bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hb adana or α259(E8)Gly→Aspβ2, A severely unstable α1‐globin variant, observed in combination with the ‐(α)20.5 KB α‐thal‐1 deletion in two Turkish patients
Authors: Çürük M. A. , Dimovski A. J. , Baysal E. , Gu L‐H. , Kutlar F. , Molchanova T. P. , Webber B. B. , Altay Ç. , Gürgey A. , Huisman T. H. J. 
Published: February 2007

AbstractWe have identified a severely unstable hemoglobin variant through sequencing of amplified DNA involving the α1‐globin gene; the mutation is located in codon 59 (CCG CAG) andresults in a Gly—Asp replacement. This amino acid substitution concerns a glycine residue at an internal position in the E helix, which is in close contact with a glycine residue of the B helix; introduction of the larger and charged aspartic acid residue greatly affects the stability of the molecule. This variant was present in association with a common α‐thalassemia‐1 deletion [‐(α)20.5 kb] in two adults and caused a severe type of Hb H disease with anemia, low levels of Hb A2, increased → chain, and Hb Bart's. In vitro chain synthesis in reticulocytes showed a high specific activity of the variant α chain. Only a minute quantity of Hb H was present but instead about 10% of Hb Bart's was observed. The increased synthesis of γ chains was likely due to specific characteristics of a chromosome with haplotype #3, which was present in both patients. The same family was studied 18 years ago [1]; the improved methodology presently available has led to a corrected diagnosis for these patients. © 1993 Wiley‐Liss, Inc.
Hemostatic molecular markers in nephrotic syndrome
Authors: Chen Tsai‐Yun , Huang Chieng‐Chung , Tsao Chao‐Jung 
Published: February 2007

AbstractQuantitative changes of hemostatic molecular markers were studied in patients with nephrotic syndrome. The plasma levels of fibrinopeptide A (FPA), thrombin‐antithrombin III complex (TAT), products of thrombin activation, and fragment F1 + 2 (F1 + 2), a product of prothrombin activation, were measured by enzyme immunoassay in 21 patients with nephrotic syndrome and in 16 normal controls. The mean value of FPA was 17.5 ± 7.5 ng/ml (mean ± SD) in nephrotic patients and 4.5 ± 0.3 ng/ml in normal controls (P < 0.02); F1 + 2 concentration was 1.4 ± 0.3 ng/ml in nephrotic patients and 0.5 ± 0.1 ng/ml in normal controls (P < 0.001); TAT level was 1.0 ± 0.3 μg/l in nephrotic patients and 0.2 ± 0.1 μg/l in normal controls (P < 0.05). These data indicated intravascular hemostasis activation. Based on these results, we propose that low antithrombin III level in nephrotic patients may be due to both urinary loss and intravascular consumption. © 1993 Wiley‐Liss, Inc.
Quantitative defect of glycoprotein ib in severe cirrhotic patients
Authors: Sánchez‐Roig María J. , Rivera José , Moraleda José M. , García Vicente Vicente 
Published: February 2007

