bluuebunny/crossref_metadata_2025_split huggingface dataset:
Translocation (3;21) in Philadelphia chromosome—positive chronic myelogenous leukemia prior to the onset of blast crisis
Authors: Coyle Thomas , Najfeld Vesna 
Published: February 2007

AbstractTwo female patients in the chronic phase of CML were found to have, in addition to t(9;22), a new karyotypic abnormality—t(3;21)(q26;q22)—present in bone marrow cells. At diagnosis, this abnormality was observed in a small number of marrow cells in both patients, and as the disease progressed in patient 1, the percentage of cells showing t(3;21) was increased, reaching 100% as the transformation to blast crisis occurred. These observations suggest that t(3;21) may represent a new and rare nonrandom rearrangement which may be identified prior to the onset of blast crisis.
Immunogold staining in bone marrow aspirate for the study of surface markers in acute lymphoblastic leukemia
Authors: Penchansky Lila , Kaplan Sandra S. , Krause John R. 
Published: February 2007

AbstractThe diagnostic value of phenotyping acute lymphoblastic leukemia (ALL) of childhood using the immunogold staining method (IGS) was assessed in 34 consecutive new ALL patients seen at Children's Hospital of Pittsburgh during a 14‐month period. IGS proved to have considerable value in the diagnosis and classification of ALL. It allowed a sharp and clear identification of surface antigens, documented surface antigen heterogeneity in normal and abnormal cells, and provided a permanent record. The combination with cytochemistries allowed further cytoplasmic identification and correlation. This method does not require sophisticated equipment and can be performed in any laboratory, thus enabling results from different institutions to be more readily compared.
Influence of T lymphocytes on hematopoiesis in a patient with T cell hypoplasia
Authors: Hoffman Ronald , Lu Li , Jansen Jan , Bruno Edward , Brandt John , Boral Leonard , Cheung Killian 
Published: February 2007

AbstractIn this communication, we describe an unusual patient with a reported lifelong history of anemia. Investigation of the pathogenesis of this patient's bone marrow failure provided an interesting opportunity to determine the role of T cells in the regulation of human blood cell production. Phenotyping of the patient's mononuclear cells revealed severe T cell hypoplasia in both the peripheral blood and bone marrow. The patient's bone marrow was capable of producing 50–60% of the normal numbers of burst‐forming units—erythroid (BFU‐E) in the presence of optimal concentrations of erythropoietin, suggesting that marrow BFU‐E formation is in part independent of T cells. Addition of small numbers of class I identical donor T cells enhanced BFU‐E cloning efficiency to a level observed in normal controls. This enhancing effect was supplied by a T4+(CD4) population of donor cells. The addition of donor T cells partially corrected the inability of patient marrow cells to produce megakaryocyte and mixed colonies. These studies suggest that prolonged T cell hypoplasia might deprive marrow progenitor and stem cells of a necessary enhancing effect that is required for sustained normal hemato‐poiesis. Such a T cell defect in rare instances may result in bone marrow failure.
Dyserythropoiesis and erythroblast‐phagocytosis preceding pure red cell aplasia
Authors: Keefer Michael J. , Solanki Dilip L. 
Published: February 2007

AbstractA 50‐year‐old woman with typical acquired primary pure red cell aplasia (PRCA) was successfully treated with prednisone. A later relapse was preceded by a period of ineffective erythropoiesis characterized by a reticulocyte response inappropriately low for the degree of anemia, serum iron of 189 üg/dl, total iron‐binding capacity (TIBC) of 213 μ/g/dl, and erythroid hyperplasia. In addition there was marked dyserythropoiesis and erythroblast‐phagocytosis. One month later, bone marrow examination showed classic PRCA. This rarely reported evolutionary stage of PRCA has several implications: 1) it suggests antibody induced erythroblast cytotoxicity as one mechanism of PRCA; 2) at a particular time in the development of PRCA there is potential for misdiagnosis as primary refractory anemia (PRA); and 3) some cases of PRA with similar morphologic and laboratory findings may be pathogenetically related to PRCA and may benefit from evaluation for immune‐mediated suppression of erythropoiesis.
Thrombocytopenic purpura in a carrier of human T‐cell leukemia virus type I
Authors: Matsuoka Tomoko , Tamura Hiroshi , Fujishita Masatoshi , Kubonishi Ichiro , Taguchi Hirokuni , Miyoshi Isao 
Published: February 2007

