bluuebunny/crossref_metadata_2025_split huggingface dataset:
Erythrocyte‐oxidized glutathione transport in pyrimidine 5′‐nucleotidase deficiency
Authors: Kondo Takahito , Ohtsuka Yoshinori , Shimada Michiro , Kawakami Yoshikazu , Hiyoshi Yasuhiko , Tsuji Yoshiro , Fujii Hisaichi , Miwa Shiro 
Published: February 2007

AbstractThe oxidized form of glutathione transport was studied in human erythrocytes in pyrimidine 5′‐nucleotidase (P5N) deficiency, a disorder in which the amounts of CTP and UTP in the erythrocytes are elevated. The inhibition of ATP‐requiring oxidized glutathione (GSSG) transport by CTP and UTP is believed to play a role in elevating the levels of the reduced form of glutathione (GSH) in the erythrocytes of patients with P5N deficiency. The current investigation was undertaken to determine if GSSG transport actually decreases in the erythrocytes of such patients. Erythrocytes from a 17‐year‐old patient and a 13‐year‐old patient with P5N deficiency hemolytic anemia and from ten normal subjects were used as materials for the experiment. Erythrocytes, which had been previously incubated with [3H]glycine, were incubated at 37°C, and the rate of [3H]GSSG transported by the cells was estimated. The velocity of GSSG transport out of the erythrocytes was quite low in the patients, 3.17–3.65 nmol GSSG/ml erythrocytes/hr at 37°C in one case, and 3.30 nmol GSSG/ml erythrocytes/hr in the other case, vs that in the normal controls (6.00 ± 0.80 nmol GSSG/ml erythrocytes/hr; mean ± SD). The activity of γ‐glutamylcysteine synthetase and glutathione synthetase did not decrease in the patients. Decreased transport activity of GSSG in addition to a normal synthesis rate for GSH may explain the increased concentration of erythrocyte GSH in P5N deficiency.
Deficiency of inducible suppressor cell activity in the Chediak‐Higashi syndrome
Authors: Nair Madhavan P. N. , Gray Robert H. , Boxer Laurence A. , Schwartz Stanley A. 
Published: February 2007

AbstractPeripheral blood lymphocytes from two Chediak‐Higashi syndrome (CHS) patients were examined for their 1) natural killer (NK) cell functions 2) concanavalin A (Con A)‐inducible suppressor cell activity, 3) soluble suppressor factor production, and 4) responsiveness to interferon alpha and interleukin‐2 in comparison with age‐matched normal controls. Peripheral blood lymphocytes or NK‐enriched large granular lymphocytes from Chediak‐Higashi syndrome patients showed negligible cytotoxic activity against several target cells. Although the NK activity of Chediak‐Higashi syndrome lymphocytes could not be restored to normal levels by treatment with either interferon or interleukin‐2, the percent enhancement of NK activity was higher for the patients than the controls. Soluble suppressor factor activity of culture supernates from the lymphocytes of Chediak‐Higashi syndrome patients significantly inhibited the NK activity of allogeneic, normal peripheral blood lymphocytes, whereas lymphocytes from Chediak‐Higashi syndrome patients precultured with Con A failed to suppress the cytotoxic activity of normal lymphocytes. These results demonstrate a previously unrecognized suppressor cell dysfunction in CHS patients.
Cyclosporin A for the treatment of aplastic anemia refractory to antithymocyte globulin
Authors: Bridges Ronald , Pineo Graham , Blahey Walter 
Published: February 2007

AbstractAntithymocyte globulin (ATG) is an established form of therapy for severe aplastic anemia (SAA). However, in patients who do not respond to this treatment and who are not candidates for bone marrow transplantation few successful therapeutic alternatives exist. We report two such patients who have shown a therapeutic response to Cyclosporin A (CSA) (Sandimmune, Sandoz). Case 1, a 15 year old male, and Case 2, a 34 year old female, were diagnosed as having SAA in September 1984 and May 1984 respectively. Treatment with high dose Methylprednisolone (MPN) and ATG in Case 1 and MPN, ATG and Oxymetholone in Case 2 for ten days was ineffective in both cases. Case 1 developed anaphylaxis with both repeat ATG and ALG (antilymphoblast globulin), and Case 2 failed to respond to repeat ATG. Both required frequent packed cells and platelet transfusions.At five and six months respectively following completion of ATG therapy, CSA was started at 10 mg/kg/day in divided doses orally. Renal and liver functions and CSA blood levels were followed. Within six weeks both patients exhibited a hematologic response and were no longer transfusion dependent. On maintenance therapy of 4 mg/kg/day (Case 1) and four months after discontinuing CSA (Case 2) the hematologic values are as follows: hemoglobin 160 and 130 g/L, absolute granulocyte count 3100 and 1640 × 109/L, and platelets 132 and 84 × 109/L respectively. Side effects included hypertrichosis, gingival hyperplasia and mild reversible nephrotoxicity. CSA appears to represent an effective form of therapy for patients with SAA refractory to ATG.
Hemophiliac patient's knowledge and educational needs concerning acquired immunodeficiency syndrome
Authors: Hargraves Martha A. , Jason Janine M. , Chorba Terence L. , Holman Robert C. , Dixon Gloria R. , Eastham Christine J. , Brownstein Alan P. , Heine Peggy , Agle David P. ,   Hemophilia/Aids Collaborative Study Group
Published: February 2007

