bluuebunny/crossref_metadata_2025_split huggingface dataset:
Megakaryoblastic transformation of polycythemia vera associated with hypercalcemia
Authors: Wong K. F. , Chan J. K. C. , Ma S. K. , Chu Y. C. 
Published: February 2007

AbstractLeukemic transformation is not uncommon in polycythemia vera, particularly after treatment with chemotherapeutic agents. The leukemias that supervene are mostly of myeloid type but megakaryoblastic transformation is distinctly uncommon. We report a case of polycythemia vera terminating in megakaryoblastic leukemia with associated hypercalcemia.
Acute lymphoid leukemias following either a previous chronic myelogenous leukemia or myelodysplastic syndrome: Phenotypic and genomic differences
Authors: Hernández J. M. , Sánchez I. , González M. , Orfao A. , González‐Sarmiento R. , San Miguel J. F. 
Published: February 2007

AbstractWe have analyzed the immunological and genomic characteristics of the lymphoid blast cells present in secondary lymphoid leukemias following either a previous chronic myelogenous leukemia (CML) or myelodysplastic syndrome (MDS). Twenty‐one of 107 secondary leukemias analyzed displayed a lymphoid phenotype (15 after a CML and 6 after a MDS). Most of the lymphoid blast crises of CML (73%) correspond to pure lymphoid transformation, all of them having a common acute lymphoblastic leukemia (ALL) phenotype (CD10+). By contrast, in all MDS cases, lymphoid blast cells coexisted with another myeloid component (hybrid leukemias) and showed an early B phenotype. IgH and TCR‐γ gene rearrangements were detected in the CML‐lymphoid blast crisis (86% of cases) more frequently than in the MDS transformations (33%). The TCR‐β gene was in germ line configuration in all cases while TCR‐δ gene rearrangements were detected in four cases, all of them corresponding to a previous diagnosis of CML. These results show the existence of both immunophenotypic and genomic differences between the lymphoid transformations of either CML or MDS, which could reflect differences at the stage of maturation of the target cell in these transformations.
Relief of intractable pruritus in polycythemia vera with recombinant interferon alfa
Authors: Finelli Carlo , Gugliotta Luigi , Gamberi Barbara , Vianelli Nicola , Visani Giuseppe , Tura Sante 
Published: February 2007

AbstractFollowing reports that recombinant interferon alfa (rIFNα), besides inducing clinical and hematologic remission in polycythemia Vera (PV), can also resolve intractable pruritus, we used rIFNα to treat 13 PV patients complaining of severe pruritus refractory to conventional treatment (venesection and/or cytostatics). rFNα was administered intramuscularly three times a week at a dosage of 3.0 × 106 U. Eight patients (61.5%) reported a ± 50% reduction of pruritus, which occurred within 2–8 weeks from the start of rIFNα treatment, leading to a substantial improvement in their quality of life. Three patients had to stop rIFNα within the first month of therapy because of unacceptable side effects. Thus rIFNα seems to be capable of providing considerable relief of otherwise intractable pruritus in a good proportion of PV patients.
Malignant lymphoma originating from the earliest T‐lineage precursor cell
Authors: Hashimoto Yoshie , Yasukawa Masaki , Takada Kiyonori , Hato Takaaki , Fujita Shigeru 
Published: February 2007

AbstractThe earliest T‐lineage precursor cells with a CD3–CD4+CDB– phenotype and germlinestate T‐cell receptor (TCR) genes have recently been identified in the murine thymus. We report a case of malignant lymphoma that was considered to originate from the human counterpart of this newly defined murine T‐cell population. The surface phenotype of the lymphoma cells was CD2+CD3–CD4+CD8–CD25–CD34+CD44+HLA‐DR+. TCR‐β, ‐γ, ‐δ chain genes and immunoglobulin heavy and light chain genes were all in a germline state. These characteristics were markedly similar to those of the murine earliest T‐lineage precursor cells. The present case thus strongly suggests the presence of the human counterpart of this novel murine T‐cell population.
T lymphocytes from autoimmune thrombocytopenic purpura show a defective activation and proliferation after cytoplasmic membrane and intracytoplasmic mitogenic signals
Authors: Garcia‐Suarez J. , Prieto A. , Manzano L. , Reyes E. , Molto L. , Alvarez‐Mon M. 
Published: February 2007