AbstractThrombocytopenic purpura developed in a 64‐year‐old woman seropositive for human T‐cell leukemia virus type I (HTLV‐I). She had no underlying disorders and HTLV‐I is suggested as a possible cause of her thrombocytopenia.
Clinical studies with low‐molecular‐weight heparin(oid)s: An interim analysis
Authors: Gate Hugo Ten , Henny C. Pieter , Cate Jan W. Ten , Büller Harry R. 
Published: February 2007

AbstractIn this review an interim analysis was made of all clinical studies performed with low‐molecular‐weight heparin(oid)s (LMWH) up to January 1987. Thus far, many experimental studies on LMWH show these substances to have an increased benefit/risk ratio concerning efficacy and bleeding as compared to standard heparin. In man, this increased ratio was verified in some small open studies. However, until the present day, controversy still exists. Some of the difficulties In assessing the results of different investigations include the existence of various kinds of LMWH and the lack of a consensus as to the unitage in which they are expressed. Furthermore, no international agreement exists as to the test measuring dosage/effect and its standard. In the following review all published clinical studies were analyzed for patient groups. Important issues concerning controversy with regard to efficacy, bleeding risk, diagnosis, and testing are discussed. In general, it may be concluded that LMWH have an antithrombotic potential comparable to that of heparin, and that the benefit/risk ratio I.e., decreased bleeding potential when compared to standard heparin, as found in animal experiments, has not been clearly demonstrated in man.
Unbalanced translocation (1;7) in childhood myelodysplasia
Authors: Horsman D. E. , Massing B. G. , Chan K. W. , Kalousek D. K. 
Published: February 2007

AbstractWe have identified an unusual pediatric patient among twelve patients with myelodysplasia and an unbalanced translocation involving chromosomes 1 and 7: −7, + der(1)t(1;7)(p11; p11). This 16‐year‐old male patient developed myelodysplasia and evolving acute leukemia, which were preceded by a 7‐year history of marrow hypoplasia. The remaining patients were adults with clinical and hematologic findings similar to other reported cases with this chromosomal abnormality. The late appearance of this unbalanced clonal abnormality in this patient with marrow hypoplasia documents the importance of close cytogenetic follow‐up of all patients with suspected bone marrow injury.
Hemoglobin pasadena: Identification of the gene mutant by DNA analysis using synthetic DNA probes
Authors: Rahbar Samuel , Rosen Robert , Nozari Guity , Lee Terry D. , Asmerom Yayesh , Wallace R. Bruce 
Published: February 2007

AbstractHemoglobin Pasadena [β75(E19)Leu→Arg] was found in a boy who had an acute episode of anemia and rapid splenic enlargement. His father was the only other member of a large family with this hemoglobinopathy. We have used gene mapping techniques for direct identification of the β‐globin gene mutation. To correlate the DNA findings with the structural identification of this variant, we have also performed globin chain separation and analysis of the tryptic peptides using high performance liquid chromatography and secondary ion mass spectral analysis.
Daunorubicin in patients with relapsed and refractory acute nonlymphoblastic leukemia previously treated with anthracycline
Authors: Velu T. , Debusscher L. , Stryckmans P. 
Published: February 2007

AbstractWhile the efficacy of daunorubicin (DNR) in first induction of patients with acute nonlymphoblastic leukemia (ANLL) has been well documented, little data are available concerning the activity of DNR in patients with relapsed and refractory ANLL previously treated with anthracycline. We administered DNR (60 mg/m2/day for 3‐5 days) in 21 patients with relapsed (n=12) or refractory (n=9) ANLL previously treated with anthracycline. The general response rate was 48%, with a complete response (CR) rate of 38%. Five of 12 patients with relapsed ANLL and 3 of 9 patients with refractory ANLL achieved CR. Among these cases with CR in refractory ANLL, the evolution of one patient is particularly illustrative since he had received previously a large cumulative dose of anthracycline (540 mg/m2) and has been shown to be refractory to high‐dose cytosine arabinoside. This study suggests that previous administration of DNR or adriamycin does not Induce significant resistance to anthracycline: DNR appears to be almost as efficient in patients with relapsed and refractory ANLL previously treated with anthracycline as in first induction.
Congenital Pelger‐Huet anomaly in triplets
Authors: Ware Russell , Kurtzberg Joanne , Brazy Jane , Falletta John M. 
Published: February 2007

