bluuebunny/crossref_metadata_2025_split huggingface dataset:
Childhood monosomy 7 syndrome
Authors: Gyger Martin , Bonny Yvette , Forest Lorraine 
Published: February 2007

AbstractIn recent years, chromosomal aberrations in various hematologic disorders have raised a great deal of interest. In fact, several nonrandom chromosomal abnormalities are now recognized to be responsible for a specific type of dyshemopoiesis while others are closely associated with characteristic hematologic features. Monosomy C, later shown to be monosomy 7 by different banding methods, has been described in children in relation to a peculiar myeloproliferative disorder. Retrospective analysis of early cases published in the literature and a recent observation that we wish to report suggest that the most consistent phenotypic expression of monosomy 7 is an increased susceptibility to bacterial infections related to a preleukemic dyshemopoiesis. Acute nonlymphocytic leukemia is the terminal event of this peculiar preleukemic syndrome, and thus suggests that monosomy 7 involves a stem cell already committed to myeloid differentiation.
Factor VIII inhibitors: A clinical overview
Authors: White Gilbert C. , McMillan Campbell W. , Blatt Philip M. , Roberts Harold R. 
Published: February 2007

AbstractThere is much evidence to indicate that inhibitors to Factor VIII in patients with classical hemophilia are the result of an immunological response to exposure to material (VIII:C or VIII:CAg) that is absent or present in reduced amounts in these patients. The inhibitor is an antibody that is usually restricted in immunochemical composition and in many instances contains predominantly or exclusively γG3 or γG4 heavy chains. Exposure to Factor VIII in many inhibitor patients leads to typical anamnestic responses with marked increases in the level of the inhibitor. The tendency to develop inhibitors and the clinical characteristics of the inhibitor may be affected by genetic factors, basal levels of Factor VIII:C and/or VIII:CAg, and the nature and amount of the “immunizing” material. Currently accepted therapeutic modalities are aimed primarily at the management of acute bleeding episodes.
Mechanism of canine cyclic hematopoiesis: The role of prostaglandin E in feedback regulation
Authors: Hammond William P. , Robertson R. Paul , Dale David C. 
Published: February 2007

AbstractProstaglandin E inhibits granulocyte‐macrophage colony formation in vitro in man and mouse, suggesting that it plays a role in feedback regulation of granulocyte production in vivo. Therefore, we examined the role of PGE in normal canine hematopoiesis and its potential role in the pathogenesis of cyclic hematopoiesis in grey collie dogs. The prostaglandin synthesis in hibitors indomethacin and ibuprofen (10−5 M) increased CFU‐C growth to 194 and 160% of control, respectively, while PGE2 addition caused a dose‐dependent inhibition of bone marrow CFU‐C growth in both normal and grey collie dogs. These concentrations of indomethacin and ibuprofen decreased bone marrow cell elaboration of PGE measured by radioimmunoassay to less than 5% of control values. The levels of PGE in leukocyte conditioned medium prepared from grey collies correlated with the number of monocytes in the conditioning cell suspension (r=0.78, n=10, p<0.05) so that PGE production per monocyte was no different in normal and grey collie dogs. The effect of PGE2 upon CFU‐C was to inhibit formation of macrophage, but not neutrophil colony subtypes. These findings make aberrant PGE‐mediated inhibition of precursor cells an unlikely mechanism to cause cyclic hematopoiesis, and show that PGE produced by monocytes acts as a feedback inhibitor for precursor cells destined to produce monocytes but not for those destined to form neutrophils.
Correlation of in vitro culture pattern and Q‐banded karyotype in acute nonlymphocytic leukemia
Authors: Richman Carol M. , Rowley Janet D. 
Published: February 2007

