bluuebunny/crossref_metadata_2025_split huggingface dataset:
Circulating CD34+ cells: An adverse prognostic factor in the myelodysplastic syndromes
Authors: Sullivan Sally A. , Marsden Katherine A. , Lowenthal Raymond M. , Jupe David M. , Jones Michael E. 
Published: February 2007

AbstractAs part of an epidemiological survey of myelodysplastic syndromes (MDS) in southern Tasmania, 62 MDS patients identified over a 2 year period were tested for the presence of CD34, the human progenitor cell antigen (HPCA), in their peripheral blood. The results were correlated with transformation to acute myeloid leukemia (AML) and patient survival, and CD34+ status was compared as a prognostic indicator with Bournemouth score, cytogenetics, and CFU‐GM colony growth which were also assessed. Circulating CD34+ cells were found in 23 of the 62 MDS patients; 9 of the 23 patients with circulating CD34+ cells transformed to AML, as compared with none of the 39 CD34 negative patients (P < 0.0001); and 11 of the 23 patients with circulating CD34+ cells were dead at the end of the 2 year period, as opposed to 6 of the 39 with no CD34+ cells (P < 0.03). The Bournemouth score was also significantly associated with transformation to AML (P < 0.0001) and poor survival (P < 0.04). These were the only significant associations of the possible prognostic factors studied with either transformation or survival. In summary, the presence of circulating CD34+ cells was significantly associated with both progression to AML and poor survival and was found to be a better prognostic indicator than cytogenetics or CFU‐GM colony growth.
Survival of hemophilic males with acquired immunodeficiency syndrome with and without risk factors for AIDS other than hemophilia
Authors: Holman Robert C. , Rhodes Phillip H. , Chorba Terence L. , Evatt Bruce L. 
Published: February 2007

AbstractBetween January 1, 1981, and June 30, 1990, 1,514 hemophilia‐associated acquired immunodeficiency syndrome (AIDS) cases in males were diagnosed in the United States. In 1,394, hemophilia was reported as the sole risk factor. For an additional 120, other risk factors were reported: of 101 of these, 40 had homosexual/bisexual activity, 53 had a history of intravenous drug use, and 8 had both of these risk factors. We examined the demographic data and the survival data of two principal groups: males for whom hemophilia was the sole reported risk factor for human immunodeficiency virus (HIV) exposure, and hemophilic males for whom homosexual/bisexual activity, intravenous drug use, or both of these additional risk factors were reported. The survival curves showed marginal differences between the hemophilia‐only and the multiple risk groups; the median survival times were 13.1 and 14.6 months, with the cumulative probability of survival at 1 year as 52.7% and 54.0%, respectively. Kaposi's sarcoma was among AIDS indicator diseases more commonly found in the multiple risk factor group. Pneumocystis carinii pneumonia was the sole reported diagnosis indicative of AIDS for 34.4% of those in the hemophilia‐only group, compared with 20.8% of those with multiple risk factors. The principal demographic difference between the two groups was the age distribution; those in the multiple risk factor group were primarily between 20 and 44 years of age. Restricting the analysis to those between 20 and 44 years resulted in a slightly longer survival time in the hemophilia‐only group and no appreciable difference between the disease distributions and survival curves of the two groups. © 1992 Wiley‐Liss, Inc.
DNA methylation patterns of the γδβ‐globin genes in human fetal and adult erythroid tissues
Authors: Loo L. S. L. , Cauchi M. N. 
Published: February 2007

AbstractAn investigation of the correlation between the γ→β‐globin switch and DNA methylation was carried out. The restriction patterns obtained with methylation‐sensitive and ‐insensitive enzymes indicated hypomethylation in the promoter region of the γ‐globin genes in fetal liver DNA but high methylation of the same region in all other samples (except in the presence of an elevated erythroblast count or leukemia). All samples appeared to be partially hypomethylated at the 5′ end of the δ‐globin gene and hypomethylated at the 3′ region of the β‐globin gene. Although consistent with a role for DNA methylation in globin gene regulation, the results also suggest that other factors besides methylation may be required for regulation of the level of expression, and switching of the globin genes. © 1992 Wiley‐Liss, Inc.
Acute undifferentiated leukemia (AUL): A case report and a proposed system of classification
Authors: Shende Ashok C. , Bonagura Vincent R. , Cheah Marc S. C. , Lanzkowsky Philip 
Published: February 2007

