bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of platelet alloimmunization with intravenous immunoglobulin in a child with aplastic anemia
Authors: Chen Shu‐Huey , Liang Der‐Cherng , Lin Marie 
Published: February 2007

AbstractA 9‐year‐old girl with severe refractory aplastic anemia had become refractory to platelet transfusion due to platelet alloimmunization. As a result, a huge right parieto‐temporo‐occipital cerebral hematoma and a subarachnoid hemorrhage subsequently occurred. Intravenous immunoglobulin (IVIG) (Green Cross, Taiwan) at a dose of 0.4 g/kg/day for 5 days, followed by another 3 doses during the following 5 days, and combined with large‐dose platelet transfusions effectively controlled the bleeding by elevating the platelet count to above 100 × 103/mm3. The patient soon recovered with only a mild sequela. Two months later, because of multiple caries and a periodontal abscess, dental extraction needed to be performed. IVIG (0.4 g/kg/day × 6 days) enabled surgery to proceed by elevating the platelet count to above 55 × 103/mm3, and no bleeding complications occurred during or after the procedure. The potential benefit of high‐dose IVIG in modulating platelet alloimmunization is a result of increasing the survival of transfused platelets. Thus, this therapy is recommended when patients with platelet alloimmunization have critical bleeding episodes or undergo surgical procedures. © 1995 Wiley‐Liss, Inc.
Adhesion of washed blood platelets in vitro is advanced, accelerated, and enlarged by oxidized low‐density lipoprotein
Authors: Zhao B. , Rickert C. H. , Filler T. J. , Liu B. , Verhallen P. F. J. , Dierichs R. 
Published: February 2007

AbstractIn order to study the influence of oxidized low‐density lipoprotein (Ox‐LDL) on platelet functional morphology at an early activation stage, washed human blood platelets were stimulated by 100 μg/ml Ox‐LDL at 37°C. The settling and spreading process of stimulated and unstimulated platelets on Formvar‐coated glass was observed for approximately 20 min by reflection contrast microscopy (RCM) and quantified by image analysis. Each group consisted of at least 250 platelets. The results show that incubation with Ox‐LDL causes platelet shape change and pseudopodia formation. The sedimentation of stimulated platelets precedes that of unstimulated platelets by approximately 3 min. The increase of the total adhesion area of all Ox‐LDL treated platelets is significantly accelerated in comparison to normal platelets (20.45 μm2/min vs. 15.45 μm2/min;P < 0.01). The mean total adhesion area of Ox‐LDL‐treated platelets was generally larger than that of untreated platelets (189.7 μm2 vs. 144.7 üm2; P < 0.01). The disappearance of intracellular granules after platelet activation, observed by RCM, is supported by transmission electron microscopy. Our results suggest that Ox‐LDL activates platelets and advances and accelerates their adhesion and thereby may contribute to pathological thrombosis and arteriosclerosis.
Idiopathic CD4+ T‐lymphocytopenia in HIV seronegative men with hemophilia and sex partners of HIV seropositive men
Authors: O'Brien Thomas R. , Diamondstone Laura , Fried Michael W. , Aledort Louis M. , Eichinger Sabine , Eyster M. Elaine , Hilgartner Margaret W. , White Gilbert , Di Bisceglie Adrian M. , Goedert James J. 
Published: February 2007

AbstractPersons with hemophilia or other HIV‐1 risk factors may be more likely to have idiopathic CD4+ T‐lymphocytopenia (ICL). We determined the frequency of ICL in prospectively followed cohorts of HIV‐1 seronegative hemophilic men and seronegative female sex partners of HIV‐1 infected hemophilic men, and examined factors potentially associated with ICL. Seven of 304 (2.3%) seronegative hemophilic men and one of 160 (0.6%) female partners met the ICL definition, but the condition resolved for two of the men and for the sole female partner. All five men with persistent ICL had lymphocytopenia (<1,200 total lymphocytes/ül) and <300 total CD4+ lymphocytes/ü only one had a low CD4+ percent‐ age. On the most recent measurement, 14.5% of the 304 seronegative hemophilic men had lymphoctopenia. Compared with matched hemophilic controls, men with persistent ICL more often had a history of liver disease (3/5 cases, 0/21 controls, P = 0.007) or splenomegaly (3/5 cases, 4/21 controls; P = 0.04), but not severe hemophilia, greater clotting factor concentrate exposure, high alanine aminotransferase levels, hepatitis B virus antigenemia, or detectable hepatitis C virus RNA in plasma. All five cases and 20/21 controls had antibodies to hepatitis C virus present in their serum. In this cohort of hemophilic men, ICL was related to lymphocytopenia associated with liver disease rather than selective loss of CD4+ lymphocytes.
Higher cytotoxic activity and increased levels of IL‐1β, IL‐6, and TNF‐α in patients undergoing cardiopulmonary bypass
Authors: Martí Francesc , Muñoz Joan , Peiró Matilde , Bertran Esther , Ferrán Carme , Octavio Carmen , García Juan , Rueda FÈLix 
Published: February 2007