AbstractCongenital Pelger‐Huet anomaly is an autosomal dominant disorder characterized by incomplete nuclear segmentation of granulocytes. The morphology of these mature but hyposegmented granulocytes may be confused with that of immature granulocytes (bands), thereby suggesting bacterial infection. We report the first known case of triplets with this condition, and emphasize the need to identify accurately this normal variant.
Chlorpromazine‐induced anticardiolipin antibodies and lupus anticoagulant: Absence of thrombosis
Authors: Canoso Rosa T. , De Oliveira Ricardo M. 
Published: February 2007

AbstractAnticardiolipin antibodies were determined in 96 psychiatric patients treated chronically with chlorpromazine by an enzyme‐linked Immunosorbent assay using anti‐IgM and anti‐IgG (fab'2 fragment) as the second antibody. Fifty‐four of these patients had an IgM‐lupus anticoagulant, and the remaining 42 were followed as controls. Elevated IgM‐anticardiolipin antibodies (ACA) levels were detected in 31 patients with the lupus anticoagulant and in 5 controls (p<0.001). During a median followup of 5 years, single episodes of deep vein thrombosis or pulmonary embolism occurred in three patients; one had the lupus anticoagulant and the other two had low‐level ACA. Contrary to the reported experience in systemic lupus erythematosus and related autoimmune disorders, chlorpromazine‐induced lupus anticoagulant and anticardiolipin antibodies levels appear not to be associated with an increased incidence of thrombosis.
Type IIB von Willebrand's disease associated with a complex thrombocytopenic thrombocytopathy
Authors: López‐Fernández M. F. , López‐Berges C. , Martin‐Bernal J. A. , Sánchez R. , Villarón L. G. , Díez‐Jarilla J. , Batlle J. 
Published: February 2007

AbstractA familial bleeding disorder characterized by an association of Type IIB von Willebrand's disease (vWD) with a complex thrombocytopenic thrombocytopathy is described in two patients from the same generation. Findings typical of type IIB vWD included enhanced ristocetin‐induced binding of patient von Willebrand factor (vWF) to platelets of patients and normal individuals in association with the absence of larger multimers from plasma. Abnormalities in platelet function included deficient platelet aggregation to ADP, collagen, epinephrine, and arachidonic acid; and defective release of 14C‐serotonin, vWF, and platelet factor 4 (PF4) in response to thrombin, collagen, or ADP. Platelet factor 4 and platelet vWF were decreased when measured per mg of total platelet protein. In addition, the binding of normal vWF to patient platelets stimulated with thrombin was decreased. Platelet size was increased with a very heterogeneous distribution width. Electron microscopic evaluation showed giant platelets with dense and alpha bodies present. The platelet count was borderline or slightly decreased in the resting state and declined to frankly thrombocytopenic levels at the time of acute bleeding episodes; this state was associated with the presence of platelet aggregates in blood smears.
Modulation of responsiveness of chronic myelogenous leukemia granulocyte‐macrophage colony‐forming cells to growth regulation following in vivo treatment with recombinant γ‐interferon
Authors: Pelus Louis M. , Vadhan‐Raj Saroj 
Published: February 2007

AbstractA patient with Philadelphia chromosome (Ph) chronic myelogenous leukemia (CML), in chronic phase, was treated with recombinant γ‐interferon (rγ‐IFN) in a phase I clinical trial. Prior to treatment, analysis of in vitro agar culture parameters indicated hyporesponsiveness of granulocyte‐macrophage colony‐forming cells (CFU‐GM) to inhibition by prostaglandin E and acidic isoferritins and diminished expression of class II major histocompatibility complex (MHC) antigens (HLA‐DR). Treatment was associated with no change in bone marrow cellularity or in the percentage of Ph cells. However, in vitro cultures of bone marrow cells showed a return to normal levels of both expression of CFU‐GM class II antigen and of sensitivity to inhibition by prostaglandin E and acidic isoferritins which predicted and/or confirmed clinical response. Throughout the course of interferon therapy, white blood cell counts (WBC) and the percentage of bone marrow blast cells were maintained at normal levels. Onset of aggressive‐phase disease was associated with increased WBC, an increase in bone marrow blast cells, a secondary chromosomal abnormality, loss of CFU‐GM sensitivity to inhibition by putative negative growth regulators, and markedly diminished MHC class II antigen expression. Following a bone marrow transplant from a matched sibling, all hematologic parameters studied were found to be normal. These findings indicate that treatment with rγ‐IFN can modulate some of the abnormal growth characteristics of CFU‐GM observed in CML.
Transmission of human immunodeficiency virus to sexual partners of hemophiliacs
Authors: Smiley M. Lynn , White Gilbert C. , Becherer Paul , Macik Gail , Matthews Thomas J. , Weinhold Kent J. , McMillan Campbell , Bolognesi Dani 
Published: February 2007