AbstractThis report describes a case of HLADR+, CD34‐ acute undifferentiated leukemia (AUL) diagnosed in an 18‐year‐old male. A definition of AUL and a system for its classification are proposed on the basis of the current state of knowledge about phenotypic features of AUL cells and their clonal counterparts that exist during early stages of normal hematopoiesis. © 1992 Wiley‐Liss, Inc.
Reduced expression of CD20 antigen as a characteristic marker for chronic lymphocytic leukemia
Authors: Almasri Nidal M. , Duque Ricardo E. , Iturraspe Jose , Everett Eric , Braylan Raul C. 
Published: February 2007

AbstractThe surface antigens expressed by the cells of chronic lymphocytic leukemia (CLL) are well known. Most CLL are monoclonal B‐cell lymphoproliferative disorders characterized by the coexpression of B‐cell antigens and CD5, an antigen present predominantly on T cells. Very little attention, however, has been paid to the quantitative characteristics of the expression of B‐cell antigens in CLL. In this study, we used flow cytometry to analyze the expression of CD20, a well‐known B‐cell‐associated antigen, in lymphocytes from 42 cases of CLL and its tissue counterpart, small lymphocytic lymphoma (SLL), and compared the results with results obtained from the analysis of 21 follicular lymphomas, 20 hyperplastic reactive nodes, and 26 samples of normal peripheral blood. The intensity of CD20 expression in the CLL/SLL cells was significantly lower than that of B cells in the other categories. This antigen expression abnormality does not appear to be a universal phenomenon in CLL/SLL, since CD19, another pan‐B antigen, was expressed in CLL/SLL at levels higher than those in follicular lymphomas and comparable to those in reactive lymph nodes. These results indicate that the low CD20 expression can be used as a marker for CLL/SLL. The few cases exhibiting intense CD20 expression may represent a biologically different disease. CLL/SLL cells faintly expressing CD20 also show concomitant low CD5 expression in a manner not observed in normal CD5‐expressing B cells. © 1992 Wiley‐Liss, Inc.
Relationship of thrombohemorrhagic complications to endothelial cell function in patients with chronic myeloproliferative disorders
Authors: Friedenberg W. R. , Roberts R. C. , David D. E. 
Published: February 2007

AbstractThrombotic and hemorrhagic disorders are common complications of the myeloproliferative disorders. Endothelial cells release both procoagulant and profibrinolytic factors, which may contribute to these hemorrhagic or thrombotic complications. The pre‐ and postvenous stasis levels of the procoagulant and profibrinolytic factors produced by endothelial cells were correlated with the occurrence of complications in polycythemia rubra vera (PRV) patients (n = 29) and essential thrombocythemia (ET) patients (n = 17) compared with normal patients (n = 17). Tissue plasminogen activator (tPA) activity, plasminogen activator inhibitor (PAI) activity, von Willebrand's factor (vWF) activity and antigen (vWF:Ag), and factor VIII activity were measured. The resting tPA activity was significantly higher in the two disease groups compared with normal controls, but no difference between the levels of tPA and either complication within the disease groups was observed. Significantly elevated tPA following venous stasis was observed in the patients of both disease groups who had bleeding complications. Significant decreases, compared with the normal group, in both resting and postvenous stasis levels of PAI were observed in the disease groups regardless of complication history. The subjects from both disease groups with thrombotic complications had significantly elevated resting vWF and both resting and postvenous stasis vWF:Ag levels compared with normal controls. The endothelial cell is likely to be responding to abnormal hemastasis rather than being primarily involved in the genesis of a hyper‐ or hypocoaguable state. © 1992 Wiley‐Liss, Inc.
Reduction in tissue iron stores with a new regimen of continuous ambulatory intravenous deferoxamine
Authors: Olivieri Nancy F. , Davis Susan A. , Liu Peter P. , Berriman Ann Marie , Tyler Beverley J. , Francombe William H. 
Published: February 2007

AbstractA new regimen of 24‐hr ambulatory continuous intravenous infusion of deferoxamine (CIV OFO) through central venous ports was instituted in nine patients aged (mean ± SD) 22.4 ± 5.8 years over a period of 15.7 ± 7.3 months. Central venous infusion sites were changed weekly in the clinic, eliminating the necessity for reconstitution of DFO and needle Insertion at home. Because CIV DFO could be interrupted only by medical personnel, patient compliance was documented accurately; patients administered 93.0% ± 3.2% of CIV DFO prescribed. Mean urinary iron excretion on CIV DFO (66.8 ± 50.4 mg/24 hr) was significantly greater than that quantitated during 12‐hr equivalent‐dose subcutaneous DFO infusions (23.4 ± 18.3 mg/24 hr; P < 0.025). Mean serum ferritin declined by 71% over the treatment period (P < 0.005). This regimen confers the advantages of uninterrupted exposure to DFO, is associated with excellent patient compliance, and should be considered in any patient with severe iron overload and erratic compliance with DFO. © 1992 Wiley‐Liss, Inc.
CD5 B lymphocyte antigen in monoclonal gammopathy
Authors: Bataille RÉGis , Duperray Christophe , Zhang Xue Guang , Brochier Jean , Klein Bernard 
Published: February 2007