AbstractThe Patient Knowledge Assessment Study (PKAS) was conducted among 107 male hemophilic patients, aged 15 to 67 years, at 19 hemophilia treatment centers (HTC). Participants were given a 30‐item questionnaire concerning the cause of acquired immunodeficiency syndrome (AIDS), the groups at risk, and modes of transmission. The questionnaire included questions on the participant's status in regard to antibody to human T‐lymphotropic retrovirus, type III/lymphadenopathy‐associated virus (HTLV‐III/LAV), and the meaning of this test result. HTC health‐care providers were asked to complete a separate questionnaire containing 17 questions about information given patients concerning their HTLV‐III/LAV antibody status and its meaning. Overall, patients had a good base of knowledge about AIDS; however, there were gaps in this knowledge. Twenty‐nine percent of patients did not know that spouses of AIDS patients were at risk for AIDS; 47% did not know that sexual partners of persons with hemophilia were at risk; and 32% did not know that hemophilic children were at risk. Further, only 69% understood that antibody‐positive individuals had had contact with the AIDS virus. Identifying these and other areas of misunderstanding will provide the information needed to design educational strategies and psychosocial support programs appropriate for the hemophilic population, and which may serve as a model for other populations.
Deficiency of mature B and T lymphocyte subsets in the blood of non‐Hodgkin lymphoma patients
Authors: Janowska‐Wieczorek Anna , Andrews E. Joy , Khaliq Abdul , Pilarski Linda M. 
Published: February 2007

AbstractThe expression of mature B‐cell markers and T markers was determined in lymphocytes isolated from the peripheral blood (PBL) of 20 healthy adults and 51 patients with non‐Hodgkin malignant lymphoma (NHL). The disease was classified as newly diagnosed, in remission, or being treated with chemotherapy and of low‐, intermediate‐, or high‐grade malignancy. To avoid technical problems associated with artifacts involving cytophilic immunoglobulins (Ig), we defined mature B‐cells by means of three criteria: a) expression of high surface density of Ig sufficient to allow polar movement of receptors to form a cap in an indirect immunofluorescence (IF) assay, b) expression of high density of the human leukocyte antigens DR (HLA‐DR) under capped conditions, and c) expression of a 41H.16 marker exclusive to surface Ig+ B‐cells. Percentages of PBL able to cap surface Ig (sIg) (λ, K), HLA‐DR (7H.3), and 41H.16 markers were significantly reduced (p<0.001) in all of the patients, regardless of treatment status, and the numbers of sIg+‐capping cells were similarly reduced in the patients, regardless of the grade of malignancy. Studies with ring fluorescence showed mean percentages of cells expressing OKT3 and OKT4 determinants significantly reduced (P<0.001) but OKT8+ cells not significantly different from control. The OKT4/OKT8 ratio was reduced in all patients and did not differ significantly in relation to the degree of malignancy. We conclude that, in NHL, essentially all patients have severe abnormalities in the number of B‐ or T‐cells needed for normal immune responses.
Cryptic cold agglutinin activity of monoclonal macroglobulins
Authors: Pruzanski W. , Jacobs H. , Saito S. , Donnelly E. M. , Lui L. C. 
Published: February 2007

