bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evidence for the synthesis of embryonic globin chains in adult erythroid progenitor cells
Authors: Bhaumik Kabita 
Published: February 2007

AbstractEmbryonic globin chains were found to be synthesized in vitro by the BFU‐E colonies derived from adult sickle cell anemia (SS) patients, their heterozygous relatives as well as a few normal controls. In the absence of sufficient material for conducting direct structural analyses of these peptides, they were confirmed by evaluating the co‐migration of the ϵ‐ and ζ‐chains with the corresponding structurally characterized globin chains obtained from K562 cell lysates on a reversed phase high performance liquid chromatogram. The presence of ζ‐chain was also confirmed using an immunologic procedure. Furthermore, significant 35S‐methionine incorporation peak was observed corresponding to the ζ‐chain synthesized by the BFU‐E‐derived colonies implying an active synthesis of this embryonic globin chain in BFU‐E cells obtained from hemopoietically adult persons.
Cyclosporine for the treatment of granulocytopenia in Felty's syndrome
Authors: Canvin Janice M. G. , Dalal Bakul I. , Baragar Fletcher , Johnston James B. 
Published: February 2007

AbstractA patient with Felty's syndrome was treated with cyclosporine, initially 10 mg/kg/day and then 4 mg/kg/day. The neutrophil count increased by 6 weeks and was normal at 3 months. Over the subsequent 27 months the neutrophil count was closely related to the cyclosporine dosage and there was no evidence of cyclosporine toxicity. This case indicates that cyclosporine may have a role in the treatment of Felty's syndrome.
Incidence and clinical characteristics of hereditary disorders associated with venous thrombosis
Authors: Tabernero Maria Dolores , Tomas Jose Francisco , Alberca Ignacio , Orfao Alberto , Borrasca Antonio Lopez , Vicente Vicente 
Published: February 2007

AbstractAt present, different congenital defects in several proteins—antithrombin III (AT III), protein C (PC), protein S (PS), and plasminogen (PLG)—are known to be causes of hereditary predisposition to thrombosis (thrombophilia).The incidence of these hereditary disorders in our 204 patients (106 males and 98 females) with venous thromboembolism were 4% (three cases deficient in PC, three in PS, two in PLG, and one patient in AT III). Their families were studied. In all cases the disorders were inherited as an autosomal dominant trait.The first thrombotic episodes occurred at a age of below 40 years. There was no relationship between protein levels and the occurrence of thrombosis, although a significant relationship was observed between a positive history of thromboembolic disease and a diagnosis of protein deficiencies.We evaluated the differences between primary thrombosis and secondary thrombosis. The most common thrombotic sites were the deep veins. There were no differences between males and females. Evaluation of PC, PS, AT III, and PLG in patients with thromboembolic disease should be considered.
Plasmin generation and fibrin(ogen)olysis following desmopressin infusion
Authors: Takahashi Hoyu , Tatewaki Wataru , Wada Ken , Niwano Hiroe , Hanano Masaharu , Shibata Akira 
Published: February 2007

AbstractDesmopressin acetate (DDAVP) is known to stimulate the release of tissue‐type plasminogen activator (t‐PA) from endothelial cells, but it is unclear whether the increased t‐PA actually elicits the plasmin generation and fibrin(ogen)olysis in the circulating blood. We measured plasma levels of plasmin‐α2‐plasmin inhibitor complex, fibrinogen degradation products (FgDP) and fibrin degradation products (FbDP) following desmopressin infusion in 19 patients with bleeding disorders or thrombophilia. Administration of desmopressin (0.3–0.4 μg/kg) produced a 4.0‐fold increase in plasmin‐α2‐plasmin inhibitor complex at 30 min, whereas neither FgDP nor FbDP was elevated significantly. These findings indicate that desmopressin infusion provokes the generation of plasmin in vivo, but most of the plasmin generated is complexed to α2‐plasmin inhibitor and does not degradate fibrin or fibrinogen.
Functional p75 interleukin‐2 receptor expression on the fresh blast cells in childhood acute lymphoblastic leukemia with natural killer cell properties
Authors: Ichikawa Motoki , Kawai Hiroshi , Komiyama Atsushi , Tsudo Mitsuru , Miyasaka Masayuki , Kinoshita Akitoshi , Nakazawa Shinpei 
Published: February 2007