AbstractBoth cytogenetic abnormalities and in vitro growth patterns of leukemic cells have prognostic significance in acute nonlymphocytic leukemia (ANLL). The present study was undertaken to analyze the interrelationship between these two factors and response to therapy. Blast cells from 43 patients with de novo ANLL, four patients with secondary ANLL, and two patients with ANLL following a preleukemic phase were studied using both in vitro culture in methylcellulose and quinacrine chromosome banding techniques. In 19 patients with de novo ANLL, minimal growth in culture (≤ 5 colonies without prominence of small clusters) was noted (Pattern I). Blast cells from the remaining 24 patients formed numerous abnormal clusters and colonies in a continuum distribution (Pattern II). Sixty‐three percent of Pattern I patients had completely normal karyotypes (NN). Only 29% of Pattern II patients were NN. No Pattern I patient had only abnormal karyotypes (AA), while 25% of Pattern II patients were AA (p<0.05). All six patients with secondary leukemia or leukemia following a preleukemic phase demonstrated both Pattern II growth and cytogenetic abnormalities. Fifty‐six percent of Pattern I patients with de novo ANLL responded to chemotherapy, 35% of Pattern II patients responded, and 0% of patients with secondary or post‐preleukemic ANLL responded. The correlation between the presence of cytogenetic abnormalities in leukemic cells and a pattern of excessive, abnormal growth in vitro coupled with the trend toward poor prognosis in these patients suggests that cytogenetically abnormal cells may have a proliferative advantage both in vitro and in vivo.
Febrile Transfusion Reaction Following Initial Transfusion in a Man With Immunoblastic Lymphadenopathy and Granulocyte Autoantibodies
Authors: Barnes Harry M. , Poon Man‐Chiu , Huang Shu Tsong , Conrad Marcel E. , Lin John , McGowan Eoline I. 
Published: February 2007

AbstractA 69‐year‐old man with immunoblastic lymphadenopathy and autoimmune hemolytic anemia who had no previous exposure to blood products developed a severe febrile nonhemolytic transfusion reaction following the initial infusion of packed red blood cells. The reaction recurred with transfusion of packed red blood cells, but not when freeze‐thawed red blood cells were used. Immunofluorescence techniques demonstrated granulocyte antibodies in his serum and on the surface of his granulocytes. Circulating immune complex, HLA, and platelet antibodies were not present. The granulocyte antibodies fluctuated in titers with disease activities, and could be completely removed from the serum by autologous granulocyte absorption. We conclude that our patient had granulocyte autoantibodies which probably produced febrile transfusion reactions.
Schistosomal stimulation of eosinophil production by human bone marrow in vitro
Authors: Robinson William A. , Entringer Maureen A. , Mahmoud Lotfy Ablel Naby , Drebing Carla J. 
Published: February 2007

AbstractThese studies were undertaken to determine if schistosomal and ragweed antigens incubated with human peripheral blood mononuclear cells (MNC) would stimulate the production of an eosinophil colony stimulating factor (CSF) which was active on human bone marrow. These studies have shown that schistosoma mansoni antigen in a concentration of 5 μg/ml incubated with MNC's from unsensitized individuals lead to production of a conditioned medium which did not stimulate an increased number of total colonies but did increase the number of eosinophil colonies (44 to 61 colonies). Ragweed antigen also stimulated increased eosinophil CSF (from 44 to 63 colonies).The granulocyte response to parasitic antigens almost invariably involves the eosinophil system. Eosinophils play a vital role in the control of these diseases in humans. Further understanding of the interaction of parasitic antigens with the granulopoietic system will hopefully give new and important clues as to how such parasites interact with man and how they are controlled.
Clostridial species in the pathogenesis of necrotizing enterocolitis in patients with neutropenia
Authors: Hopkins D. Gregg , Kushner James P. 
Published: February 2007

AbstractThe role of gram‐negative bacteria in the pathogenesis of necrotizing enterocolitis has been stressed. We describe a 21‐year‐old woman with chronic neutropenia with cyclic fluctuations who died of necrotizing enterocolitis due to Clostridium septicum. Seven patients with cyclic neutropenia and necrotizing enterocolitis have been described in the literature and in each case in which bacteriologic data were reported, a Clostridial infection was documented. Patients with cyclic neutropenia should be treated with antibiotics effective against Clostridial species when abdominal symptoms complicate neutropenic periods.
Burkitt cell leukemia with abnormality of chromosome no. 1
Authors: Solomons H. D. , Mendelow B. , Levinstein A. , Johnson A. E. , Bernstein R. 
Published: February 2007

AbstractA case of L3 (Burkitt cell) leukemia with chromosome 1 abnormality but no detectable abnormalities of chromosomes 8 or 14 is reported. This is the first time that L3 leukemia has been shown to be associated with a primary abnormality of chromosome 1. The implications of these findings relative to the development of B‐cell ALL and the role of chromosome 1 in human neoplasia is discussed.
Biochemical characterization of three mutant isozymes of erythrocyte pyruvate kinase: PK‐“Gainesville,” PK‐“San Juan,” and PK‐“Cape Canaveral”
Authors: Paglia Donald E. , Keitt Alan S. , Valentine William N. , Gordon Shirley 
Published: February 2007