AbstractBecause B lymphocytes bearing the CD5 antigen have been involved in many B‐cell malignancies, we have investigated the presence of the CD5 B‐cell antigen on B and plasma cells in monoclonal gammopathy. Quantification of CD5 B cells was made in the peripheral blood of seven individuals with monoclonal gammopathy of undetermined significance (MGUS) and in that of 21 patients with multiple myeloma (MM). The bone marrow of ten patients with MM was also studied. Patients with progressive MM presented a significant reduction in both B and CD5 B lymphocytes (i.e., percentages and absolute numbers), when compared with individuals with MGUS and patients with stable MM. These latter individuals and patients did not differ from healthy donors. No CD5 B cells were found in the bone marrow of patients with MM. Moreover, no CD5 antigen could be detected on eight freshly established human myeloma cells lines including six totally dependent on Interleukin‐6. However, it was weakly expressed on two standard myeloma cell lines not requiring exogenous interleukin‐6 (i.e., RPMI 8226 and U 266). In conclusion, our data show mainly an overall reduction of the polyclonal CDS B lymphocytes similar to what is observed for the other polyclonal B lymphocytes in patients with active MM. Finally, the expression of the CDS antigen human myeloma cell lines is not constant. © 1992 Wiley‐Liss, Inc.
Inefficacy of intravenous immunoglobulin in patients with low‐risk thrombotic thrombocytopenic purpura/hemolytic‐uremic syndrome
Authors: Finazzi Guido , Bellavita Piero , Falanga Anna , Viero Piera , Barbui Tiziano 
Published: February 2007

AbstractObjective: To assess the efficacy of intravenous immunoglobulin (IVIG), in comparison with plasma exchange (PE), in the treatment of patients with thrombotic thrombocytopenic purpura/hemolytic‐uremic syndrome (TTP/HUS). © 1992 Wiley‐Liss, Inc.Design: Prospective, nonrandomized comparative study.Setting: Hematology department in a general hospital.Patients: 17 consecutive adult patients, six of them pregnant, with diagnosis of TTP/ HUS. Three had a severity score at diagnosis ≦4 and were treated with IVIG and 14 had a severity score of ≧5 and/or were pregnant and received PE. The response was evaluated after 5 days of therapy.Results: Complete remission was obtained in 0/3 cases treated with IVIG and 10/14 (71%) with PE (Fisher's exact test P = 0.05). Three patients died for widespread TTP‐HUS, and four had persistent disease. In three of the four resistant patients, complete remission was obtained by further PE but not by further IVIG. The overall remission rate was 76% (13/17).Conclusions: Our study does not confirm the utility of IVIG in the management of TTP‐HUS, as suggested by earlier single case reports. © 1992 Wiley‐Liss, Inc.
Proliferation of abnormal bone marrow histiocytes, an undesired effect of granulocyte macrophage‐colony‐stimulating factor therapy in a patient with hurler's syndrome undergoing bone marrow transplantation
Authors: Lang Eric , Cibull Michael L. , Davey Diane D. , Gallicchio Vincent S. , Jean Henslee‐Downey P. , Harder Edward J. , Messino Michael J. 
Published: February 2007

AbstractGranulocyte macrophage‐colony‐stimulating factor (GM‐CSF) has shown promise as a means of alleviating leukopenia associated with a wide variety of disorders. It is currently undergoing evaluation as an adjunct to bone marrow transplantation but its use in patients with metabolic disorders, such as Hurler's syndrome (HS), has not been explored. We followed bone marrow morphology in a 2‐year‐old male with HS who received up to 8 μg/kg GM‐CSF per day because of failure of allogeneic bone marrow engraftment. Both premortem and postmortem bone marrow sampling revealed almost complete replacement of the marrow space by sheets of histiocytes demonstrating metachromatic cytoplasmic granules. Such cells were present in far greater numbers than are usually seen in untreated patients with HS or patients with HS undergoing successful bone marrow transplantation without GM‐CSF. Moreover, the in vitro culture of bone marrow from a second HS patient showed a GM‐CSF dose‐related increase in colony formation up to a dose of 250 units/ml. Microscopic examination of these colonies showed a high percent‐age of histiocytes identical to those seen in the patient's bone marrow. These observations suggest that caution should be exercised when considering administration of CSFs to patients with HS and similar metabolic storage diseases. © 1992 Wiley‐Liss, Inc.
Intrathoracic extramedullary hematopoietic tumor in hemoglobin H disease
Authors: Wu Jin‐Hou , Shih Lee‐Yung , Kuo Tseng‐Tong , Lan Ray‐Shee 
Published: February 2007