AbstractExtracorporeal circulation (EC) by surgical bypass is often associated with a systemic inflammatory response. The purpose of this study was to assess the effect of EC on the serum levels of pro‐inflammatory mediators Interleukin (IL)‐1β, IL‐6, tumor necrosis factor (TNF)‐α, and soluble CD‐23. Peripheral blood lymphocyte subsets and natural killer cell (NK) cytotoxic activity were also analyzed before and after the bypass process. The results from eight patients who underwent cardiac surgery showed a significant increase in the levels of IL‐1β, IL‐6, and TNF‐α, a decrease in CD4+/CD8+ lymphocyte ratio, and an overstimulated NK cytotoxic activity. These changes on serum cytokine levels and cellular immunology parameters could play an important role in the development of adverse effects associated with EC. © 1995 Wiley‐Liss, Inc.
Factor V Leiden (FVQ 506) is common in a Brazilian population
Authors: Arruda Valder R. , Annichino‐Bizzacchi Joyce M. , Costa Fernando F. , Reitsma Pieter H. 
Published: February 2007

AbstractResistance to activated protein C (APC) is the most common risk factor in venous thrombosis. A missense mutation in the factor V gene (factor V Leiden) is the molecular basis for this phenotype. Factor V Leiden was previously found in about 20% of unselected patients with thrombosis but also occurred in 3‐5% of healthy Dutch controls. In the present study of Brazilian patients with venous thrombosis we also observed a frequency of 20% for this factor. In addition, in healthy Brazilian controls the frequency of the mutation was 2%. These data suggest that factor V Leiden has a similar distribution worldwide irrespective of the ethnic origin of the population. © 1995 Wiley‐Liss, Inc.
Fetal hemoglobin expression in compound heterozygotes for −117 (G→A) Aγ HPFH and β039 nonsense thalassemia
Authors: Pistidda Paola , Frogheri Laura , Oggiano Lina , Guiso Luciana , Manca Laura , Dore Fausto , Masala Bruno , Gilman John G. , Longinotti Maurizio 
Published: February 2007

AbstractThe‐117(G→A) Aγ hereditary persistence of fetal hemoglobin (Greek HPFH) and β039‐thal mutations are rather frequent in Sardinia so that their interaction is to be expected. Characterization of eight compound heterozygotes for these defects indicated that HPFH was linked to haplotype VII and β039‐thal to haplotype II. Haplotype II β039‐thal chromosome carries the AγT gene which is a useful marker of γ‐gene expression. Since the Hb F level in these compound heterozygotes was significantly higher than in 46 −117 HPFH carriers, the Aγt, and Gγ globin level was determined. AγT was underexpressed while Gγ was significantly increased, which suggests that in −117 Aγ HPFH/β039‐thal healthy subjects the increase in Hb F production is determined only by the −117 mutated Aγ gene and the adjacent Gγ gene. © 1995 Wiley‐Liss, Inc.
Low proteins C and S and activation of fibrinolysis in treated essential thrombocythemia
Authors: Wieczorek Iwona , Macgregor Ian R. , Ludlam Christopher A. 
Published: February 2007