AbstractPyruvate kinase was partially purified from erythrocytes of three unrelated, nonconsaguineous patients with chronic hemolytic anemia of differing clinical severities. Characterization of the defective PK isozymes by internationally standardized criteria indicated that one (PK‐“Gainesville”) had severely impaired substrate affinity, another (PK‐“San Juan”) had markedly reduced residual activity, and the third (PK‐“Cape Canaveral”) had a combination of milder defects. Each appears representative of subsets emerging from the heterogeneous molecular defects that make up pyruvate kinase deficiency.
Aging of the erythrocyte. 23. Changes in the permeation of spin‐labeled electrolytes
Authors: Bartosz Grzegorz , Gwozdzinski Krzysztof 
Published: February 2007

AbstractThe transport of spin‐labeled anions and cations was studied in bovine erythrocyte fractions of various ages. Permeation of a spin‐labeled anion, 3‐carboxy‐2,2,5,5‐tetramethylpyrrolidine‐1‐oxyl into the cells was found to be significantly slower in older cells, suggesting a functional impairment of the band 3 protein of the erythrocyte membrane during red cell aging in vivo.
Percentages of abnormal hemoglobins in adults with a heterozygosity for an α‐chain and/or a β‐chain variant
Authors: Huisman Titus H. J. 
Published: February 2007

AbstractThe author reviews quantitative data from blood samples involving more than 700 simple and compound heterozygotes for one of 21 types of α‐chain and 36 types of β‐chain variants. All samples were analyzed during the past few years by the same analytical procedure. Various factors influencing the percentages of the different abnormal hemoglobins are discussed; these include the variability of the method, the number of active α‐chain genes, the location of the substitution, the type of substitution, the effect of the loss of a critical amino acid residue, and the introduction of a new residue on the physicochemical and/or functional properties of the protein. The use of a single accurate method is promoted; it may well be that high‐pressure liquid chromatographic procedures will be the recommended methods for future analyses.
Impaired pentose phosphate shunt function in sickle cell disease: A potential mechanism for increased heinz body formation and membrane lipid peroxidation
Authors: Lachant Neil A. , Davidson Warren D. , Tanaka Kouichi R. 
Published: February 2007

AbstractThe red cells' antioxidant defense mechanisms were compared between individuals with sickle cell disease and those with hemolytic anemia and reticulocytosis. In sickle cell disease, there was a significant increase in incubated Heinz body formation (p < .001), a decrease in reduced glutathione concentration (p < .01), an increase in glucose‐6‐phosphate dehydrogenase activity (p < .01), and a decrease in glutathione reductase activity (p < .005). The patients with sickle cell disease had an absolute increase in the activity of the pentose shunt in the intact red cell after methylene blue stimulation (p < .05) and in red cell hemolysates (p < .025). Heinz body formation (r = .75) and pentose shunt activity in red cell hemolysates (r = .83) were strongly related to the degree of reticulocytosis. Although there was a correlation between the pentose shunt activity in the stimulated red cell and in red cell hemolysates for the patients with hemolytic anemia (r = .58), stimulated shunt activity did not increase as the hemolysate shunt activity increased for the patients with sickle cell disease. There were very strong relationships between the ATP concentration and the reticulocyte count (r = .80) and the hemolysate pentose shunt activity (r = .77) in sickle cell disease. These data suggest that in spite of an absolute increase in stimulated pentose shunt activity, there is a relative suppression of stimulated shunt activity in the youngest sickle erythrocytes. This may be related, in part, to the inhibitory effects of high concentrations of ATP on the activity of glucose‐6‐phosphate dehydrogenase.
Acute leukemia and infections: Perspectives from a general hospital
Authors: Balducci Lodovico , Halbrook John C. , Chapman Stanley W. , Vance Ralph B. , Thigpen J. Tate , Morrison Francis S. 
Published: February 2007

