers of endogenous CFU‐E were seen in 7‐day cultures. Assays on bone marrow cells taken from both patients revealed that baseline δ‐aminolevulinic synthase activity was considerably reduced, but increased six to seven fold (to normal levels) when patients' cells were exposed to pyridoxal phosphate (PLP). In both cases, ferrochelatase and δ‐aminolevulinic acid dehydratase activities were normal. Bone marrow heme oxygenase showed no significant differences in activities between normals and patients values in the absence or presence of PLP. In contrast, heme synthesis by patients' bone marrow was less than that of normals. This study demonstrates that bone marrow cells from patients with this rare disorder have some disturbances in heme metabolism, whereas erythropoiesis appeared to be normal when cultured with adequate nutrients in vitro.