bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clonal immunoglobulin gene rearrangements in chronic lymphocytic leukemia: A correlative study
Authors: Soper L. , Bernhardt B. , Eisenberg A. , Cacciapaglia B. , Bennett L. , Sanda A. , Baird M. , Silver R. , Benn P. 
Published: February 2007

AbstractForty‐two patients with chronic lymphocytic leukemia (CLL) were studied for immunoglobulin gene and T‐cell receptor gene rearrangements. Immunoglobulin heavy chain gene rearrangements were demonstrable in 41 cases. One rearrangement of the T‐cell receptor beta chain gene was detected. Quantification of the relative intensities of germline and rearranged DNA bands suggests that a significant component of the lymphocytosis may be due to cell populations other than the malignant clonal population, particularly in earlier stages of the disease. A direct relationship was found between severity of disease and the relative amount of clonal immunoglobulin heavy chain gene rearrangement. Preliminary data for 12 patients followed sequentially indicated that clinical deteriorations or improvements are reflected in an increase or decrease, respectively, in the proportion of cells with rearranged immunoglobulin genes.Change in the relative proportion of cells with germline versus rearranged genes may provide an additional useful criterion for staging CLL, for more precisely defining the abnormal lymphocyte population, and for monitoring progression of the disease and efficacy of treatment.
Serum‐free culture of human hemopoietic progenitors in attenuated culture media
Authors: Sonoda Yoshiaki , Ogawa Makio 
Published: February 2007

AbstractTo elucidate the precise mechanisms of molecular and cellular regulation of hemopoiesis, it is necessary to develop a chemically defined culture assay for purified hemopoietic progenitors. To approach this long‐term goal, we attempted to develop a serum‐free culture system for enriched human progenitors that permits expression of all hemopoietic lineages and stages of development. Preliminary studies indicated that α‐medium was superior to iscove's modified Dulbecco's medium (IMDM) and that culture under low (5%) oxygen condition was better than an ambient level of oxygen. We developed an attenuated (modified quarter‐strength) α‐medium and compared the colony‐supporting ability of the three media by plating 1,000 bone marrow null cells per dish in the presence of a combination of recombinant human colony‐stimulating factors (CSFs). The numbers of colonies supported in α‐medium and attenuated α‐medium were approximately 70% of those in serum‐containing cultures. IMDM failed to support colony formation. While, in general, the colony sizes were smaller in the serum‐free cultures than in the serum‐containing cultures, a variety of types of single lineage and multilineage colonies were seen in serum‐free culture. A linear relationship between cell number and colony formation was seen in 100‐2,000 cells per dish. Serum‐free cultures of enriched human progenitors should be an important tool for analysis of the mechanisms of recombinant CSFs.
Acute tumor lysis syndrome in non‐Hodgkin lymphoma induced by dexamethasone
Authors: Dhingra Kapil , Newcom Samuel R. 
Published: February 2007

AbstractAcute tumor lysis syndrome developed in a 30‐year‐old man with non‐Hodgkin lymphoma after dexamethasone administration. To the best of our knowledge this case is the first one reported following single agent corticosteroid treatment.
Unique promotion of erythrophagocytosis by malondialdehyde
Authors: Hebbel Robert P. , Miller Wesley J. 
Published: February 2007

AbstractModification of the normal erythrocyte membrane by reagent malondialdehyde (MDA) promotes phagocytosis of red blood cells by human macrophages, a phenomenon previously shown to involve both IgG‐dependent and IgG‐independent mechanisms and to be demonstrable even at micromolar MDA concentrations. In the present studies, we demonstrate this effect using 1mM MDA prepared both by acid hydrolysis of malonaldehyde bis‐(dimethyl acetal) and by enzymatic synthesis from 1,3‐propanediol. Remarkably, we find that equimolar amounts of other mono‐ and dialdehydes fail to promote erythrophagocytosis despite similarity to MDA in size and structure and ability to cross link. Authentic MDA seems to be unique among small aldehydes in ability to promote erythrophagocytosis at low aldehyde concentration and, therefore, has a special biologic relevance among the great variety of peroxidation byproducts.
Testing for cell surface forms of class II major histocompatibility complex antigens and Ii by radioiodination, biotinylation, and membrane immunofluorescence
Authors: Elliott William L. , Sorli Christopher H. , Reisert Patricia S. , Humphreys Robert E. 
Published: February 2007