AbstractA decrease of platelet agglutination induced by ristocetin has been described in Cirrhotic patients. In order to investigate the relationship of such phenomenon with a putative defect on piatelet membrane glycoprotein Ib, we have studied the quantitative and functional status of Gp Ib in eleven severe alcoholic cirrhotic patients, and the ability of their formalin‐fixed platelets to agglutinate in the presence of normal plasma plus ristocetin. Interestingly, we found a significant decrease of immunoreactive GP Ib molecules (9,978 ± 1,534 vs. 17,064 ± 404 molecules per platelet) and ristocetin‐dependent binding of vWF (9,113 ± 1,338 vs. 13,992 ± 1,968 molecules per platelet) (P < 0.01) in comparison to the levels found in a control group of healthy subjects. Immunoblotting analysis of platelet lysates confirmed the reduction of GP Ib level in cirrhotic patients, but showed no modification on the precipitation pattern of this glycoprotein. The ristocetin‐induced platelet agglutination (light transmission %) was also significantly lower in patients with Cirrhosis (48 ± 7.6 vs. 92 ± 3.6, P < 0.01), and correlated with the binding of normal vWF (r = 0.863, P = 0.0013). Patients with bleeding times longer than 7 min and/or clinical history of bleeding episodes showed the lowest values of platelet agglutination and GP Ib level. In conclusion, our present results indicate that llver cirrhosis is associated with a relevant decrease of functional GP Ib molecules on the platelet surface. This reduction might be related to the prolongation of bleeding time and to the bleeding diathesis observed in cirrhotic patients. © 1994 Wiley‐Liss, Inc.
Dyshematopoiesis in combined immune deficiency with congenital neutropenia
Authors: Gasparetto Cristina , Smith Clay , Firpo Meri , Dennig Dieter , Small Trudy , Gillio Alfred P. , Lichtenberg Robert , O'Reilly Richard J. , Moore Malcolm A. S. 
Published: February 2007

AbstractThis report describes a patient with combined immune deficiency associated with congenital neutropenla (CID/CN) and reports a partial characterization of his hematopoletic abnormalities. The CID/CN syndrome described is characterized by neutropenla and by deficiencies in B‐lymphoid and T‐lymphoid cell number and function. Red cell and platelet counts were normal. In vitro assays indicate that the myeioid lineage was developmentally arrested at the level of the committed monocyte/granulocyte progenitor (CFU‐GM), while precursors to the CFU‐GM progenitor were normal. In vitro studies showed that the defect in myeiold development was not corrected with G‐CSF or GM‐CSF. However, combinations of cytokines present in conditioned media from the T‐cell lines MO or C5MJ, or defined multiple cytokine combinations containing IL‐1, IL‐3, GM‐CSF, kit ligand, IL‐6, and IL‐9, restored myelopoiesis in‐vitro. In contrast, CBMJ‐conditioned media did not correct deficiencies in immune function in the patient's lymphocytes and accessory cells. No abnormalities in the production of G‐CSF, GM‐CSF, M‐CSF, or IL‐1 from the patient could be identified to account for the defects in myelopoiesis orimmune function. © 1994 Wiley‐Liss, Inc.
A novel mediterranean “δβ‐thalassemia” determinant containing the δ+27 and β°39 point mutations in cis
Authors: Oggiano Lina , Guiso Luciana , Frogheri Laura , Loudianos Georgios , Pistidda Paola , Rimini Elena , Pirastu Mario , Cao Antonio , Longinotti Maurizio 
Published: February 2007

AbstractThe term δβ‐thalassemia with normal HbF has been recently proposed to define heterogenous δ and β globin gene molecular defects involving the same chromosome in cis. Here, we describe a Sardinian family in which three members showing microcytosis, border‐line HbA2 levels and normal HbF proved to be heterozygotes for δ +27 and β°39 point mutations in cis by allele specific oligonucleotyde hybridization as well as by ECO 0 109 I endonuclease digestion and electrophoresis. As some of these β‐thalassemia carriers shows normal HbA2 levels, knowledge of the molecular basis of this novel δβ‐thalassemia silent phenotype would be useful in thalassemia screening and genetic counselling. © 1994 Wiley‐Liss, Inc.
A new deletional α‐thalassemia detected in yemenites with hemoglobin H disease
Authors: Shalmon Lea , Kirschmann Chava , Zaizov Rina 
Published: February 2007

AbstractA new large deletion from the human α‐giobin gene cluster is characterized. It involves at least 39 kb and includes the two α‐giobin genes, the ϑ 1‐gene, all the pseudogenes, and the two hypervariable regions (HVRs), interzeta‐HVR and α‐globin 3′HVR. The conserved ζ‐globin gene has been identified in various restriction fragments of abnormal size. The new deletion was found in four unrelated Israeli patients with Hb H disease, all orlginating in Yemen, and has been designated ‐ ‐YEM. It is the only two‐gene deletion identified in this ethnic group. © 1994 Wiley‐Liss, Inc.
Elevated plasma tissue factor antigen level in patients with disseminated intravascular coagulation
Authors: Wada Hideo , Nakase Tsutomu , Nakaya Ryusuke , Minamikawa Kouzou , Wakita Yoshihiro , Kaneko Toshihiro , Ohiwa Michiaki , Deguchi Katsumi , Shirakawa Shigeru 
Published: February 2007