AbstractOne hundred and twenty sera with monoclonal immunoglobulin M's (IgM's) were tested for cryptic cold agglutinin activity against papain‐ and neuraminidase‐treated adult and cord red blood cells (RBC). Fifteen sera (12.5%) agglutinated papain‐treated RBC. Fourteen of these 15 sera also agglutinated neuraminidase‐treated RBC. Ten additional sera agglutinated neuraminidase‐treated but not papain‐treated cells. Three patterns were observed among agglutinators of papain‐treated RBC: either predominant agglutination of adult RBC, or of cord RBC or equal reaction, thus resembling I, i and non‐Ii patterns of conventional cold agglutinins. We propose to call these patterns, tentatively, Icr (cr for cryptic), icr, and non‐Iicr, respectively. Purified monoclonal IgM's retained the above patterns. Eluates from enzyme‐treated cells contained monoclonal IgM's. Investigation of neuraminidase‐treated RBC has shown that in distinction to T‐anti‐T system (Thomsen‐Friedenreich receptor system), the majority of sera with monoclonal IgM's agglutinated more strongly adult RBC, and some agglutinated more strongly cord RBC. There was no loss of agglutinating activity after repeat freezing and thawing of the sera. Thus, it seems that monoclonal IgM's which react with neuraminidase‐treated RBC detect antigenic determinants different from the T antigen. Cryptic CA activity of IgM's should be added to the list of antibody activities of these monoclonal immunoglobulins.
Cyclosporin‐a for the treatment of pure red cell aplasia in a patient with chronic lymphocytic leukemia
Authors: Chikkappa G. , Pasquale D. , Phillips P. G. , Mangan K. F. , Tsan M. F. 
Published: February 2007

AbstractA 62‐year‐old man with B‐cell chronic lymphocytic leukemia had three separate episodes of pure red cell aplasia (PRCA). The last episode was treated with cyclosporin‐A (CyA) and prednisone. After the patient was on the therapy for 2 weeks, erythropoietic recovery was observed and with continued therapy the hematocrit (Hct) became normal. The PRCA remission was associated with a fall in the blood lymphocyte count, and a reduction in the spleen and lymph node size and bone marrow lymphocyte density. At diagnosis of PRCA the blood T‐cells bearing IgG Fc receptors (T gamma cells) were increased, and the marrow contained very few or no late‐stage erythroid progenitors. After remission of PRCA the T gamma cell fraction decreased, and the marrow erythroid progenitor's number became normal. We speculate that therapy with CyA and prednisone inhibited the production of interleukins‐1 and ‐2 from monocytes and T‐cells, respectively, and was responsible for the reduction of the T gamma cell fraction and B‐cell leukemic mass in this patient. Further, we believe that normalization of T gamma cells in association with the therapy was responsible for the PRCA remission.
Relationship of fluorescence polarization to cell lineage in lymphocytes from normal subjects and patients with chronic lymphocytic leukemia
Authors: Glotzer Taya V. , Liebes Leonard F. , Kanganis Denise , Silber Robert 
Published: February 2007

AbstractPrevious investigations have shown differences in fluorescence polarization between normal and chronic lymphocytic leukemia lymphocytes following incubation with the probe 1,6‐diphenyl‐1,3,5‐hexatriene. In the present study, we determined the fluorescence polarization of unseparated or enriched subpopulations of T and B lymphocytes from normal subjects and patients with chronic lymphocytic leukemia. As had been observed by others, the mean polarization (P) value at 25°C for unseparated chronic lymphocytic leukemia lymphocytes, .240 ± .007 (N = 22), was lower than that of unseparated normal lymphocytes, .248 ± .005 (N = 18), P < .001 (Student's t‐test). The difference was greater when B‐enriched populations were compared. The mean P value of B‐cell‐enriched chronic lymphocytic leukemia lymphocytes, .240 ± .007 (N = 5), was significantly lower than that of B‐cell‐enriched normal preparations, .256 ± .004 (N = 5), P < .001. In contrast, no significant difference was found between normal and chronic lymphocytic leukemia T cells. The anomalous fluorescence polarization manifested by chronic lymphocytic leukemia lymphocytes of B‐cell origin serves to distinguish this lineage from its normal counterpart and from T cells of either source.
Association in cis of β+‐thalassemia and hemoglobin S
Authors: Baklouti Faouzi , Francina Alain , Dorléac Evelyne , Richard Georges , Rosenberg Daniel , Godet Jacqueline , Delaunay Jean 
Published: February 2007

AbstractA Moroccan woman was investigated because of a typical β‐thalassemia trait associated with a low‐percentage (11%) hemoglobin (Hb) variant. The β‐thalassemia trait was manifested by a microcytosis, a high HbA2 (above 6%), and an increase of the α/β biosynthetic ratio (1.31). The variant was identified to HbS by amino acid analysis of the abnormal peptide (β T1) and by DNA mapping with Sau I (Mst II) restriction endonuclease. No additional amino acid substitution was recorded in the βs‐chain. The reduction of β‐globin synthesis occurred exclusively at the expense of the βs‐chain. These results are consistent with the existence of a βs mutation and a β+‐thalassemia in cis.
Excess HLA antigens after transfusion of sickle cell patient with AIDS
Authors: Castro Oswaldo , Alarif Lamya , Saxinger Carl , Frederick Winston 
Published: February 2007