AbstractAntibodies to either Ii or class II major histocompatibility complex (MHC) antigens did not recognize cell surface forms of Ii in immunoprecipitates of cells that had been radioiodinated by the lactoperoxidase method, whereas they bound [35S]methionine metabolically labeled molecules. N‐hydroxysuccinimidobiotin (NHS‐B) and biotin hydrazide (B‐H) were used to react more generally with cell surface proteins via amino groups and nitrene coupling, respectively. Each of these latter compounds labeled α and β chains of class II MHC antigens as seen in Western‐blotted, electrophoresed immunoprecipitates probed with 125I‐labeled streptavidin but not Ii or its associated forms. Although tyrosine residues might have been inaccessible to radioiodination in carbohydratederivatized forms of Ii, the lack of Ii biotinylation in these controled, sensitive studies was consistent with the view that Ii forms were not surface expressed, with the possible exception of the chondroitin sulfate‐derivatized forms of Ii (Ii‐CS).
Pseudothrombocytopenia induced by a monoclonal IgM kappa platelet agglutinin
Authors: Hoyt Robert H. , Durie Brian G. M. 
Published: February 2007

AbstractPseudothrombocytopenia is an in vitro phenomenon usually associated with anticoagulant (EDTA)‐dependent IgG platelet agglutinins. A low Coulter platelet count was investigated in a 63‐year‐old woman with multiple sclerosis and a monoclonal IgM kappa gammopathy. An unusual type of EDTA‐ and temperature‐independent IgM platelet agglutinin was identified by in vitro agglutination of donor platelets and was removed by immunoabsorption.
Elevated urokinase‐type plasminogen activator level and bleeding in amyloidosis: Case report and literature review
Authors: Sane David C. , Pizzo Salvatore V. , Greenberg Charles S. 
Published: February 2007

AbstractHyperfibrinolytic states are reported to be a cause of bleeding in patients with amyloidosis. We reviewed the literature on excessive fibrinolysis in association with amyloidosis and report our findings from a patient with idiopathic amyloidosis who developed a bleeding diathesis. Coagulation laboratory studies indicated elevated plasminogen activator levels associated with a reduction of plasminogen and α2‐plasmin inhibitor (α2‐PI) levels. The level of tissue‐type plasminogen activator (t‐PA) inhibitor and t‐PA antigen were normal. However, the patient did have a five‐ to sevenfold increase in amidolytic activity for the urokinase substrate pyro‐Glu‐Gly‐Arg‐pNA (S‐2444). This case therefore represents a novel example of a hyperfibrinolytic state associated with amyloidosis caused by elevated urokinase‐type plasminogen activator (u‐PA). Epsilon‐amino caproic acid (EACA) therapy resulted in an increase in α2‐PI and plasminogen levels and effectively reduced the blood loss. Hyperfibrinolytic states in amyloidosis have now been reported to be due to elevated t‐PA and u‐PA and depleted t‐PA inhibitor.
Platelet glass bead retention is useful in monitoring response to 1‐deamino‐8‐D‐arginine‐vasopressin (d‐DAVP)
Authors: Zeigler Z. R. 
Published: February 2007

AbstractSeven patients with a suspected mild bleeding disorder had consistently abnormal glass bead platelet retention. This was the only coagulation abnormality detected in six of these patients; the seventh individual showed evidence of mild von Willebrand disease. Nevertheless, platelets from the six that were studied behaved in a similar fashion in the two‐stage platelet retention assay of McPherson and Zucker. This indicated that their platelets were defective in maintaining repetitive platelet‐platelet interaction in the second phase of this assay but functioned normally in the first phase. Their one‐stage platelet retention defects consistently normalized following the infusion of 1‐deamino‐8‐D‐arginine‐vasopressin (d‐DAVP). The duration of response was variable and easily defined by monitoring this parameter. These findings suggest platelet retention analysis may be useful in identifying mild bleeding disorder patients, who may benefit from d‐DAVP.
Interstitial deletion of chromosome 5, del(5q), in a newborn with down syndrome and an unusual hematologic disorder
Authors: Adams Roberta H. , Lemons Richard S. , Thangavelu Maya , Beau Michelle M. Le , Christensen Robert D. 
Published: February 2007

AbstractA newborn with Down syndrome was noted on the 1st day of life to have an elevated white blood cell count of 79,900/mm3 with 62% lymphoblasts and a platelet count of 61,000/3, consistent with either transient myeloproliterative disorder of Down syndrome (TMD) or acute leukemia. Karyotype analysis of a bone marrow aspirate revealed that 20% of the cells had a 47,XY, + 21 karyotype, and 80% had a 47,XY, + 21, del(5)(q13q31) complement. Cytochemical and immunophenotyping of the peripheral blasts were consistent with the presence of an acute undifferentiated precursor blast clone. Results of clonogenic assays of hematopoietic progenitors from this patient's bone marrow were similar to those of patients with TMD. This patient's hematologic abnormalities resolved spontaneously without treatment by week 10 of life. This is the first report of an interstitial deletion of 5q associated with a hematologic abnormality present in an infant at birth.
Bone marrow aplasia associated with proliferation of large granular lymphocytes and subsequent transformation to acute lymphoblastic leukemia
Authors: Schiller Gary J. , Naeim Faramarz , Champlin Richard E. 
Published: February 2007