AbstractT lymphocyte activation and proliferation are complex cellular processes involving membrane and cytoplasmatic molecules as well as the secretion and response to cytokines, mainly interleukin 2. There is increasing evidence that autoimmune thrombocytopenic purpura (ATP) is associated with an alteration of the regulation of the immune system. The blastogenic response of purified T lymphocytes to mitogens that interact with membrane molecules (phytohemaglutinin, anti‐CD3 monoclonal antibody) and with intracytoplasmic protein kinase C (phorbol myristate acetate) has been investigated in 22 ATP patients and 18 healthy controls. After the signal given by the three different mitogens [3H]‐thymidine uptake in T lymphocytes from ATP patients was found to be significantly decreased with respect to that found in healthy controls under similar experimental conditions (P < 0.05). Analysis of the cell cycle progression in these T lymphocytes from ATP patients, showed a significantly diminished percentage of cells in S‐phase after PHA stimulation (P < 0.05). The percentage of CD3+ cells in the CD2+ lymphocyte preparations was significantly decreased in ATP patients relative to healthy controls (P < 0.05). But there was no significant correlation between this percentage and the blastogenic response to PHA in the CD2+ cellular preparations from both groups of subjects. No significant differences were found in the percentages of CD4+ and CD8+ cells. These data indicate that the impaired blastogenic response of T lymphocytes from ATP patients may be ascribed to an intrinsic defect in these T cells. This defective proliferative response of T lymphocytes from ATP patients cannot be ascribed to either defective interleukin 2 production or receptor expression which were both similar to those of healthy controls (P > 0.05). And, the presence of saturating amounts of exogenous interleukin 2 did not normalize the defective proliferative response the mitogenic signals on the part of T lymphocytes from ATP patients. We conclude that T lymphocytes from ATP patients have a defective proliferative response to membrane and intracytoplasmatic mitogenic signals. © 1993 Wiley‐Liss, Inc.
Significant hyponatremia following DDAVP administration in a healthy adult
Authors: Humphries John E. , Siragy Helmy 
Published: February 2007

AbstractWe report the development of significant hyponatremia (121 mEq/L) following three daily intravenous doses of desmopressin acetate (DDAVP; 18 μg; 0.3 μg/kg) in a healthy adult with moderate von Willebrand's disease. Previous reports suggest that clinically important hyponatremia due to DDAVP administration only occurs in the very young, those receiving hypotonic intravenous fluids or those given multiple frequent (e.g., every 8–12 hours) doses of DDAVP. None of these conditions was present in this case. Consideration should be given to monitoring serum sodium levels and fluid balance in patients receiving more than a single dose of DDAVP, even in the absence of previously reported risk factors for the development of hyponatremia. © 1993 Wiley‐Liss, Inc.
Detection of the (–SEA) double α‐globin gene deletion by a simple immunologic assay for embryonic ζ‐globin chains
Authors: Ireland James H. , Luo Hong‐Yuan , Chui David H. K. , Chu Berbie , Yuen Juliet , Hsia Y. Edward 
Published: February 2007