AbstractNeoplastic cells of childhood acute lymphoblastic leukemia (ALL) with natural killer (NK) cell properties were studied for the expression of p75 interleukin‐2 receptors (IL‐2R) and the receptor functions. Freshly prepared blast cells from a patient with ALL had NK cell properties: (1) the phenotype such as CD56+, CD2+, E‐rosette+, CD3−, and CD19−; and (2) the presence of spontaneous cytotoxicity against NK‐sensitive K562 target cells. Although p55 Tac antigen was not detectable, there was the expression of p75 IL‐2R on the freshly prepared blast cells: 70% of the cells reacted with Mik‐β1 monoclonal antibody against p75 IL‐2R as determined by flow cytometry. Two‐color flow cytometry revealed that the blast cells expressed both p75 IL‐2R and NKH‐1. NK activity of the blast cells was augmented by their treatment with 1,000 U/ml recombinant IL‐2 (rIL‐2): the cytotoxicity level as percentage lysis increased to 38.7% from 22.0% when the normal lymphocyte value increased to 62.1% from 46.2%. Although the blast cells possessed no apparent level of proliferative capacity, the addition of 1,000 U/ml rlL‐2 yielded a 2.7‐fold increase in their thymidine uptake. These results demonstrate the expression of functional p75 IL‐2R on the patient's blast cells with NK cell properties.
Heparin‐induced thrombocytopenia and thrombosis: Reversal with streptokinase a case report and review of literature
Authors: Mehta Daksha P. , Yoder Ernest L. , Appel Joel , Bergsman Kenneth L. 
Published: February 2007

AbstractHeparin‐induced thrombocytopenia and thrombosis is associated with a significant incidence of morbidity and mortality. Prompt recognition of this complication and immediate withdrawal of heparin therapy are imperative. This report describes a case of heparin‐induced thrombosis and thrombocytopenia with major vascular insufficiency of the extremities. This is the first reported instance of the use of intravenous streptokinase for the treatment of heparin‐induced venous thrombosis.
A new frameshift β°‐thalassemia mutation (codons 27–28 +C) found in a Chinese family
Authors: Cai Shiping , Chui David H. K. , Ng Judy , Poon Annette O. , Freedman Melvin H. , Olivieri Nancy F. 
Published: February 2007

AbstractA new β°‐ halassemia mutation, a frameshift mutation with an insertion of a single cytosine nucleotide in codon 27–28, is described. The propositus, who is compound heterozygous for this mutation and the IVSII‐654 C→T β°‐thalassemia mutation, has the phenotype of severe β‐thalassemia major.
Low‐dose subcutaneous erythropoietin in continuous ambulatory peritoneal dialysis
Authors: Rotellar C. , Mazzoni M. J. , Mosher W. F. , Aminrazavi M. , Bauer A. , Rakowski T. A. , Schreiner G. E. , Winchester J. F. 
Published: February 2007

AbstractWe evaluated changes in hematocrit in patients on continuous ambulatory peritoneal dialysis (CAPD) before and after the administration of erythropoietin (EPO). Thirty‐five patients were evaluated at the beginning of treatment with CAPD and after an average of 3.5 years on CAPD; mean hematocrit (Hct) rose from 25.4 ± 5.4% to 28.1 ± 6.7% (P < 0.001). In the period before EPO administration 11 patients required a total of 44 transfusions (one patient needed 23 transfusions). Fifteen patients were started on subcutaneous erythropoietin 3,000 units 3 times a week and were followed for a mean period of 6.3 months. Hct rose from 23.8 ± 1.8% to 25.2 ± 2.4% (P < 0.01) within the first 2 weeks and up to 27.5 ± 3.7% (P < 0.01) in the fourth week. By the eighth week the target Hct (30 to 35%) was reached. During the next 5 months the EPO doses were adjusted to each patient's needs ranging between 2,000 U per week to 4,000 U 3 times per week. Mild hypertension was the only side effect seen in some of the patients. In conclusion low dose subcutaneous EPO is effective in managing the anemia of patients on CAPD with only minor side effects.
GM‐CSF in the treatment of Felty syndrome
Authors: Joseph Geetha , Neustadt David H. , Hamm John , Kellihan Michael , Hadley Terence 
Published: February 2007

AbstractMany therapeutic agents have been tried with variable success in the treatment of Felty neutropenia, but the reports are anecdotal. We now describe the second trial of recombinant granulocyte‐macrophage colony‐stimulating factor (GM‐CSF), in a splenectomized, infected patient with Felty syndrome.
Benign familial thrombocytosis
Authors: Williams Eliot C. , Shahidi Nassrolah T. 
Published: February 2007

AbstractA 13‐year‐old girl was found to have a platelet count in excess of 4 million/μl while being evaluated for a minor bleeding episode. Her father and two sisters also had thrombocytosis. All four affected patients were asymptomatic and had no clinical or laboratory evidence of a myeloproliferative disorder other than an elevated platelet count. This represents the second reported instance of benign familial thrombocytosis.
On the temporal development of erythrocyte sedimentation rate using sealed vacuum tubes
Authors: Kallner Anders 
Published: February 2007