AbstractThe plasma tissue factor (TF) antigen level was measured in patients with disseminated intravascular coagulation (DIC). The plasma TF antigen was detected In normal volunteers, and it was significantly higher in DIC patients than in non‐DiC patients. However, in some patients with DiC, the plasma TF antigen level was within the normal range. The plasma TF antigen level in patients with DIC significantly decreased after therapy, but it was not correlated with organ failure or outcome. The plasma TF antigen level in patients with DIC was not correlated with other hemostatic markers. The plasma TF antigen level tended to be higher in DIC patients with nonlymphoid leukemia than in those with lymphoid tumor. TF might be implicated in the occurrence and progression of DIC. © 1994 Wiley‐Liss, Inc.
Spontaneous factor VIII inhibitor occurring in association with chronic graft‐versus‐host disease
Authors: Seidler Christopher W. , Mills Letha E. , Flowers Mary E. D. , Sullivan Keith M. 
Published: February 2007

AbstractThe development of spontaneously acquired Factor VIII inhibitors is rare and may lead to serious hemorrhagic sequeiae. We report here the case of a patient who acquired a Factor VIII inhibitor two years after an allogeneic bone marrow transplant for CML. This occurred in association with a flare of chronic graft versus host dlsease (GVDH). He responded to corticosteroid therapy. A review of autoimmune phenomena associated with chronic GVDH and the treatment of Factor VIII Inhibitors is discussed. © 1994 Wiley‐Liss, Inc.
Thrombocytopenia following acute acetaminophen
Authors: Fischereder Michael , Jaffe Jeffrry P. 
Published: February 2007

AbstractAn unusual case of severe thrombocytopenia following acetaminophen overdose is pre sented along with a retrospective review of 174 patients with hospital admissions for acute acetaminophen toxicity. Thrombocytopenla occurred in 3.4% of these patients and strongly correlated with the degree of hepatotoxicity (as measured by peak AST values) but did not correlate with serum acetaminophen levels. The mechanism appears to be a transient direct toxic effect on platelets or megakaryocytes, and the finding of thrombocytopenia early in the course of acetaminophen overdose may identify a subset of patients at risk for significant hepatotoxicity. © 1994 Wiley‐Liss, Inc.
IgE plasma cell leukemia successfully treated with combination VAD (vincristine, doxorubicin, dexamethasone) and MP (melphalan, prednisolone) followed by interferon‐α
Authors: Yamagata Noboru , Shimazaki Chihiro , Goto Hideo , Hirata Toshiyuki , Ashihara Eishi , Oku Naritoshi , Inaba Tohru , Fujita Naohisa , Nakagawa Masao 
Published: February 2007

AbstractA 69‐year‐old woman with IgE/K plasma cell leukemia (PCL) was treated with a sequential combination of VAD (vincristine, doxorubicin, dexamethasone) and MP (melphalan, prednisolone) followed by interferon‐α (IFNα). A complete remission was achieved for 14 months. Maintenance therapy with IFNα has continued for an additional 10 months. IgE PCL Is extremely rare. The biological characteristics of the myeloma cells, including surface marker, adhesion molecule, karyotype, DNA analysis, and the response to various cytokines, are presented. © 1994 Wiley‐Liss, Inc.
Autologous peripheral stem cell transplantation of the blastic phase of chronic myeloid leukemia following sequential high‐dose cytosine arabinoside and melphalan
Authors: Matulonis Ursula A. , Griffin James D. , Canellos George P. 
Published: February 2007