AbstractHomozygous α‐thalassemia [α‐thal‐1], with loss of all four α‐globin genes, causes lethal hydrops fetalis. The most common mutation producing this syndrome is the Southeast Asian (–SEA) double α‐globin gene deletion. Erythrocytes from adults heterozygous for the (–SEA) deletion have minute amounts of embryonic ζ‐globin chains detectable by anti‐ζ‐globin monoclonal antibodies.Among 225 peripheral blood samples tested by a simple anti‐ζ‐immunobinding tetra‐zolium dye test, 81 were positive and 144 were negative. The majority of subjects were of Filipino, Chinese, or Laotian ancestry. All 81 positive samples were confirmed by Bam HI digests and a ζ‐cDNA probe to have the (–SEA) mutation. The (–SEA) double α‐deletion was the only abnormality in 58. In the others, it was combined with α‐globin or β‐globin mutations, or coincidental iron deficiency. Four other samples from (–SEA) heterozygotes were negative by this immunologic assay.Anti‐ζ negative samples included 78 deletions of the total α‐globin region, (–Tot), 23 single α‐globin deletions, and a variety of β‐globin mutations; 16 normocytic samples with normal α‐genes were also negative. Ten anti‐ζ positive and 25 anti‐ζ negative samples had benign triplicated ζ‐globin genes.In this population, the sensitivity of this test was 95%; and specificity for the (–SEA) mutation was 100%. Anti‐ζ immunobinding testing provides rapid, simple, and reliable screening for the (–SEA) double α‐globin deletion, although it does not detect the (–Tot) total α‐deletions. © 1993 Wiley‐Liss, Inc.
106 depletion of WBC with CF‐2, a new leukocyte removal filter
Authors: Endo Yoshihiro , Tani Tohru , Yokota Tohru , Tsutamoto Yoshihiro , Kodama Masashi 
Published: February 2007

AbstractA new leukocyte removal filter, Nipro CF‐2, was evaluated. A non‐woven polyester filter, gravity flow device that does not require priming and rinsing after use was developed to remove white blood cells from 400 ml of whole blood or concentrates of 400 ml of whole blood. A flow cytometric technique was developed to measure extremely low WBC counts. To evaluate the efficiency of this filter, leukocyte counts were measured by flow cytometry using a Coulter EPICS‐C cytometer. After filtration through a CF‐2 filter, more than 99.9999% of WBCs were depleted from 6 of 17 samples. The mean value of WBC depletion was 99.9994%. The CF‐2 filter achieved nearly a million‐fold depletion of WBCs from whole blood concentrates. © 1993 Wiley‐Liss, Inc.
Simultaneous demonstration of the Philadelphia chromosome in T, B, and myeloid cells
Authors: Mackinney Archie A. , Clark Steven S. , Borcherding Wayne , Fizzotti Marco , Hong Richard 
Published: February 2007

AbstractA patient presented with lymphoblastic lymphoma in lymph‐nodes and chronic myeloge‐nous leukemia (CML) in marrow and peripheral blood. All marrow and unstimulated peripheral blood cells contained the Philadelphia chromosome{t(9:22)}. Lymphoma cells were analyzed by flow cytometry and were identified as T cells (CD2+CD5+CD7+CD34+). All fresh lymphoma cells contained the t(9:22) translocation. Cultures of purified peripheral blood T and B cells and specifically stimulated NK cells revealed that 59% of the B cells, 10% of the NK cells, and none of the normal T cells contained the translocation. The lack of translocation in normal peripheral T cells is attributed to their long lifespan. No rearrangement of immunoglobulin or T cell receptor beta or gamma genes was found in either the leukemia or lymphoma cells. Analysis of the DNA from cryopreserved lymphoma biopsy showed clonal rearrangement within the common breakpoint cluster region of the bcr gene identical to the bcr rearrangement in DNA from leukemia blood cells. The data support the concept that T and B cells originate in the patient's totipotent stem cell from which the CML is also derived. © 1993 Wiley‐Liss, Inc.
Effects of sCD23 on proliferation of leukemic cells from a patient with chronic myelogenous leukemia during blast crisis
Authors: Morel F. , Delwail V. , Brizard A. , Meseri A. , Guilhot F. , Goube de Laforest P. , Lecron J. C. 
Published: February 2007