AbstractThe temporal development of the erythrocyte sedimentation rate (ESR) was studied in wide, short vacuum tubes. It was found that in about 3% of the specimens arriving in the laboratory the ESR developed in three different phases during 60 min, whereas the other showed only two. The specimens with three phases behaved similarly in the Westergren method. It was shown that the Westergren ESR can be estimated with an acceptable accuracy already from measurements obtained after 30 min. Reproducibility and precision were improved by using a special instrument. Several advantages by this procedure were recognized, e.g., quicker results, identification of several otherwise missed rapid ESR. Accurate timing of the readings further improves accuracy and precision, and permits estimation of ESR (Westergren) up to 100 mm. In view of the obvious phases in the development of the ESR, it is proposed that this abbreviation is interpreted as erythrocyte sedimentation reaction and that the kind of quantity that is length is expressed in mm.
Heterogeneity of peripheral blood reticulocytes: A flow cytometric analysis with monoclonal antibody HAE9 and thiazole orange
Authors: Mechetner Eugene B. , Sedmak Daniel D. , Barth Rolf F. 
Published: February 2007

AbstractThe expression of a human erythroid cell surface antigen recognized by monoclonal antibody (mAB) HAE9 has been studied on peripheral blood reticulocytes by one‐ and two‐color flow cytometry. Total reticulocyte count was determined using Thiazole Orange (TO) and flow cytometry. In normal individuals, 4.56% of reticulocytes were stained by FITC‐labeled mAB HAE9. The correlation between reticulocyte percentage by TO and HAE9 staining was 0.828 (P < 0.0001) in patients with hematocrits less than 0.25. A HAE9‐positive reticulocyte percentage of 6–44% was observed when analyzed by two‐color flow cytometry with TO and mAB HAE9. These findings, in conjunction with previous studies, suggest that mAB HAE9 recognizes an early, less differentiated population of peripheral blood reticulocytes. Enumeration of immature reticulocytes may be of clinical utility.
Skin necrosis following prolonged administration of coumarin in a patient with inherited protein S deficiency
Authors: Goldberg Stuart L. , Kessler Craig M. , Yalisove Barbara L. , Elgart Mervyn L. , Orthner Carolyn L. 
Published: February 2007

AbstractDepression of the vitamin K‐dependent modulators of coagulation during the initial phase of oral anticoagulant therapy may lead to a transient hypercoagulable state and skin necrosis. We report a case of skin necrosis associated with inherited protein S deficiency following prolonged coumarin therapy. As a result of a stasis ulceration causing a rise in c4b binding protein and concurrent treatment with topical sulfonamide, we hypothesize that a precipitous decrease in free protein S led to skin necrosis. Progression of the necrosis was halted with unactivated prothrombin complex concentrate. This case confirms the rare association between coumarin induced skin necrosis and protein S deficiency and emphasizes potential drug interactions.
Increased response of erythroid progenitors to interleukin‐3 in sickle cell anemia: CFU‐E‐like behavior of circulating erythroid progenitors in liquid culture
Authors: Isoyama Keiichi , Baliga Surendra , Shah Arvind , Mankad Vipul N. 
Published: February 2007

AbstractErythroid progenitors circulating in peripheral blood and their response to erythropoietin (EPO), interleukin‐3 (IL3), and phytohemagglutinin‐stimulated, lymphocyte‐conditioned medium (PHALCM) were assessed in sickle cell anemia (SCA) patients and controls. SCA patients have significantly higher numbers of circulating burst‐forming unit–erythroid (BFU‐E) compared with controls (mean ± SEM, 940.27 ± 129.11 per ml and 86.56 ± 19.74 per ml, respectively; P < 0.0001). At low doses of EPO, BFU‐E‐derived colonies were significantly increased in SCA patients compared with controls (each P < 0.05). The EPO dose required to produce 50% of maximum colony numbers was 47 times greater in control subjects than in SCA patients. Moreover, in 11 of 17 patients with SCA, spontaneous BFU‐E‐derived colonies were formed without added erythropoietin. This phenomenon was not observed in control subjects (P = 0.035). PHALCM developed from mononuclear cells of SCA patients had significantly greater stimulatory effect than did that derived from controls regardless of the source of target cells (each P < 0.05). A two‐step study of IL3 sensitivity of erythroid progenitors was conducted. First, in a liquid culture system, circulating erythroid progenitors of SCA patients and controls were incubated in the presence of varying doses of IL3. During a second step, CFU‐E‐like colonies were observed in methylcellulose cultures of these cells. The mean numbers of colony‐forming unit‐erythroid (CFU‐E)‐like colonies was significantly higher in SCA patients compared with control subjects at low doses of IL3 (each P < 0.02). The increased response of erythroid progenitors to IL3 and the increased production of hemopoietic growth factors (IL3 or non‐IL3) contribute to the hemopoietic response in SCA patients. These mechanisms and increased sensitivity of the BFU‐E to EPO may explain lower than expected EPO levels in SCA patients.
Effect of tranexamic acid on platelet ADP during extracorporeal circulation
Authors: Soslau Gerald , Horrow Jan , Brodsky Isadore 
Published: February 2007