AbstractBlast‐phase chronic myelogenous leukemia (CML) is the terminal phase in CML and is uniformly fatal. We treated 12 patients with blast‐phase CML with a program of high‐dose cytarabine 3.0 g/m2 and melphalan 140 mg/m2. followed by reinfusion of stem cells obtained from peripheral blood during the chronic phase.Seven patients achieved either a partial or complete hematologic remission, while five patients showed no response to therapy. One petlent returned to chronic phase features with loss of a chromosomal abnormality acquired at blast phase, restoration of he matopolesis, and a decrease In the amount of bone marrow blasts to less than 10%. SIX patients cleared their peripheral blasts and showed recovery of their myelold and platelet lineages, but all six required treatment for acceleration within 3 months. Of the five nonresponding patients, three died with aplastlc bone marrow, one patient never cleared peripheral blasts after chemotherapy, and one patient had evidence of peripheral blasts 3 weeks after the autologous stem cell reinfuslon. None of the patients returned to a normal karyotype. The ablative regimen was effective in eradicating bone marrow blasts to <10% in 8 of 10 patients in whom interpretable bone marrow samples were performed following chemotherapy. Overall, the median survival for all patients from the time of stem cell reinfusion was 5.5 months.We conclude that autotransplants with peripheral blood can successfully be used to support hematopoiesis during high‐dose therapy for CML blast crisis, however, has no role by itself in the curative therapy of blast crisis CML. A small number of patients can be restored to chronic phase features, and this may provide an opportunity to administer subsequent alternative treatments designed to eradicate the malignant stem cell population. Autotransplants with stem cells may also be used as therapy for patients without a histocompatible marrow donor. However, the autotransplant may be more effective when used during the chronic phase of CML, with the use of hematopoletlc growth factors, and with reinfusion of stem cells depleted of the malignant Philadelphia chromosomepositlve clone. © 1994 Wiley‐Liss, Inc.
Molecular basis and hematological characterization of Hb H disease in Southeast Asia
Authors: Liu Ta‐Chih , Chiou Shyh‐Shin , Lin Sheng‐Fung , Chen Tyen‐Po , Tseng Wen‐Ping , Chen Pao‐Huei , Chang Jan‐Gowth 
Published: February 2007

AbstractWe molecularly characterized sixty‐seven cases of Hb H disease by the polymerase chain reaction. The strategy depends on amplifying the α‐thalassemia‐1 (α‐thal‐1) gene by prlmers flanking the breakpoint and sequence differences of the 3′ end of the α‐globin gene and the nonhomologous elements I, II, and III among different types of α‐thala‐2. In the 67 cases studied, all involved α‐thal‐1 of the Southeast Asia type (SEA) In combination with deletional or nondeletional α‐thal‐2. Thirty‐two cases were of the deletion form and 35 cases were of the nondeletion form. In 32 cases of the deletion form, 29 cases were rightward deletion (‐α3.7), and three cases were leftward deletion (‐α4.2). We found that all of the nondeletion forms were α‐thal‐1 of SEA type with Hb CS. After the subtyping of Hb H with ‐α3.7, 26 out of 29 were type I deletion and 3 out of 29 were type II deletion. Comparlsons of clinical data of deletion forms and the nondeletion form showed that there were earlier occurrence of anemic symptoms and a larger erythrocyte volume in the nondeletion form group (P < 0.005). © 1994 Wiley‐Liss, Inc.
Sequential peripheral blood progenitor cell transplantation after mobilization with salvage chemotherapy and G‐CSF in patients with resistant lymphoma
Authors: Sica Simona , Mario Antonella Di , Salutari Prassede , Etuk Benedict , Jovino Michela S. , Pierelli Luca , Marra Roberto , Teofill Luciana , Menichella Giacomo , D'Onofrio Giuseppe , Leone Giuseppe 
Published: February 2007