AbstractThe present study has attempted to further delineate the growth factor requirements of peripheral blasts of a patient with CML in acute phase. Phenotypic analysis of leukemic blasts from this patient before culture has shown a homogenous population of CD34+ cells at the onset of blast crisis. In the second and third samples the percentage of CD34+ DR+ blast cells decreased slightly and up to 32% of cells in the third sample expressed the CD19 antigen. Optimal proliferation of cells derived from the first sample required the presence of exogenous sCD23 and to a lesser extent IL7. The stimulatory effects of sCD23 and IL7 were clearly reduced 4 months later and no longer detected after 6 months. This variability in growth factor response along with disease progression may be related to phenotypic differentiation. There was no evidence for lymphoid or myeloid maturation after 4 days of liquid culture. Our results in conjunction with previous studies are in agreement with sCD23‐involvement in the complex control of proliferative processes at both normal and leukemic stages, demonstrating that cytokines are critical in determining CML cell proliferation. © 1993 Wiley‐Liss, Inc.
Amegakaryocytic thrombocytopenia with a positive direct Coombs' test
Authors: Koduri Prasad Rao 
Published: February 2007

AbstractA 50‐year‐old previously well woman developed a sudden onset of ecchymoses and petechiae over the trunk and extremities and gum bleeding. There was no history of alcohol use or drug ingestion. Physical examination was normal except for the ecchymoses, purpuric rash, and gum bleeding. Complete blood count showed Hb 8.0 g/dl, leucocytes 10.9 × 109/L with a normal differential, platelets 8 × 109/L and reticulocytes, 4%. The bone marrow was normocellular and showed absence of megakaryocytes; there was normoblastic erythroid hyperplasia and the myelopoiesis was normal. The direct Coombs test result was positive. There was poor response to treatment with prednisone and bolus doses of vincristine. Therapy with danazol resulted in the complete normalization of the blood counts, and the patient remains well 32 months after diagnosis and is currently taking 100 mg danazol daily. © 1993 Wiley‐Liss, Inc.
Effect of intravenous immunoglobulin on inhibition of fibrinogen binding to platelets by sera from patients with immune thrombocytopenia
Authors: Kamiyama Mikio , Yoshimura Yuichi , Chen Karen , Arkel Yale S. 
Published: February 2007

AbstractWe previously described an ELISA to measure the inhibition of platelet glycoprotein llb/IIIa (GPIIb/IIIa) binding to fibrinogen due to immune complexes and/or anti‐platelet antibodies from patients with immune thrombocytopenia (ITP) or HIV‐related ITP. Circulating immune complexes (CIC) were the main factor in the inhibition of GPIIb/IIIa binding to fibrinogen in HIV‐related ITP, whereas in non‐HIV ITP, inhibition was only partially due to CIC; anti‐platelet antibodies specific to GPIIIa were also shown to play a role. In this study, we correlated the rise in the platelet count after intravenous immunoglobulin (IVIG) infusion with the decrease in inhibition of fibrinogen binding to GPIIb/IIIa by the sera of patients with ITP and HIV‐related ITP. In the majority of the patients' sera tested, as the platelet count increased following the administration of IVIG, the degree of inhibition of GPIIb/IIIa binding to fibrinogen decreased. We also observed a decrease and/or disappearance of the antibodies specific to GPIIb and/or GPIIIa after IVIG administration. In HIV‐seronegative ITP patients, the decrease or disappearance of anti‐platelet antibodies directly correlated with the decreased inhibition of GPIIb/IIIa binding to fibrinogen by the 2% PEG supernatants of sera which contained anti‐platelet antibodies. These findings suggest that IVIG directly affects the binding of CIC and anti‐platelet antibodies to platelets and thereby improves platelet survival. Our results also suggest that the anti‐idiotypic effect may contribute to IVIG's therapeutic action. In contrast, in the HIV‐seropositive group, the decreased inhibition by PEG precipitates after IVIG administration was more strongly associated with an increase in the platelet count. © 1993 Wiley‐Liss, Inc.
Increased vascular endothelial cell markers in patients with disseminated intravascular coagulation
Authors: Wada Hideo , Minamikawa Kouzou , Wakita Yoshihiro , Nakase Tutomu , Kaneko Toshihiro , Ohiwa Michiaki , Tamaki Shigehisa , Deguchi Katsumi , Shirakawa Shigeru , Hayashi Tatsuya , Suzuki Koji 
Published: February 2007