AbstractSeventeen adults received the antifibrinolytic drug tranexamic acid during cardiac surgery utilizing extracorporeal circulation (ECC). In 8 patients, drug administration began prior to skin incision (pre‐ECC); infusions commenced after ECC and protamine administration in another 9 patients (post‐ECC). Compared with the post‐ECC group, the pre‐ECC group exhibited less bleeding via mediastinal drains (420 vs. 655 mL/12 h median, P = 0.024), decreased frequency of the presence (≥10 γg/mL) of fibrin split products (P < 0.05), and greater platelet dense granule content of adenosine diphosphate after surgery (15.47 vs. 4.05 nmoles/mg protein median, P = 0.021).Follow‐up in vitro study of tranexamic acid inhibition of plasmin‐induced platelet activation utilizing normal human platelet rich plasma and porcine plasmin revealed a 13‐fold lower concentration of tranexamic acid for 50% inhibition when plasmin was preincubated with the drug (1.2 γg/mL, 95% CI = 1.13 – 1.60 γg/mL) compared to when platelet rich plasma was preincubated with the drug (16 γg/mL, 95% CI = 7.3 – 99. γg/mL). Plasmin inactivated with tranexamic acid retained its ability to inhibit thrombininduced platelet activation, thus suggesting that tranexamic acid inhibits plasmin's catalytic activity and not its binding to platelets.Both clot lysis and platelet dysfunction may contribute to bleeding after ECC. Tranexamic acid blocks plasmin‐induced partial platelet activation during ECC, thus preserving platelet function and promoting hemostasis after ECC.
Small bowel hematoma in a hemophiliac as a cause of pseudoappendicitis: Diagnosis by ct imaging
Authors: McCoy Harry E. , Kitchens Craig S. 
Published: February 2007

AbstractAcute abdominal pain in a hemophiliac should be approached as hemorrhage until proven otherwise. With factor replacement available, a conservative approach can be taken as opposed to immediate exploratory surgery. Diagnostic imaging can define areas of hemorrhage. We report a case of “pseudoappendicitis” in a hemophiliac with terminal ileum circumferential bowel wall hematoma diagnosed by CT with the initial diagnostic scan. Factor replacement resulted in resolution of the signs and symptoms of the pseudoappendicitis and follow‐up CT scan revealed resolution of the hematoma. A review of the literature reveals few CT documented circumferential bowel wall hematomas with limited clinical histories and no cases with follow‐up scans after conservative management.
Application of a monoclonal antibody specific for the δ chain of hemoglobin A2 in the diagnosis of β thalassemia
Authors: Shyamala M. , Kiefer Charles R. , Moscoso Hugo , Garver Fred A. 
Published: February 2007

AbstractWe have developed a murine monoclonal antibody (mAb) specific for the δ chain of hemoglobin (Hb) A2 that does not cross‐react with α, β, or γ chains. The mAb reacted with Hb P‐Nilotic (β δ hybrid), but not with Hb Lepore‐Boston (δ β hybrid), indicating an epitope consisting of positions 116 (Arg) and 117 (Asn) or 125 (Gin) and 126 (Met) of the δ chain. By using this antibody, we have established a simple and rapid enzyme‐linked immunosorbent assay (ELISA) for the detection and quantification of Hb A2 in adult, cord, and fetal hemolysates. We analyzed 70 adult, 8 newborn, and 19 fetal hemolysates from normal subjects and those with various hemoglobinopathies. The mean percentage of Hb A2 was 2.5 for normal adults, 5.4 for β thalassemic (β thai) heterozygotes, and <0.1% in β thal fetal samples. We were able to distinguish and characterize certain phenotypes of β thai patients such as β thai heterozygotes, β° thai homozygotes, and Cβ° thal, and Cβ° thal double heterozygotes with the aid of this and other mAbs we have generated. This technique is a valuable addition to current methods for the diagnosis of β thai based on quantification of Hb A2.
Hemophagocytic syndrome: A cause of pancytopenia in human ehrlichiosis
Authors: Abbott Kevin C. , Vukelja Svetislava J. , Smith Craig E. , McAllister C. Kenneth , Konkol Keith A. , O'rourke Timothy J. , Holland Cynthia J. , Ristic Miodrag 
Published: February 2007