AbstractProliferation of large granular lymphocytes may be associated with neutropenia and, less frequently, thrombocytopenia. In this report, we describe a patient with severe aplastic anemia in the setting of a proliferation of cells with natural killer (NK) phenotype. We demonstrated evolution to acute lymphoblastic leukemia by cells of identical phenotype.
Cell‐mediated suppression of human hematopoiesis: Evaluation by limiting‐dilution analysis of hematopoietic progenitors
Authors: Takaue Yoichi , Reading Christopher L. , Watanabe Tsutomu , Kawano Yoshifumi , Ninomiya Tsuneo , Iishi Yashuhiko , Kosaka Masaaki , Shimizu Eiji , Ichioka Takao , Hosoda Teizoh , Suzue Takeshi , Abe Takanori , Koyama Tetsuya , Kuroda Yashuhiro , Dicke Karel A. 
Published: February 2007

AbstractWe have used limiting‐dilution clonal analysis (LDA) in microwells to study the inhibitory effects of T lymphocytes (T‐cells) or natural killer (NK) cells on human marrow progenitor cell growth. In four subjects with normal hematopoiesis, the growth of progenitors showed single‐hit kinetics both before and after T‐cell removal, indicating that, in the presence of colony‐stimulating activity, T‐cells have no effect on progenitor growth. In a patient with marrow hypoplasia associated with thymoma, hypogammaglobulinemia, and an increased number of suppressor T‐cells (Good's syndrome), the progenitor growth deviated from linearity, demonstrating the presence of cells with suppressor activity. After T‐cells were removed from this sample, the progenitor growth showed single‐hit kinetics. The suppressive action of E‐rosette‐positive cells with NK or cytotoxic activities was also suggested in a patient with severe combined immune deficiency and in a patient with T± lymphocytosis. Poor progenitor‐cell growth in three other patients with aplastic anemia was not significantly altered by T‐cell removal. Thus, LDA of human hematopoietic progenitors is useful for evaluating cell‐mediated interactions affecting hematopoiesis. This method may facilitate elucidation of mechanisms of myelosuppression in clinical settings.
Use of DDAVP in inherited and acquired platelet dysfunction
Authors: Dimichele Donna M. , Hathaway Wm. E. 
Published: February 2007

AbstractTwenty‐one patients with prolonged bleeding times secondary to inherited disorders of platelet function and eight patients with prolonged bleeding times secondary to acquired platelet dysfunction were given 0.3 μg per kilogram of DDAVP, 1‐deamino‐8‐D‐arginine vasopressin, intravenously. Sixteen of twenty‐two DDAVP trials in patients with inherited platelet dysfunction (73%) and seven of the nine DDAVP trials in patients with acquired platelet dysfunction (78%) resulted in normalization or shortening of the prolonged bleeding times by at least 4 min. The bleeding time response did not correlate with changes in the levels of von Willebrand factor (vWf) antigen or ristocetin cofactor activity, nor was it associated with changes in vWf multimeric analysis or in vitro platelet aggregations following the administration of DDAVP. Shortening of the bleeding time with DDAVP was seen in patients with a failure to release/storage pool type defect, thromboxane synthesis type defect, Bernard‐Soulier syndrome, Glanzmann's thrombasthenia, the May‐Hegglin anomaly, liver disease, nonuremic renal disease, myelofibrosis, and Tangier's disease.
Porphyria cutanea tarda after allogeneic bone marrow transplantation for chronic myelogenous leukemia
Authors: Guyotat D. , Nicolas J. F. , Augey F. , Fiere D. , Thivolet J. 
Published: February 2007

AbstractA case of porphyria cutanea tarda (PCT) occurring after bone marrow transplantation (BMT) is reported. A 43‐year‐old male with chronic myelogenous leukemia received an human leukocyte antigen (HLA)‐identical allogeneic transplantation with T‐cell depleted marrow. Because of graft rejection, a second transplant was performed 4 months later. A grade II acute graft‐ vs.‐host disease and a cytomegalovirus (CMV) infection were subsequently observed. Two years after the second transplant, cutaneous symptoms of PCT with typical biochemical abnormalities developed. Liver biopsy revealed signs of hepatitis with iron overload. CMV was isolated from liver tissue. The possible roles of underlying disease, BMT, and CMV liver disease are discussed in view of the recently reported cases of PCT in patients with AIDS or hematological disorders.
Varied expressions of receptors on the human megakaryoblastic cell line MEG‐01 by monoclonal antibodies
Authors: Imamura Nobutaka , Mtasiwa Deodatus M. , Inada Tominari , Kuramoto Atsushi , Ogura Michinori , Saito Hidehiko 
Published: February 2007