AbstractWe examined vascular endothelial cell markers, thrombomodulin (TM), plasminogen activator inhibitor‐l (PAI‐I), tissue plasminogen activator (t‐PA), and von Willebrand factor, in 80 patients with disseminated intravascular coagulation (DIC). The levels of thrombin‐antithrombin III complex (TAT), plasmin‐α2 plasmin inhibitor complex (PIC) and FDP‐D‐dimer were significantly increased both before and after the onset of DIC, but were not correlated with organ failure or prognosis. However, the PIC/TAT ratio was lower in patients with poor prognosis than in those with good prognosis, and it was also lower in those with organ failure than in those without. Plasma TM, PAI‐I, and t‐PA levels were increased in DIC patients with organ failure or poor outcome, but were not significantly increased before the onset of DIC. We consider that the prognosis of patients with DIC might be related to organ failure or endothelial cell damage and that plasma levels of TM, PAI‐I, and t‐PA might be useful in the detection of these disorders and in assessing prognosis. A hypofibrinolytic state might enhance organ failure in patients with DIC. © 1993 Wiley‐Liss, Inc.
Phenotypic and genotypic characterization of Hodgkin's disease
Authors: Koduru P. R. K. , Susin M. , Schulman P. , Catell D. , Goh J. C. , Karp L. , Broome J. D. 
Published: February 2007

AbstractAlthough a few studies have reported clonal cytogenetic changes in Hodgkin's disease (HD), their correlation with histologic groups is poorly defined. This is because of insufficient numbers of clonally abnormal cases ascertained in each of these studies, an inherent problem associated with the cytogenetic studies of HD. In this report we present results of pathologic, phenotypic, and genetic studies on 29 HD tumors consecutively ascertained by us and the results of a comprehensive analysis of the cytogenetic data available in the literature. In our series 75% of the tumors were positive for Epstein‐Barr virus (EBV) by polymerase chain reaction (PCR) assay. A higher frequency of EBV‐positive tumors showed clonal karyotypic abnormalities than the EBV‐negative tumors. Unlike the case in the previous reports, none of the 24 tumors studied by PCR showed the presence of t(14;18) (q32;q21)‐carrying cells. From the comprehensive analysis of the literature, we identified recurring nonrandom numerical changes, deletions, and chromosome breaks in HD. Some of these are associated either with nodular sclerosis or with mixed cellular histologies. A comparison of the pattern of these nonrandom cytogenetic changes in HD and those reported for non‐Hodgkin's lymphomas (NHL) identified common deletions and breaks between them. These common genetic lesions probably play a role in disease evolution. © 1993 Wiley‐Liss, Inc.
Anticoagulant activity of an anti‐β2‐glycoprotein I antibody is depentent on the presence of β2‐glycoprotein I
Authors: Matsuda Juzo , Gohchi Kengo , Tsukamoto Miyo , Saitoh Noriko , Asami Katsuhisa , Hashimoto Masakatsu 
Published: February 2007

AbstractWe studied whether or not an anti‐β2‐glycoprotein I antibody (aGPI) had lupus anticoagulant‐like activity, employing the diluted Russel viper venom time (dRVVT) and kaolin clotting time (KCT) as indices. aGPI prolonged the dRVVT and KCT of β2‐glycoprotein I (GPI)‐depleted normal plasma in the presence of extrinsic GPI. This prolongation of the dRVVT and KCT occurred immediately after the addition of aGPI and GPI, and was subsequently enhanced further in a time‐dependent manner. The GPI/aGPI combinaion was judged to have lupus anticoagulant‐like activity by the dRVVT‐platelet neutralization test, but this was not confirmed by a lupus anticoagulant‐specific test, i.e., the activated partial prothrombin time (APTT) using hexagonal phospholipid. From these findings, it can be concluded that aGPI has lupus anticoagulant‐like activity in the presence of GPI, but may be a partially or considerably different antiphospholipid antibody from lupus anticoagulant. Further investigations may be needed to clarify this point. © 1993 Wiley‐Liss, Inc.
Hemoglobin sickle‐lepore: Report of two siblings and review of the literature
Authors: Seward Daniel P. , Ware Russell E. , Kinney Thomas R. 
Published: February 2007