AbstractThe incidence and etiology of infections in 210 acute leukemics at the University of Mississippi Medical Center between 1962 and 1978 were reviewed. Infectious episodes occurred 269 times in 148 patients. In 193 infections, potential pathogens were cultured. Infection was a contributing cause of death in 89 patients. E. Coli, S. aureus, K. pneumoniae, and P. aeruginosa accounted for 58% of the isolates. No unusual pattern of antimicrobial resistance were observed. The outcome of the infections was related to the absence or resolution of neutropenia. Among 48 patients febrile on first admission, four cases of gram‐negative pneumonia, two cases of fungal pneumonia, and two cases of pseudomonas cellulitis were diagnosed.We conclude that the etiology of infections was similar to that of cancer centers; multidrug‐resistant gram‐negative organisms were not prevalent; absence or resolution of neutropenia indicates a good prognosis for outcome of infection; and untreated acute leukemics may acquire opportunistic infections.
Effect of splenic congestion associated with hemolytic anemia on mortality of rats challenged with haemophilus influenzae b
Authors: Chen L. T. , Moxon E. R. 
Published: February 2007

AbstractThe effect of splenic congestion associated with acute hemolytic anemia on susceptibility to bacterial infection was investigated in rats inoculated with Haemophilus influenzae b by intranasal (in) or intravenous (iv) challenge. The rats were made anemic by phenylhydrazine treatment and were challenged with 106 (in) or 5 × 107 (iv) H influenzae b. Forty‐eight hours after in inoculation of bacteria, the number of bacteria in the blood was 10 times greater in the anemic rats with extensive splenic congestion than in controls. After iv inoculation of bacteria, a significantly (P < 0.001) higher mortality rate was found in the anemic rats with extensive splenic congestion and fewer bacteria were present in the spleens but not the livers, as compared to normal rats. Since phenylhydrazine‐induced hemolytic anemia exhibited an extensive congestion in the spleen, higher mortality rate observed in the anemic rats challenged with H influenzae b may result, in part, from decreased intravascular clearance of bacteria by the spleen.
Calmodulin level in whole blood correlates with the percentage of reticulocytes
Authors: Warth James A. , Brewer George J. , Gnegy Margaret E. , Treisman Glen , Near Karen 
Published: February 2007

AbstractWe studied the calmodulin‐like activity (CaM) level in hemolyzed samples of whole blood after determining the percentage and absolute number of reticulocytes present. Twenty‐six samples from 25 people with a range of reticulocyte counts were studied. CaM levels correlated with the percentage (r = 0.63, P < .01) and absolute number (r = 0.64, P < .01) of reticulocytes present, indicating that the calmodulin‐like activity level in whole blood was inversely related to erythrocyte age.
The clonogenic assay as a reproducible in vitro system to study predictive parameters of treatment outcome in acute nonlymphoblastic leukemia
Authors: Browman George , Goldberg Jack , Gottlieb Arlan J. , Preisler Harvey D. , Azarnia Nozar , Priore Roger L. , Brennan James K. , Vogler William R. , Winton Elliott F. , Miller Kenneth B. , Grunwald Hans 
Published: February 2007

AbstractSeven institutions studied the cloning pattern of leukemic cells from pretherapy bone marrows of 273 patients with newly presenting and relapsed acute nonlymphoblastic leukemia. The cloning assay was done in all centers using an identical double‐layer agar method with a common source of colony‐stimulating factor. Cells were incubated for seven days, and clones were identified visually with an inverted microscope. All centers were able to obtain clonal growth in a substantial proportion of patients. Differences in growth pattern were observed between the major contributing center and the pooled results for all other centers. However, an analysis of clinical results suggested that in vitro differences were more likely related to differences in the patient populations than to variability in laboratory technique. The proportion of marrows in which leukemic cells formed colonies (>40 cells) and large clusters (20‐40 cells) was greater in relapsed patients than in newly presenting patients (P < .06). A progressive improvement in induction treatment outcome was seen with decreasing clonal growth. Patients whose marrows did not produce clones had a complete remission (CR) rate of 83% versus 50% for those patients whose marrow leukemic cells formed colonies and/or large clusters (P=.05). In vitro drug sensitivity studies with cytosine arabinoside and adriamycin were performed on bone marrow cells of patients treated with this combination clinically. The percent killing of clonogenic cells in this assay correlated with remission induction outcome. Complete remission was obtained in 73% of 15 patients whose bone marrow leukemic cells showed >30% killing by in vitro drug exposure, and CR was obtained in only 30% of 23 patients whose marrows showed < 30% killing (P< .01). The results indicate that the clonogenic assay correlates with treatment outcome and can be used for drug sensitivity testing in patients with acute nonlymphoblastic leukemia. The limitations of the assay are discussed.
Treatment of acute sickle cell crises with a vasopressin analogue
Authors: Charache Samuel , Moyer Martha A. , Walker W. Gordon 
Published: February 2007