AbstractThe aim of this study was to investigate whether abnormalities in the fibrinolytic system and in the naturally occurring anticoagulant proteins could contribute to the thrombotic risk in essential thrombocythemia. Euglobulin lysis time, fibrin plate lysis area, tissue plasminogen activator antigen, and activity and plasminogen activator inhibitor antigen were measured before and after venous occlusion in a group of 16 patients with essential thrombocythemia and in 16 healthy age and sex matched controls. In addition, resting levels of antithrombin III, D‐dimer, prothrombin fragment 1 + 2, and protein C and S were assessed. The results were related to the presence or absence of a thrombotic history.The results demonstrated that the patients had a significantly elevated fibrin plate lysis area and significantly decreased plasminogen activator antigen, both at baseline and after venous occlusion. They also had significantly decreased levels of plasma protein C and total protein S. There was a modest, non‐significant elevation in the plasma concentration of D‐Dimer and F 1 + 2. Those patients with a history of thrombosis had significantly lower protein C levels compared with individuals without a thrombotic history.We conclude that patients with essential thrombocythemia have evidence of activated fibrinolysis in the resting state and after stimulation. This, and the decreased levels of protein C and total protein S, may be secondary to chronic clinically occult thrombosis occurring in myeloproliferative disorders. © 1995 Wiley‐Liss, Inc.
Molecular characterization of a new family with α‐Thalassemia‐1 (——MA mutation)
Authors: Villegas Ana , Sanchez Jesus , Carreno Dolores L. , Ropero Paloma , Gonzalez Fernando A. , Espinos Domingo , Penalver Maria A. , Lozano Matilde 
Published: February 2007

AbstractA Spanish family with α‐thalassemia‐1 (α‐Thal‐1), deletion (—MA), is described. In addition to the loss of 22 kb of DNA with a deletion of the α1, α2, α1, α2, and Ψζ1 genes, a triplication of the ζ gene cluster in “cis” is produced. The structure of this triplication is formed by the Ψα1, gene, the interzeta region, and, possibly, the insertion of the Ψα2 fragment. © 1995 Wiley‐Liss, Inc.
Discordant morphologic features in bone marrow involvement by malignant lymphomas: Use of gene rearrangement patterns for diagnosis
Authors: Crisan Domnita , Mattson Joan C. 
Published: February 2007

AbstractDiscordant morphology between lymph node or extra‐nodal site and bone marrow (BM) involvement by non‐Hodgkin's malignant lymphoma (NHL) is a common occurrence, causing diagnostic difficulties. Additional diagnostic problems are posed by lymphoid aggregates commonly found in the BM of elderly patients, the age group with the highest incidence of lymphoma. Morphologic features are used to distinguish between benign and malignant lesions but no feature is diagnostic and exceptions are numerous. Immu‐nophenotyping is helpful for detecting B cell monoclonality, but it cannot detect T cell monoclonality. Unique B and T cell gene rearrangement patterns, the molecular “signature” of the lymphoma, can be used to detect monoclonal lymphoid populations. Finding the same rearrangement pattern in the BM as in the primary mass is proof of BM involvement by the same clone of malignant cells. We used B/T and Bcl‐2 gene rearrangements to help diagnose cases with discordant morphology between primary site and BM. One hundred and seventy‐five specimens, obtained from patients undergoing staging or restaging for NHL, were analyzed for B/T cell and Bcl‐2 gene rearrangements by multiple restriction endonuclease digestion and Southern hybridization with 32P labeled JH, JK, CTα, and Bcl‐2 probes. Forty‐two specimens (24%) from 24 patients showed discordant morphology: of 13 specimens with atypical lymphoid aggregates, only one had B cell gene rearrangement; of 15 specimens with morphologically benign lymphoid aggregates, one demonstrated B cell gene rearrangement; and of 14 specimens positive for NHL with different morphology than the lymph node, 13 were positive for B cell gene rearrangements. Molecular analysis can aid in the diagnosis of NHL, can establish a “baseline” for detection of recurrence, and is useful in monitoring therapy. These data suggest that it is also a tool for the pathologist in cases of discordant morphology between the primary tumor and BM, and should be strongly considered for each site. © 1995 Wiley‐Liss, Inc.
Immune mediated agranulocytosis and anemia associated with thymoma
Authors: Postiglione Kathy , Ferris Richard , Jaffe Jeffrry P. , Stroncek David 
Published: February 2007