AbstractA woman with sickle cell‐hemoglobin C disease developed the acquired immunodeficiency syndrome (AIDS), possibly as the result of blood transfusions administered 4 years earlier. Three months after subsequent transfusions, she had excess HLA‐A and DR antigens (triplets) on her circulating lymphocytes. Three of her first‐degree relatives were HLA‐typed, and none had excess antigens. Family studies also indicated that the patient had at least one HLA‐A antigen that was probably not inherited. These findings suggest the possibility that transient hemopoietic engraftment can occur in transfused sickle cell patients with AIDS.
Corticosteroid‐responsive pure red cell aplasia in chronic lymphatic leukemia
Authors: Vashi P. , Patel B. , Musson P. , Fahrner J. , Rosenbaum R. 
Published: February 2007

AbstractAcquired pure red cell aplasia characterized by selective failure of erythropoiesis was seen in a 37‐yr‐old female patient with a diagnosis of chronic lymphatic leukemia (CLL). Though different immunosuppressive therapies have been tried in the past with variable response, there has been no case report of such favorable response to corticosteroids alone. We report this rare and unusual case of PRCA associated with CLL who responded to steroid therapy.
Relationship of HLA and platelet‐reactive antibodies in alloimmunized patients refractory to platelet therapy
Authors: Brubaker Daniel B. , Romine Maxine 
Published: February 2007

AbstractPlatelet crossmatching assays have been used to predict the outcome of platelet transfusions in alloimmunized patients by detecting antibodies against platelets. The transfusion failure of HLA‐matched platelets predicted by platelet crossmatching may be related to HLA antibodies undetected by lymphocytotoxicity but detected by platelet immunoglobulin‐binding assays or platelet‐specific antibodies (both antibodies defined here as platelet‐reactive antibodies). To differentiate platelet‐reactive antibodies from lymphocytotoxic HLA antibodies, we used HLA characterized lymphocytes in parallel with platelets from individuals to form separate frozen panels. Sera from 10 allosensitized patients were studied in the lymphocyte panel by lymphocytotoxicity and in the platelet panel by enzyme‐linked immunoassay (ELISA). By comparing pattern and percent wells reacting in each panel, lymphocytotoxic HLA antibodies and antibodies reactive with platelets in ELISA were detected separately. In all 10 allosensitized patients, platelet‐associated antibodies were present and 7 had additional lymphocytotoxic HLA antibodies. Using this double parallel panel technique, we found platelet‐reactive antibodies important in platelet alloimmunization, unrecognized by lymphocytotoxicity. These data indicate platelet‐crossmatching be solely used in the selection of platelets for allosensitized patients.
Erythrocyte pyruvate kinase (PK): The variable significance of “nucleotide specificity” in the characterization of mutant variants
Authors: Valentine W. N. , Paglia D. E. , Nakatani M. , Brockway R. A. 
Published: February 2007

AbstractThe half‐saturation constant (K0.5s) phosphoenolpyruvate (PEP) for red cell pyruvate kinase (PK) with co‐factors UDP and GDP is less than one‐half that with ADP with or without additions of the allosteric modifier, fructose‐1, 6‐dephosphate (F‐1, 6‐P2) to the assay. The Vmax is markedly greater with ADP than with UDP or GDP, but with (PEP) at 0.5 mM, activity with all co‐factors is about equal and at lower concentrations greater with UDP and GDP. With high K0.5s (PEP) mutant enzymes, and at the usual test concentration (1mM) for PEP when nucleotide specificity is assessed, the abnormally low saturation of variant enzymes may result in higher activity with UDP and GDP than with ADP–the opposite of the “normal situation.” The apparent aberration in nucleotide specificity may thus be illusory and secondary to the abnormal K0.5s (PEP) of the mutant. Example data are recorded. Variations in K0.5s (PEP) may also be introduced during enzyme preparation for assay, particularly when partial purification is employed.
Lack of evidence of circulating retroviral antibodies in patients with classic Hodgkin's disease
Authors: Chorba T. L. , Kalyanaraman V. S. , Lacher M. J. , Schulof R. S. 
Published: February 2007