AbstractA 67‐year‐old white man with human ehrlichiosis infection complicated by pancytopenia, hemophagocytic syndrome, disseminated intravascular coagulopathy and septic shock is presented. The patient had been on a three‐week camping trip to California, Colorado, Utah, and New Mexico. The diagnosis of human ehrlichiosis was confirmed by sixteenfold rise in antibody titer to Ehrlicha canis, and supported by the characteristic cytoplasmic inclusions. Human ehrlichiosis should be considered in the differential diagnosis in patients with fever and cytopenia associated with hemophagocytosis. Pancytopenia associated with ehrlichiosis is transient; however, it may be severe, and appears to be associated with destruction of normal blood elements.
Lupus anticoagulant associated with primary malignant lymphoplasmacytic lymphoma of the spleen: A report of four patients
Authors: Ciaudo M. , Horellou M. H. , De Carbonnieres C. , Conard J. , Samama M. , Audouin J. 
Published: February 2007

AbstractPrimary lymphoma of the spleen is characterized by predominant splenomegaly. Lymphoplasmacytic malignant lymphoma of the spleen, of low malignancy in the Kiel classification, low and intermediate grade in the National Cancer Institute Working Formulation (NCIWF), is rare. It is often associated with a monoclonal immunoglobulin M (IgM).Four patients presenting with primary splenic lymphoma of plasmacytic type associated with a high level of monoclonal IgM and a lupus anticoagulant (LA) are described. This association has not previously been reported. In contrast with the usual heterogeneity of LA, this LA is relatively homogeneous with an important prolongation of the prothrombin time (>18 see for a control of 12), more prolonged partial thromboplastin time (PTT) of the mixture patient + control plasma than PTT of the patient plasma. Despite the important coagulation abnormalities, none of these four patients has presented any hemorrhagic or thrombotic complications, even during major surgery such as splenectomy.The lupus‐like anticoagulant effect ran parallel with the monoclonal IgM. Survival, after splenectomy and chemotherapy, appears to be favourable: three patients are alive with survivals of ≧7 years. The follow‐up is as yet too short for the last patient.
Hemoglobin constant spring in bangkok: Molecular screening by selective enzymatic amplification of the α2‐globin gene
Authors: Thonglairoam V. , Fucharoen S. , Tanphaichitr V. S. , Pung‐amritt P. , Embury S. H. , Winichagoon P. , Wasi P. 
Published: February 2007

AbstractHemoglobin Constant Spring (Hb CS) is a hemoglobin variant with an elongated α‐globin chain secondary to a chain termination mutation. The diagnosis of HbCS by electrophoresis is difficult because it is present in very low amounts in the red cells of heterozygotes. Selective enzymatic amplification of the α2‐globin gene and allele‐specific hybridization for Hb CS gene provided accurate diagnosis of Hb Constant Spring. We have used this approach to detect the αcs mutation in the cord blood that contained all four α‐globin genes but had Hb Bart on electrophoresis. The αcs mutation was found in six subjects whose Hb Bart levels were 3.0, 3.2, 3.7, 4.0, 4.9, and 9.8%. The latter also had −α mutation on the other chromosome, giving rise to the genotype αcsα/−α, which produced high Hb Bart. The gene frequency for αcs in the Thai calculated from a total of 406 cord blood studied in Bangkok was found to be ∼0.008.
Relationship Between Cell‐Cycle State of Erythroid Progenitors and Fetal Hemoglobin Synthesis
Authors: Isoyama Keiichi , Baliga Surendra , Shah Arvind , Mankad Vipul N. 
Published: February 2007

AbstractDNA‐synthesis state of circulating burst forming unit‐erythroid (BFU‐E) was evaluated in patients with sickle cell anemia and correlated with percent of fetal hemoglobin synthesized in the BFU‐E‐derived cells. Percentage of S‐phase BFU‐E inversely correlated with percent fetal hemoglobin synthesized in the BFU‐E‐derived cells (simple linear correlation coefficient, r = −0.8, P = 0.0302; polynomial regression, R = ‐0.99, P = 0.0002). This observation is of relevance to our understanding of the relationship between the developmental stage of the erythroid progenitors and expression of globin genes.