AbstractWe report a Chinese patient with hemoglobin H (Hb H) disease who developed intrathoracic extramedullary hematopoiesis (EMH) 17 years following splenectomy for a blunt abdominal injury. The patient initially presented with extreme hyperbilirubinemia and multiple intrathoracic tumors. Hb H disease was diagnosed after investigation, and the marked jaundice, which declined gradually after supportive treatment, was attributed to his chronic hemolysis superimposed on an acute hepatitis C virus infection. A biopsy of the intrathoracic tumors revealed an EMH. Intrathoracic EMH, which is usually encountered in patients with β‐thalassemia and hereditary spherocytosis, has never been reported in Hb H disease. In areas where thalassemia is prevalent, EMH should be considered in the differential diagnosis of patients who have chronic anemia with asymptomatic intrathoracic tumor to avoid unnecessary surgical interventions. © 1992 Wiley‐Liss, Inc.
Alpha granule proteins in type I von Willebrand's disease
Authors: McKeown Laurie P. , Williams Sybil B. , Shafer Brenda , Murray Natalie , Gralnick Harvey R. 
Published: February 2007

AbstractPlatelet von Willebrand factor (vWf) is located in the alpha granules. Individuals with type I von Willebrand's disease (vWd) with prolonged bleeding times are best discriminated from those who have normal bleeding times by the normal level of platelet vWf ristocetin cofactor activity (vWf activity) and, to a lesser extent, by their platelet vWf antigen content. We have studied the content of adhesive proteins and platelet factor‐4 (PF‐4), and β‐thromboglobulin (βTG) in the platelet alpha granules of types I and III vWd patients to determine if other alterations in alpha granule contents or proteins occur in vWd. We found that type I vWd patients with prolonged or normal bleeding times could not be differentiated on the basis of their platelet levels of βTG, PF‐4, fibronectin, or fibrinogen. The levels of the alpha granule constituents in the type I vWd patient were similar to normal except for the platelet fibrinogen concentration. Patients with type I vWd, regardless of the level of platelet vWf activity or antigen, had increased levels of platelet fibrinogen. The patients with type III vWd who had undetectable levels of platelet and plasma vWf also had increased levels of platelet fibrinogen. In our study we could not attribute the variation in the platelet vWf activity and antigen in type I vWd to the size of the alpha granule pool as determined by the measurement of other alpha granule proteins. The mechanism(s) of increased platelet fibrinogen in these vWd patients is at present unknown. © 1993 Wiley‐Liss, Inc.
Unusually large von willebrand factor multimers preferentially promote young sickle and nonsickle erythrocyte adhesion to endothelial cells
Authors: Wick Timothy M. , Moake Joel L. , Udden Mark M. , McLntire Larry V. 
Published: February 2007

AbstractSickle red blood cells (RBC) suspended with endothelial cell (EC)‐derived unusually large (UL) von Willebrand factor (vWF) multimers, but not large plasma vWF forms, adhered to human venous EC under shear flow conditions. When sickle RBC were separated by density gradient centrifugation, fractions rich in less dense RBC were the most adhesive to EC in the presence of ULvWF. Incubation of sickle RBC with monoclonal antibodies against platelet surface receptors GPlb or GPIIb/llla, or with the integrin receptor agonist Arg‐Gly‐Asp‐Ser (RGDS) decreased the ULvWF‐mediated sickle RBC adhesion to EC 84%, >99%, and 90%, respectively. When incubated with EC before the flow studies, anti‐GPIb antibody and RGDS inhibited the ULvWF‐mediated sickle RBC adhesion to EC.ULvWF also promoted the adhesion to EC of nonsickle RBC (HbAA) from patients with an increased proportion of young erythrocytes. When the EC supernatant was depleted of most vWF forms, young nonsickle RBC adhesion decreased by 90%. Preincubation of young nonsickle RBC with anti‐GPIb antibody, anti‐GPIIb/llla antibody, or RGDS inhibited the ULvWF‐mediated young RBC adhesion to EC by 47%, 88%, and 92%, respectively.These data indicate that (1) low‐density erythrocyte fractions enriched in young sickle or young nonsickle RBC are capable of binding ULvWF multimers via GPIb‐like and GPIIb/IIIa‐like receptors; (2) the RBC vWF receptors are lost or modified as erythrocytes age in the circulation; and (3) ULvWF/RBC complexes also bind to EC via a GPIb‐like receptor. © 1993 Wiley‐Liss, Inc.
8;21 translocation and multilineage involvement
Authors: Kwong Y. L. , Ching L. M. , Liu H. W. , Lee C. P. , Pollock A. , Chan L. C. 
Published: February 2007