AbstractBecause of the T‐cell abnormalities observed in Hodgkin's disease and the growing number of Hodgkin's disease cases observed in association with the acquired immunodeficiency syndrome (AIDS), concern has been expressed that a retrovirus may be the primary cause of Hodgkin's disease. We examined plasma specimens from 17 patients with Hodgkin's disease that were drawn in 1979. Because serum containing antibodies to either human T‐lymphotropic virus type I (HTLV‐I) or HTLV‐II precipitate the major core protein, p24, of HTLV‐I, lack of reactivity to HTLV‐I p24 in all the specimens demonstrated absence of antibodies to HTLV‐I or ‐II. None of the specimens was reactive to human immunodeficiency virus type 1 (HIV‐1) by ELISA. None of the specimens were reactive on Western blot assays for HTLV‐I or ‐II or HIV‐1. Lack of evidence of cross‐reacting antibodies to prototype strains of those retroviruses in specimens drawn before the AIDS epidemic suggests that classic Hodgkin's disease is not the result of infection with one of the known human lymphocytotropic retroviruses or a closely related agent.
Response in serum ferritin and haemoglobin to iron therapy in blood donors
Authors: Mackintosh Wayne , Jacobs Peter 
Published: February 2007

AbstractSeven hundred seventeen healthy male blood donors regularly donating four or more units a year were surveyed for haemoglobin and serum ferritin levels. One hundred fiftyone (21%) had a haemoglobin less than 13.5 g/dl and were therefore disqualified from further blood donation, having a mean serum ferritin of 28 μg/liter. Of the remaining 566 donors with haemoglobin levels equal to or greater than 13.5 g/dl, the mean serum ferritin was 33 μg/liter, although in 299 (53%) the value was less than 28 μg/liter. To document response to iron therapy 46 donors with haemoglobin levels equal to or greater than 13.5 g/dl were stratified into those with the lowest iron stores (group 1; n = 23), defined as a serum ferritin less than 20 μg/liter, and controls (group 2; n = 23), with serum ferritin between 50 and 150 μg/liter. Within each stratum donors randomly received ferric polymaltose at a dose of 100 mg elemental iron twice daily for 56 days (groups 1a and 2a) or an identical iron‐free placebo tablet administered on the same schedule (groups 1b and 2b). Iron therapy in the iron‐deficient group (group 1a:n = 11) resulted in a significant rise in haemoglobin (p = .03) and iron stores reflected in serum ferritin (p = .002) compared to those receiving placebo (group 1b). In the control group iron therapy or placebo was without significant effect. Thus, ferric polymaltose preparation is bioavailable and is notable for the virtual absence of gastrointestinal tract side effects.
Intraarticular methylprednisolone therapy in hemophilic arthropathy
Authors: Shupak R. , Teitel J. , Garvey M. B. , Freedman J. 
Published: February 2007

AbstractThis small pilot study examined the use of intraarticular methylprednisolone in hemophilic synovitis. Nineteen joints in ten adult hemophiliacs were studied. There was subjective improvement at 24 hr following injection in 79% of joints injected, and the improvement persisted up to 8 wk in 58%. The number of hemarthroses decreased following intraarticular steroids (mean of 7.7 bleeds in the 8 wk prior to injection versus a mean of 1.9 bleeds in the 8 wk following injection). Similarly the amount of clotting factor used for the injected target joint decreased from a mean of 7,616 units to 2,315 units postinjection (p <.001). Improvement correlated with presence of synovitis but not with radiologic stage of the joint. Aspirated synovial fluids were analyzed and showed characteristics consistent with low‐grade inflammation. These preliminary observations suggest that intraarticular corticosteroid injection may be a useful therapeutic tool in the medical management of hemophilic arthropathy.
Genotypic analysis using a Y‐chromosome‐specific probe following bone marrow transplantation
Authors: Morisaki Hiroko , Morisaki Takayuki , Nakahori Yutaka , Ogura Hiromi , Kanno Hitoshi , Tani Kenzaburo , Kodo Hideki , Fujii Hisaichi , Asano Shigetaka , Miwa Shiro 
Published: February 2007

AbstractTo monitor successful engraftment after bone marrow transplantation, we performed Southern hybridization analysis or dot blot analysis of DNA in a set of sex‐mismatched cases using a Y‐chromosome‐specific DNA probe (pHY10). This method was extremely sensitive and rapid for checking which cells contain the Y‐chromosome. Using this probe, analysis of cells from peripheral blood and bone marrow after transplantation demonstrated the usefulness of confirming engraftment of donor cells and of detecting mixed lymphohematopoietic chimerism.