AbstractHemoglobin Lepore is composed of two normal α‐globin chains and two δ‐β fusion globin chains that result from nonhomologous crossing over of genetic material during meiosis. The doubly heterozygous condition of sickle hemoglobin with Lepore hemoglobin (Hb S‐Lepore) is rare, having been described previously in only nine patients. We report two siblings with Hb S‐Lepore who have similar hematologic characteristics but a marked difference in clinical severity. © 1993 Wiley‐Liss, Inc.
Recombinant human granulocyte‐macrophage colony‐stimulating factor plus recombinant human erythropoietin may improve anemia in selected patients with myelodysplastic syndromes
Authors: Hansen Per Boye , Johnsen Hans E. , Hippe Erik , Hellström‐Lindberg Eva , Ralfkiaer Elisabeth 
Published: February 2007

AbstractThe purpose of this study was to improve erythropoiesis in patients with anemia due to myelodysplastic syndromes (MDS). We treated 13 patients first with recombinant human granulocyte‐macrophage colony‐stimulating factor (rhGM‐CSF) for 6 weeks, then with recombinant human erythropoietin (rhEpo) and rhGM‐CSF for the next 12 weeks. Five patients had refractory anemia (RA), 3 refractory anemia with ringed sideroblasts (RAS), and 5 refractory anemia with excess of blasts (RAEB). Ten patients were transfusion‐dependent at the time of inclusion. Eleven patients completed this phase II study. Five responded with an increase in hemoglobin level (3 patients) or a reduction in transfusion requirement (2 patients). We registered no response in the remaining 6 patients during treatment. Patients responding to combined treatment had relatively low concentrations of plasma Epo and plasma ferritin before treatment with rhEpo and a normal karyotype throughout the study. Long‐term bone marrow cultures did not predict the response. Still, responders seemed to have a higher number of colony‐forming progenitors than non‐responders. In conclusion, combined therapy with rhGM‐CSF and rhEpo may stimulate hematopoiesis and correct or improve anemia in some patients with MDS. © 1993 Wiley‐Liss, Inc.
Multimeric pattern of plasma and platelet von willebrand factor is normal in uremic patients
Authors: Castaman Giancarlo , Rodeghiero Francesco , Lattuada Antonella , La Greca Giuseppe , Mannucci Pier Mannuccio 
Published: February 2007

AbstractSeveral studies have reported increased von Willebrand factor (vWF) levels in plasma of uremic patients, with a normal multimeric pattern. Two recent studies, however, have shown a reduction of the larger multimers of plasma vWF and one has also found low levels of platelet vWF in uremic patients with prolonged bleeding time (BT). We have measured plasma and platelet vWF and analyzed its multimeric pattern in 20 uremic patients, 11 with a prolonged BT and 9 with a normal BT. For Plasma vWF, no difference for vWF:Ag, RiCof, and ristocetin‐induced platelet agglutination was found between the two groups with normal and prolonged BT. The multimeric pattern of plasma vWF, as evaluated by densitometric scanning of the electrophoretic gels, was normal in both groups. For platelet vWF, vWF:Ag and RiCof content was similar in the two groups. The multimeric pattern was indistinguishable from that of normal platelets. In conclusion, our study does not confirm the presence of a structural defect of plasma vWF and the reduction of platelet vWF content in uremia. © 1993 Wiley‐Liss, Inc.