AbstractWe enrolled 18 patients affected by refractory or relapsed lymphoma (HD, NHL) in a two‐step protocol that included salvage chemotherapy with mitoxantrone, carboplatinum, methylprednisolone, and cytosine arabinoside (MICMA) plus G‐CSF (5 μg/kg/day), peripheral blood progenitor cell (PBPC) collection, and subsequent transplantation after BUCY2 regimen. After MiCMA chemotherapy, four patients (22%) achieved complete response, eight patients (44%) obtained a partial response, and six showed progression of disease (PD). Fourteen out of 18 patients (78%) were considered eligible for PBPC transplantation. Three patients with complete response refused PBPCT; they are currently in continuous complete remission (CCR) at 15, 13, and 15 months, respectively. One patient has been recently transplanted but is too early to be evaluated. Ten patients so far completed the study, eight of whom are currently alive in CR, with a median follow‐up of 7.5 months (range 2–13). Hematologic reconstitution was very rapid with a median time to achieve WBC > 1 × 109/L, PMN > 0.5 × 109/L, platelets > 50 × 109 /L and > 100 × 109/L of 13 (range 9–15), 12(range 9–14), 10(range 0–22), and 14 (range 5–49) days, respectively. Our protocol is highly effective as a salvage treatment, while permitting PBPC collection after G‐CSF administration. Hemopoietic reconstitution after transplantation of PBPCs collected with this procedure is complete, rapid, and sustained. © 1994 Wiley‐Liss, Inc.
Immune and serologic profiles of HIV‐infected and noninfected hemophilic children and adolescents
Authors: Jason Janine , Evatt Bruce , Murphy John , Sleeper Lynn A. , Donfield Sharyne M. , Warrier Indira , Arkin Steven , Gomperts Edward D. 
Published: February 2007

AbstractObjectives: To assess relationships among the effects of HIV on hemophilic children and adolescents' immunologic parameters and vaccine‐related serology.Methods: We analyzed data from extensive baseline immunologic evaluations of 207 HIV antibody‐positive (HIV+) and 126 HIV antibody‐negative (HIV−) hemophilic children and adolescents.Results: HIV+ and HIV‐ participants differed significantly in T‐lymphocyte subpopulation numbers, immunoglobulin levels, and seroprevalence rates for diphtheria toxoid, measles, and mumps antigens. IgG levels, IgM levels, and serologic titers to vaccine antigens showed little correlation with T‐cell parameters. Proportionately more HIV+ participants were nonreactive to each and all of a panel of 7 skin test antigens (71 % vs 28% anerglc, RR 2.6). The odds of anergy increased 1.6 times for every decline of 200 CD4 ± cells/μTl.Conclusions: HIV had significant, largely independent T‐ and B‐lymphocyte effects on this pediatric cohort. © 1994 Wiley‐Liss, Inc.
Inhibition of leukemic cell proliferation by factor(s) released from irradiated lymphocytes of B‐chronic lymphocytic leukemia patients
Authors: Bessler Hanna , Notti Ida , Cohen Amos M. , Klein Baruch , Djaldetti Meir 
Published: February 2007

AbstractAn attempt was made to clarify the mechanism by which splenic irradiation in patients with B‐chronic lymphocytic leukemia (B‐CLL) can induce a reduction in lymph node size. For this purpose peripheral blood lymphocytes from B‐CLL patients were exposed to cobalt irradiation and were cultured for 1–8 days. The effect of the supernatants on the proliferation capacity of normal and malignant human cells was examined. A suppression of phytohemagglutinin (PHA)‐induced proliferation of autologous and heterologous B‐CLL lymphocytes was observed, whereas there was no effect on the proliferation of lymphocytes obtained from healthy volunteers. in addition, supernatants of irradiated B‐CLL lymphocytes inhibited thymidine incorporation into blasts derived from patients with acute leukemia and the lymphoblastoid cell line Daudi, but they did not exert any effect on normal cells obtained from human embryonic liver. These results suggest the secretion of some factor(s) by irradiated B‐CLL lymphocytes, which may inhibit the proliferation of malignant cells but has no effect on normal cells. © 1994 Wiley‐Liss, Inc.
Allogeneic bone marrow transplantation for leukemia following piperazinedione and fractionated total body irradiation
Authors: Dimopoulos Meletios A. , Yau Jonathan C. , Huan Susan D. , Jagannath Sundar , Spitzer Gary , Spinolo Jorge A. , Zagars Gunar K. , Lemaistre Charles F. , Dicke Karel A. , Zander Axel R. 
Published: February 2007