AbstractWe herein describe the regulation of specific receptors on the megakaryoblastic cell line MEG‐01 by means of fluorescence‐activated cell sorting (FACS IV) with a panel of monoclonal antibodies (MAbs). MEG‐01 cells expressed GpIIb/IIIa (CD41a) and OKM5 (CD36) antigens on their cell surface, whereas they showed only a little expression of GPIb (CD42b), suggesting that these are megakaryoblastic cells.The up regulation of von Willebrand factor receptor (GPIb) and thrombospondin receptor (GPIV) and the down regulation of fibrinogen receptor (GPIIb/IIIa) and C3bi receptor (CD11b) were found by incubation with MAbs AN51 (CD42b), OKM5 (CD36), J15 (CD41a), and OKM1 (CD11b), respectively. This phenomenon was enhanced in the Ca2+ containing medium, except for the experiment with OKM5 (CD36) MAb. We thus suggest that several kinds of receptors on the surface of MEG‐01 cells are dexterously regulated through stimulation from outside the cell.
Serum transferrin receptor measurements in hematologic malignancies
Authors: Klemow Dawn , Einsphar David , Brown Todd A. , Flowers Carol H. , Skikne Barry S. 
Published: February 2007

AbstractAn enzyme‐linked immunosorbent assay using specific monoclonal antibodies was used to measure circulating transferrin receptor (TR) in 87 patients with various hematologic malignancies. The mean serum TR was significantly elevated in patients with myeloproliferative disorders (15.47 ± 12.54 μg/ml), whereas there were no differences in chronic granulocytic leukemia (7.89 ± 3.56 μg/ml), myelodysplastic disorders (9.25 ± 4.73 μg/ml), and acute nonlymphocytic leukemia (3.85 ± 3.50 μg/ml) as compared to normal (5.63 ± 1.42 μg/ml). Among patients with lymphoproliferative disorders, the mean level was normal in lymphoma (5.73 ± 2.59 μg/ml), multiple myeloma (5.47 ± 1.31 μg/ml), and hairy cell leukemia (7.04 ± 3.69 μg/ml). The serum TR was significantly elevated in chronic lymphocytic leukemia (CLL; 14.17 ± 12.29 μg/ml), and the serum levels reflected the clinical stage of the disease. These findings suggest that serum TR measurement may provide a useful laboratory index of disease activity in certain disorders such as CLL, whereas it most likely reflects the intensity of erythropoiesis in the remaining hematological disorders that were evaluated in this study.
Human immunodeficiency virus‐1 disease progression in hemophiliacs
Authors: Becherer Paul R. , Smiley M. Lynn , Matthews Thomas J. , Weinhold Kent J. , McMillan Campbell W. , White Gilbert C. 
Published: February 2007

AbstractA retrospective study of 153 hemophiliacs infected with human immunodeficiency virus‐1 (HIV‐1) was performed to determine the clinical and immunological consequences of HIV‐1 infection and the markers and cofactors associated with these changes. Nearly 80% of HIV‐1‐infected hemophiliacs have developed a significant reduction in their CD‐4+ counts (<400 CD‐4+ cells/mm3) with 40% having less than 200 CD‐4+ cells/mm3 by the end of 1987. The rate of CD‐4+ cell count decline was slightly greater in patients who have already developed the acquired immunodeficiency syndrome (AIDS) compared to those who have not (50 vs. 31 cells/mm3/6 months). Thrombocytopenia and older age were associated with a more rapid CD‐4+ count deterioration, but the quantity of clotting factor utilized did not affect immunologic progression. In patients with < 200 CD‐4+ cells/mm3, the incidence of AIDS was significantly higher in adults (>21 years old) compared to children/adolescents. Cytomegalovirus (CMV) seroprevalence increased with age but did not correlate with the amount of concentrated clotting factor used. Although there was no relationship between CMV status and progression to AIDS, CMV‐seropositive patients were older and had a lower CD‐4+ count. Thus the majority of HIV‐1‐infected hemophiliacs are developing progressive immune dysfunction measured by CD‐4+ count decline. This drop in CD‐4+ count significantly correlates with a risk for the development of AIDS in adults but not in children (<21 years old).
Responsiveness of bone marrow erythropoietic stem cells (CFU‐E and BFU‐E) to recombinant human erythropoietin (rh‐Ep) in vitro in aplastic anemia and myelodysplastic syndrome
Authors: Aoki Isao , Homori Masashi , Chikazawa Hiroo , Ishikawa Kyozo , Higashi Katsumi 
Published: February 2007