AbstractTo examine the variables associated with heterosexual transmission of human immuno‐deficiency virus (HIV), we studied 32 couples in our hemophilia center who had steady sexual relationships for periods more than 1 year. Of the 32 sexual partners of the hemophiliacs, five (15.6%) were HIV seropositive. All five hemophiliacs with HIV transmission to their sexual partners had measurable immunologic deficiencies, as shown by their lower median T‐helper (CD‐4 +) lymphocyte count of 172 cells/mm3. The hemophiliacs without transmission had a slightly higher median CD‐4 + count of 297 cells/mm3 (P = 26). To determine if factors other than the degree of immunologic deficiency in the hemophiliac might contribute to HIV transmission, 18 of the 32 couples were studied more intensively by confidential, coded questionnaires. Regular condom use was reported by nine couples (50%). Two of nine women (22%) without condom usage acquired HIV. One of nine women (11%) using condoms was seropositive; she also reported eight needlestick injuries while assisting her spouse with clotting factor treatments. Intravenous drug abuse was reported in two of the five couples with HIV transmission. Thus, hemophiliacs are at risk for transmitting HIV parenterally as well as venereally. Despite various risk behaviors associated with HIV transmission, the prevalence of infection in our cohort of hemophiliacs' sexual partners is low and within the range (6.8–22%) reported by others. This study underscores the need for comprehensive education and counseling in what previously appeared to be a homogeneous clinic population at risk for transmitting HIV to others.
Thrombosis following desmopressin for uremic bleeding
Authors: Byrnes John J. , Larcada Alberto , Moake Joel L. 
Published: February 2007

AbstractAn elderly patient with evidence of atherosclerosis and uremic bleeding diathesis developed two foci of cerebral thrombosis immediately after an infusion of desmopressin (DDAVP). Because large molecular weight multimers of von Willebrand factor (vWF) have been demonstrated to cause platelet aggregation under conditions of elevated fluid shear stress as occurs in atherosclerotic vessels, we investigated his plasma vWF at the time of the event and compared it to baseline values obtained 2 weeks later. Unusually large vWF multimers induced by the DDAVP infusion were present and likely contributed to the thrombotic process. Consequently, we believe DDAVP should be given with greater caution to patients with atherosclerosis.
Cephalosporin‐induced immune cytopenia in the dog: Demonstration of erythrocyte‐, neutrophil‐, and platelet‐associated igg following treatment with cefazedone
Authors: Bloom John C. , Thiem Patricia A. , Sellers Teresa S. , Lewis Hugh B. , Deldar Ahmed 
Published: February 2007

AbstractCephalosporin treatment in man has been associated with a low incidence of hemolytic anemia, thrombocytopenia, and neutropenia; some cases have been shown to be immune‐mediated. This triad of blood dyscrasias was also demonstrated in our laboratory in a series of toxicity studies in dogs of two cephalosporin compounds, cefonicid and cefazedone; these studies provided evidence for drug‐associated immune hemolytic anemia, based on conventional laboratory tests. To further investigate possible immune mechanisms of the cephalosporin‐induced cytopenias, we measured erythrocyte‐associated, platelet‐associated (PAIgG), and serum antineutrophil IgG over the course of cephalosporin treatment, using highly sensitive 125I‐staphylococcal protein A (SPA) assays, as well as the direct antiglobulin test; we compared these findings with the hematologic changes. Intravenous treatment with high doses of cefazedone (540 mg/ kg/day, increased to a maximum of 840 mg/kg/day for 4 months or until hematologic effects were evident) resulted in a high incidence of anemia (7/14), thrombocytopenia (11/14), and neutropenia (7/14). Of the affected dogs examined, 6/7 with anemia, 9/9 with thrombocytopenia, and 7/7 with neutropenia showed increased levels of the respective cell‐associated antibody, compared with untreated controls. Unaffected dosed animals generally did not show these changes. In 3/3 dogs examined following remission of thrombocytopenia, PAIgG returned to levels comparable with controls; as one of these dogs suffered a relapse, increased PAIgG was again observed. Animals sacrificed during cytopenic episodes showed cytologic and histologic evidence of increased hemophagocytosis. We conclude that antibody‐mediated blood cell destruction contributes to all three cephalosporin‐induced cytopenias in the dog.
Isolated thrombocytopenia in patients infected with HIV: Treatment with intravenous gammaglobulin
Authors: Bussel James B. , Haimi Joseph S. 
Published: February 2007