AbstractBetween 1980 and 1988, 126 patients with leukemia were treated with piperazinedione and fractionated total body irradiation (TBI) followed by allogeneic bone marrow transplantation from HLA matched siblings. Sixty‐one patients had acute myelogenous leukemia, 46 acute lymphoblastic leukemia, and 19 chronic myelogenous leukemia. Patients with acute leukemia in first complete remission were transplanted only if perceived to have a low probability of remaining in remission with conventional therapy. The toxicity from the preparative regimen was similar to that of cyclophosphamide and TBI except that none of the patients in the study had hemorrhagic cystitis or veno‐occlusive disease. After a median follow up of 114 months, 29 patients (23%) are still alive without relapse. The survival of patients with acute myelogenous or lymphoblastic leukemia transplanted in their first remission were 35% and 43%, respectively. The survival of patients transplanted in their first chronic phase of chronic myelogenous leukemia was 60%. The results of this preparative regimen are comparable to those of cyclophosphamide and TBI. © 1994 Wiley‐Liss, Inc.
Low fetal morbidity in pregnancy associated with acute and chronic idiopathic thrombocytopenic purpura
Authors: Sharon Rivka , Tatarsky Ilana 
Published: February 2007

AbstractForty‐six mothers with immune thrombocytopenic purpura (ITP) gave birth to 72 babies. Sixty‐two babies were delivered vaginally and 10 babies by cesarean section. There was no mortality among mothers or babies. Eighteen infants were born thrombocytopenic (PLT < 100 ± 109/l). Eleven infants had a platelet count of less than 50 ± 109/l. All the severely thrombocytopenic babies (except 1) were born to post splenectomy thrombocytopenic mothers, regardless of steroid treatment during pregnancy. Five babies had clinical manifestations of bleeding; 3 had mild purpura, 1 severe gastrointestinal bleeding, and 1 intracranial bleeding. The latter 2 babies were born prematurely to the same mother who was severely thrombocytopenic despite splenectomy in childhood. In view of very low morbidity in babies of ITP mothers, we suggest that they be delivered vaginally. Cesarean delivery should be performed in selected cases where the mother is severely thrombocytopenic despite splenectomy or where prematurity or obstetrical complications are encountered. © 1994 Wiley‐Liss, Inc.
Use of alveolar carbon monoxide measurements to assess red blood cell survival in hemodialysis patients
Authors: Medina Angela , Ellis Carol , Levitt Michael D. 
Published: February 2007

AbstractWe recently described a simple technique for measuring RBC survival based on measurements of the concentration of carbon monoxide (CO) in alveolar air, corrected for environmental CO using a device that equilibrates with atmospheric CO at the same rate as does the patient. The purpose of the present report is to demonstrate the clinical utility of this method via measurements of RBC turnover in hemodialysis patients. Prior to dialysis, the mean RBC survival of 9 chronic dialysis patients was 70 ± 9 days, about 50% shorter than that of 32 healthy subjects. During the dialysis, the endogenous Pco increased by 14% (P < 0.05) while subjects not undergoing dialysis had a 6% fall in endogenous Pco over this same time period. Thus, this technique demonstrated that the hemodialysis procedure resulted in about a 20% increase in RBC destruction. This increased RBC destruction has not been detectable with previous methodologies (including conventional measurements of CO production) due to the insensitivity and lack of reproducibility of conventional techniques. We conclude that the simple, non‐invasive measurement of endogenous Pco provides the most accurate available means of assessing the influence of a variety of acute manipulations on RBC survival. © 1994 Wiley‐Liss, Inc.