AbstractEight patients with sickle cell anemia were treated for acute painful crises with DDAVP and intravenous fluids; five were treated with placebo and the same regimen of fluid administration. Although hyponatremia was produced in both treatment groups, duration of hospitalization did not differ between them, nor did it differ from concurrent hospitalization of other patients who received conventional treatment. Safe induction of hyponatremia required intensive laboratory surveillance, and serum sodium could be lowered without use of DDAVP. These data suggest that a controlled trial hyponatremia for acute sickle cell crises should not be performed.
A variant 8;21 translocation in acute myeloblastic leukemia
Authors: Oguma Nobuo , Misawa Shinichi , Testa Joseph R. 
Published: February 2007

AbstractA three‐way rearrangement involving chromosomes 1, 8, and 21 was observed in bone marrow cells from a female with acute myeloblastic leukemia, subtype M2 (AML‐M2). The translocation break points in chromosomes 8 and 21 were identical to those found in the standard t(8;21) that is associated with this disease type. A review of this and four previously reported cases involving variant 8;21 translocations indicates that a consistent pattern of exchange may be involved in these complex rearrangements even though the third chromosome affected may differ.
Adult T‐cell lymphoma leukemia in western countries
Authors: Farcet Jean Pierre , Kuentz Mathieu , Andre Chantal , Darves Jean Marc , Reyes Félix , Dreyfus Bernard , Rochant Henri 
Published: February 2007

AbstractA new T‐cell disorder has recently emerged: the so‐called adult T‐cell lymphoma leukemia (ATLL) initially described in Japan. Subsequently, ATLL cases were recognized in patients from the Caribbean. We summarize the clinical and hematological features of 19 published cases from Western countries, in addition to a new case we encountered. The leukemic cells display characteristic morphological features and a T3+ T4+ T8− T6− surface antigenic phenotype. Overall survival is of short duration, but remission could be obtained in our case despite a subsequent relapse in skin and CNS. Geographic clusters of ATLL cases have led to the discovery of the possible role of a new retrovirus, HTLV, in the genesis of this rare malignancy.
Evidence that less replacement therapy is required for dental extractions in hemophiliacs
Authors: Steinberg Stephen E. , Levin Jack , Bell William R. 
Published: February 2007

AbstractThe requirements for factor VIII (AHF) or factor IX (PTC) of hemophilic patients undergoing dental extractions were evaluated to determine the minimum effective regimen. Sixteen patients underwent 19 operative procedures. The mean total dose of factor VIII or IX was 45.8 U/kg for 11 procedures with preoperative replacement therapy and 34.5 U/kg for the 8 without. Four patients received no replacement therapy at all.Our results compared favorably to published studies employing factor replacement alone or in combination with antifibrinolytic agents such as epsilon‐amino‐caproic acid (EACA), with respect to blood products utilized and duration of hospitalization. However, our patients received less factor replacement than usually suggested. General anesthesia with intubation, a common recommendation, was not required in any patients.Dental extractions can be carried out in hemophiliacs using less replacement therapy than currently recommended. In some circumstances, no replacement therapy may be necessary. The reported efficacy of antifibrinolytic agents in reducing the requirement for replacement therapy is difficult to assess because of the relatively large amount of prophylactic factor replacement used in conjunction with these agents.
Transferrin receptors on human reticulocytes: Variation in site number in hematologic disorders
Authors: Shumak Kenneth H. , Rachkewich Rose A. 
Published: February 2007

AbstractAssays of binding of 125iodine‐labeled (125I) human transferrin were used to study transferrin receptor sites on reticulocytes from 15 normal subjects and from 66 patients with various hematologic disorders. In normal subjects, few or no transferrin receptors were detected whereas the average number of receptors per reticulocyte varied greatly from patient to patient, ranging from 0 to 67,700 in samples, from 35 patients, on which Scatchard analysis of binding of [125I]‐transferrin was done. Marked heterogeneity in the number of reticulocyte transferrin receptors in different hematologic disorders was also found in assays with [125I]‐OKT9 (monoclonal antibody to the human transferrin receptor). The number of receptors was not correlated with either the reticulocyte count or the hemoglobin.