AbstractIsolated thrombocytopenia occurs frequently in patients infected with HIV. Studies of mechanisms of thrombocytopenia and clinical response to therapy suggest that the thrombocytopenia is often antibody mediated (ITP). The best approach to treatment of these patients is uncertain in that the routine modalities (steroids, splenectomy, vinca alkaloids) that are used to increase the platelet count in patients with classic ITP are known to be immunosuppressive. We report here the results of intravenous gammaglobulin (IVGG) treatment of 22 patients with HIV‐related acute and chronic ITP who had severe thrombocytopenia and bleeding symptoms. Only one patient had an opportunistic infection at the time of treatment. Eight patients were homosexual, eight had hemophilia, three were i.v. drug abusers, two children had congenital acquisition of HIV, and one was the wife of an HIV + i.v. drug abuser. The average pretreatment platelet count was 22,000/μl (hemophiliacs were treated at higher platelet counts than were the other patients), and the mean peak platelet count measured on days 5 to 8 was 182,000/μl. Nineteen of 22 patients had peak platelet counts >50,000/μl following IVGG and 17/22 had peak counts > 100,000/μl. After the initial infusions, all but three refractory patients could maintain adequate platelet counts with IVGG alone infused no more often than once every 2 weeks. The outcomes for the 22 patients after multiple maintenance IVGG infusions were remission, 5; stable without therapy, 1; maintenance, 13; and refractory, 3. The eight hemophiliacs with ITP responded better than did the eight homosexual ITP patients; their mean peak platelet count was 227,000/μl versus 142,000/μl in the homosexuals. In summary, patients with HIV‐related ITP without opportunistic infections responded well to IVGG, with peak platelet counts comparable to those of ITP patients not infected with HIV. IVGG may be a useful therapy of ITP in HIV + patients, since it appears to be less immunosuppressive than are conventional therapies, and none of the 22 HIV + patients developed an opportunistic infection while receiving IVGG alone.
An antibody to platelet membrane antigen associated with thrombotic events in essential thrombocythemia
Authors: Rock G. , Smiley R. K. , Tittley P. , Hashemi S. , McCombie N. 
Published: February 2007

AbstractA 71‐year‐old man with essential thrombocythemia presented with acute onset of thrombosis in his fingers. The platelet count at presentation was 752 × 109/liter, and his PTT was within the normal range. Platelet aggregation was normal in response to ADP, decreased to epinephrine, and absent to collagen, with the patient receiving 1.2 g aspirin/day. Plasma vWF:Ag was 158 U/dl, VIII: C was 150 U/dl, and the vWF:Ag multirner pattern was normal. HLA antibodies were not detected in his serum, and the platelet‐associated IgG was normal at 3.9 fg/platelet. Incubation of normal, unactivated platelets with the patient's plasma did not result in agglutination of the platelets. However, a serum antibody reacting against a platelet antigen with an apparent molecular weight of 45,000 could be demonstrated by Western blotting. This antibody also reacted with endothelial cells. The reactive pattern was similar to that of commercially obtained actin; the serum antibody could be removed by preincubation with actin.Platelet‐plasmapheresis was carried out daily for 10 days, after which the serum still reacted weakly with the platelet antigen. By day 20, the band could still be identified on the electroblot, but 2 weeks later no band could be seen. Similar assay of sera from six other patients with myeloproliferative disorders and comparable thrombocytosis but without thrombotic manifestations did not show any such reactions. The data suggest a correlation between the presence of an antibody possibly directed against platelet and/or endothelial cell actin and the vascular events in this patient with essential thrombocythemia.
Fetal lamb coagulation system during normal birth
Authors: Andrew M. , O'Brodovich H. , Mitchell L. 
Published: February 2007

AbstractThe contribution of the birth process to the unique coagulation system in the young was Investigated using the fetal lamb model. Specific components of the coagulation system were measured prior to and immediately following delivery of the lamb. The results show that the birth process itself does not contribute to the physiologically low levels of coagulation factors present in the newborn.