AbstractThe translocation (8;21)(q22;q22) is commonly associated with acute myeloid leukemia (AML) M2 according to the French‐American‐British (FAB) classification. We describe 11 cases of t(8;21) diagnosed by strict FAB criteria. Six cases were diagnosed as AML M2, 3 cases as AML M4, 1 case as refractory anemia with excess of blasts in transformation, and 1 case as Philadelphia chromosome negative chronic myeloid leukemia in acceleration. Translocation (8;21) could thus occur in a wider variety of hematological abnormalities. Accordingly, we propose that t(8;21) may involve different hemopoietic lineages.
Primary plasmacytoma of generalized lymph nodes: A long survivor
Authors: Matsushima Takafumi , Murakami Hirokazu , Tamura Jun'Ichi , Sawamura Morio , Naruse Takuji , Tsuchiya Jun 
Published: February 2007

AbstractExtramedullary plasmacytomas are occasionally seen in the course of multiple myeloma; however, primary plasmacytomas are unusual. Most of the primary plasmacytomas arise in the upper respiratory system, but a plasmacytoma of the lymph nodes is very rare. A case of a 56‐year‐old female with primary plasmacytoma in the lower mandibular, cervical, axillary, and para‐aortic lymph nodes accompanied by IgA Kappa paraproteinemia is described. After ten courses of combination chemotherapy with cyclophosphamide and prednisolone, she obtained complete response. However, 6 years later, she had a relapse of plasmacytoma in the generalized lymph nodes. She received ten treatments of melphalan‐prednisolone and again obtained complete response. She has remained well without chemotherapy or recurrent signs for 9 years. It is considered that conventional chemotheapy can be useful for primary plasmacytoma of generalized lymph nodes.
CD34+ and CFU‐GM progenitors are significantly decreased in sivsmm9 infected rhesus macaques with minimal evidence of direct viral infection by polymerase chain reaction
Authors: Hillyer Christopher D. , Lackey Dixon A. , Villinger Francois , Winton Elliott F. , McClure Harold M. , Ansari Aftab A. 
Published: February 2007

AbstractHematologic abnormalities in the peripheral blood and bone marrow are associated with human immunodeficiency and simian immunodeficiency virus (HIV, SIV) infection. The reasons for these abnormalities remain unclear. Bone marrow specimens collected from uninfected animals (Group A, Controls) and from rhesus macaques experimentally infected with SIVsmm9 during the asymptomatic stage (Group B, SIV+ “well”) and during the clinically ill stage (Group C, SIV+ “sick”), underwent a variety of in vitro assays of hematopoiesis. Colony forming unit‐granulocyte/macrophage (CFU‐GM) per plate growth was 46.7 ± 7.7, 31.9 ± 8.4 and 6.5 ± 5.0 (means ± sd, P <.02 each mean compared to the others) in the 3 groups respectively. Burst forming unit‐erythroid (BFU‐E) growth was similarly decreased in bone marrow samples from the SIV+ animals. There was no change in the number of CFU‐GM per plate with the removal of plastic adherent or T‐cell mononuclear cell fractions. There was no increase in CFU‐GM per plate growth with the addition of low dose GM‐CSF (1 or 5 ng/mL) though there was a near 67% increase (48 to 80 CFU‐GM per plate) with the addition of 100 ng/mL recombinant rhesus IL‐3 and 100 ng/mL GM‐CSF in SIV+ 'sick' animals. Variation in frequency of CD34+ progenitor cells in SIV+ animals was seen, with 3.0% CD34 cells in SIV− controls, 4.9% CD34+ cells in SIV+ 'well' animals and 0.5% CD34+ progenitor cells in SIV+ 'sick' monkeys (P <.01, each mean compared to the others). Finally, there was minimal evidence of SIV sequences by polymerase chain reaction in pooled cultured CFU‐GM, and no evidence in flowcytometrically sorted CD34+ progenitor cells from selected animals. Thus, the SIV seropositive rhesus monkey appears to have similar hematopoietic aberrations as are found in HIV infected human subjects and may be an excellent model for studying the interaction of lentiviruses on the kinetics of blood formation.