AbstractResponsiveness of bone marrow erythropoietic stem cells (CFU‐E and BFU‐E) to recombinant human erythropoietin (rh‐Ep) was examined in vitro in 23 patients with aplastic anemia and 14 with myelodysplastic syndrome (MDS) to investigate the clinical use of rh‐Ep for these diseases. Bone marrow mononuclear cells were cultured by methylcellulose methods for CFU‐E and BFU‐E assays. In normals, the CFU‐E numbers reached a plateau of increase at Ep doses of almost 2–5 units, and no further increase was observed with the addition of larger Ep doses. In aplastic anemia, the responses of CFU‐E to Ep were relatively good in nonsevere type and generally poor in severe type. However, the CFU‐E numbers increased with increasing doses of Ep in some of the patients with aplastic anemia. Among the patients with MDS, the responses of CFU‐E to Ep were relatively good in primary acquired refractory anemia (PARA) and primary acquired sideroblastic anemia. On the other hand, the responses of CFU‐E to Ep were poor in refractory anemia with an excess of blasts (RAEB) and RAEB in transformation among the MDS patients. BFU‐E responses to Ep were poor in severe aplastic anemia, RAEB, and RAEB‐T. However, there are Ep responsive patients in some of aplastic anemia and PARA. High titers of rh‐Ep were suggested to be effective clinically in some patients with aplastic anemia and those with PARA.
Effect of a factor VIII concentrate on type IIB von willebrand's disease‐associated thrombocytopenia presenting during pregnancy in identical twin mothers
Authors: Ieko Masahiro , Sakurama Shoki , Sagawa Akira , Yoshikawa Megumi , Nakagawa Shoichi , Satoh Masahiro , Yasukouchi Taro 
Published: February 2007

AbstractMarked thrombocytopenia developed during pregnancy in both identical twins mothers who had systemic lupus erythematosus (SLE) and also type IIB von Willebrand's disease (vWD).The proband's platelet count decreased in the third trimester of pregnancy. Large‐dose γ‐globulin and prednisolone treatments were performed because of the suspicion of immune thrombocytopenic reaction associated with SLE. These treatments were not effective. Her platelet count returned to the normal range immediately after delivery. Postpartum examinations revealed the decreased ristocetin cofactor activity and the deficiency of large von Willebrand factor (vWF) multimers in preserved plasma samples from the third trimester. These abnormal findings improved after delivery. Investigation of family members revealed that the proband had inherited type IIB vWD from her mother.The other twin, who was also under treatment for SLE, became pregnant about 1 year after delivery in the proband and followed almost the same course as that observed in the proband. As bleeding tendency was observed a few days before delivery, a factor VIII concentrate (Haemate P) was administered to compete with her variant vWF. This concentrate could prevent the further decrease in her platelet count, thereby correcting the hemorrhagic tendency. It seems evident that factor VIII concentrate would be effective in treating thrombocytopenia associated with type IIB vWD.
Polymerase chain reaction amplification applied to the direct detection of a 4 bp deletion in the promoter region of the Aγ gene
Authors: Manca Laura , Cocco Elena , Masala Bruno , Gilman John G. 
Published: February 2007

AbstractWe report here the identification of a 4 bp deletion in the AγT globin gene promoter by means of Fnu4Hl digestion of DNA amplified by polymerase chain reaction (PCR). This deletion has been previously associated with haplotype II β‐thalassemia in Sardinia. This simple, non‐radioactive procedure should facilitate the screening of various populations of normal and β‐thalassemic subjects for this specific genetic alteration.
Thrombocytopenic purpura in HIV‐seronegative users of intravenous cocaine
Authors: Koury Mark J. 
Published: February 2007

AbstractSevere thrombocytopenic purpura occurred in seven patients who had histories of recent or current intravenous cocaine use. All of the patients denied heroin use and all were seronegative for antibodies to the human immunodeficiency virus (HIV). The seven patients had normal or increased numbers of megakaryocytes in their bone marrows. While receiving corticosteroid therapy their platelet counts rose to 1011/L over periods ranging from 5 to 11 days. In view of the widespread use of cocaine, severe thrombocytopenia in the HIV‐seronegative patient should suggest the possibility of current or recent intravenous use of cocaine.