AbstractThymoma has been associated with a variety of autoimmune disorders. We report a case of agranulocytosis and anemia in a 68‐year‐old woman with a spindle cell thymoma. She was unresponsive to treatment with antibiotics, granulocyte‐colony stimulating factor (G‐CSF), prednisone, and high‐dose intravenous immunoglobulin. Serial bone marrow examinations on this therapy showed progression from a cellular marrow with mild my‐eloid and erythroid hyperplasia and lymphocytosis, to granulocyte aplasia and severe erythroid hypoplasia. Her serum contained granulocyte‐specific antibodies and inhibited the growth in culture of her own marrow cells and marrow cells from a normal donor. An IgG fraction from her serum also inhibited the growth of marrow cells.Although the patient's spindle cell thymoma was surgically removed, she remained neutropenic. She was treated with six plasma exchanges followed by 1,000 milligrams of intravenous cyclophosphamide 2 days after the final plasma exchange and daily G‐CSF. Three weeks later her peripheral blood showed marked leukocytosis with pronounced neutrophilia and a left shift. Although her agranulocytosis resolved, she died of fungal sepsis.This case demonstrates that aggressive plasma exchange and immunosuppressive therapy may benefit patients with agranulocytosis associated with thymoma. © 1995 Wiley‐Liss, Inc.
Expression of the intestinal T‐lymphocyte‐associated‐molecule recognized by the HML‐1 antibody on mononuclear cells from HTLV‐I‐infected subjects
Authors: Otsuka Maki , Hanada Shuichi , Utsunomiya Atae , Ishitsuka Kenji , Uozumi Kimiharu , Arima Terukatsu 
Published: February 2007

AbstractWe investigated the expression of a monoclonal antibody (HML‐1) defined antigen that appears on human intestinal T‐lymphocytes in HTLV‐I‐related disease. We studied 25 ATL, and 24 healthy HTLV‐I carriers. Patients with acute ATL showed a variety of the expression of the HML‐1 antigen (range 0.4–74.8%). HML‐1 expression on mononuclear cells (MNCs) in blood from patients with chronic ATL ranged from 1.7–43.6% (mean 13.5%). This level of expression was less than that of patients with acute ATL, but not significantly. In patients with smoldering ATL, the degree of patients with acute ATL, but not significantly. In patients with smoldering ATL, the degree of expression ranged from 1.6–13.3% (mean 8.0%). In contrast to patients wtih acute ATL, MNCs from patients with acute myelogenous leukemia (AML), acute lymphocytic leukemia (ALL), and B‐cell type chronic lymphocytic leukemia (B‐CLL) did not express the HML‐1 antigen, except for the 2 patients with ALL. Healthy HTLV‐I carriers and healthy controls also were negative for HML‐1 reactivity. In acute ATL, patients with gastrointestinal tract infiltration tended to have high expression of the HML‐1 epitope. After stimulation with phytohemagglutinin (PHA), healthy HTLV‐I carriers showed significantly increased expression of the HML‐1 epitope (P< 0.05).Recently, the β7 integrin family has been found to play a specific role in mucosal localization or adhesion, and HML‐1 protein was found to match the deduced β7 N‐terminal sequence. We propose that the cellular gene responsible for HML‐1 epitope expression may, like IL‐2, IL‐2R, etc., be transactivated by infection with HTLV‐I, and that HML‐1 antigen gene expression by HTLV‐I infection may lead to infiltration of ATL cells with highly expressed HML‐1 epitope into the gut mucosa.
Flow cytometric evaluation of platelet activation in blood collected into EDTA vs. Diatube‐H, a sodium citrate solution supplemented with theophylline, adenosine, and dipyridamole
Authors: Kühne Thomas , Hornstein Adriana , Semple John , Chang Wilda , Blanchette Victor , Freedman John 
Published: February 2007

AbstractWith platelet activation, there is modulation of platelet surface molecule expression. In flow cytometric analyses of in vivo platelet activation, results are often confounded by activation induced in vitro by the preparative procedures. It is particularly important therefore to prevent or retard platelet activation as soon as possible after withdrawal of the blood sample. Taking blood into paraformaldehyde, or fixing the cells with paraformaldehyde as soon as possible after withdrawal, has been employed to prevent platelet activation in vitro, but paraformaldehyde‐fixed platelets cannot be further used in functional studies. We investigated the efficacy of Diatube‐H, a commercially available combination of platelet antagonists (theophylline, adenosine, and dipyridamole), in preventing or retarding platelet activation in vitro, along with its effects on modulation of platelet membrane glycoproteins (GP) and adhesion molecules. In contrast to blood taken into EDTA, blood taken into Diatube‐H vacutainer tubes could be stored at room temperature for up to 4 hr prior to paraformaldehyde fixation without significant in vitro platelet activation, as measured by CD62P, CD63 and modulation of GPIb and GPIIbIIIa surface expression. Hence, paraformaldehyde fixation could be deferred for several hours, permitting transport of samples from distant sites. Studies of thrombin‐induced platelet activation indicated that platelets taken into Diatube‐H remained functional i.e. were able to be activated. Expression of the CD29, CD49b and CD31 adhesion molecules on the platelet surface was unaffected by storage in Diatube‐H. The results suggest that Diatube‐H may be a useful reagent for flow cytometric studies of platelets when the samples cannot be processed immediately.
Sickle cell anemia, right atrial thrombosis, and the antiphospholipid antibody
Authors: Yeghen T. , Benjamin S. , Boyd O. , Pumphrey C. , Bevan D. H. 
Published: February 2007

AbstractWe report a patient with sickle cell anemia (homozygous Hb SS) and typical features of sickle chest syndrome but with no response to exchange transfusion. A right atrial thrombus was found, and antiphospholipid antibodies were detected in his blood. He responded to thrombolytic therapy. The relationship between right atrial thrombus and massive pulmonary embolus, and the implications of an additional thrombophilic state in sickle cell disease are discussed.
Autologous bone marrrow transplantation versus chemotherapy in relapsed/refractory non‐hodgkin's lymphoma: Estimates of long‐term survival from the recent literatur
Authors: Meehan Kenneth R. , Pritchard Robert S. , Leichter Joshua W. , Littenberg Benjamin , Gilbert Welch H. 
Published: February 2007

AbstractLong‐term survival following chemotherapy or autologous bone marrow transplantation in adults with relapsed/refractory non‐Hodgkin's lymphoma was evaluated. English language articles published from January 1, 1988 to September 1, 1993 were obtained from a broad‐based MEDLINE search retrieving 3,854 citations regarding therapy for lymphomas.Citations were evaluated using both computer‐based evaluation and manual review. Articles were included if they addressed the disease of interest (non‐Hodgkin's lymphoma, Working Formulation D‐H), the population of interest (adults with either relapsed or refractory disease), and the therapies of interest (chemotherapy or autologous bone marrow transplantation). Articles were excluded if they did not provide convincing information on long‐term survival (as evidenced by either survival analysis or individual patient data) or if they reported a small number of patients (N < 15).No randomized trials of the two therapies were found. Nine case series were found reporting on 444 eligible patients receiving chemotherapy; eight were found reporting on 256 patients undergoing autologous marrow transplantation. After weighting by sample size, the mean 3‐year survival rate was 25% (95% Cl, 20–30%) following chemotherapy and 40% (95% Cl, 33–47%) following marrow transplantation. The reporting of potentially relevant prognostic factors was inconsistent among articles.Despite our comprehensive synthesis and evaluation of currently available data, the survival advantage of marrow transplantation in relapsed/refractory non‐Hodgkin's lymphoma that we report must be viewed as tentative, given the limitations of the case series data. In addition, establishing the comparability of patients treated with these therapies is made more difficult by the inconsistent reporting of potentially relevant prognostic factors. The results of an international randomized trial of these two therapies is forthcoming and may address some of these shortcomings.
Recognition of unusual presentation of natural killer cell leukemia
Authors: Gardiner C. M. , Reen D. J. , O'Meara A. 
Published: February 2007

AbstractExpansion of the natural killer (NK) subset of lymphocytes represents a rare leukemia phenotype with variations in clinical presentation, morphology, surface phenotype, and effector function. This paper reports on a 5‐year‐old male patient who had an unusual presentation of an NK cell leukemia that was initially diagnosed as neuroblastoma. A bone marrow (BM) aspirate showed clumps of undifferentiated cells with the following phenotype: CD56bright+, CD33dim+, CD45‐, CD2‐, CD19‐, CD16‐, and CD57‐. Cytochemistry was noncontributory. The patient, having failed to respond to conventional neuroblastoma chemotherapy, was subsequently diagnosed as having NK cell leukemia based on functional in vitro assays. The patient responded to acute lymphoblastic leukemia (ALL) chemotherapy but relapsed 4 weeks into treatment and eventually died 25 weeks after initial presentation. The cell surface phenotype observed is consistent with a rare NK cell subset, the biology of which has not been well defined. Freshly isolated BM cells killed K562 cells in a conventional 51Cr‐release assay. Both interleukin‐2 (IL‐2) and interferon‐α (IFN‐α) induced LAK activity against the Daudi cell line. IL‐2 induced proliferation of the leukemic cells. TNF‐α, IFN‐γ, IL‐6, IL‐1ra, and TGF‐β levels were assessed and found to be concentrated in BM, in contrast to plasma samples. TNF‐α was present at a high concentration in BM (150.9 pg/ml), probably a reflection of the associated disease pathology of severe bone pain and pyrexia. In summary, this paper details clinical and laboratory investigations of a leukemia of a rare NK cell subset.