bluuebunny/crossref_metadata_2025_split huggingface dataset:
A long‐term study of hemophilic arthropathy of the knee joint on a program of factor VIII replacement given at time of each hemarthrosis
Authors: Brettler Doreen B. , Forsberg Ann D. , O'Connell Francis D. , Cederbaum Andrew I. , Chaitman Andrea K. , Levine Peter H. 
Published: February 2007

AbstractBecause there is little available data to demonstrate whether demand therapy is adequate to prevent progression of hemophilic arthropathy, a cohort of 64 patients with severe Hemophilia A (Factor VIII level < 1%) and no inhibitor were studied with respect to the progression of knee arthropathy over a period of 6 years. Both degree of disease in flexion and extension and progression of disease were rated on an arbitrary scale. At the outset of the study, the majority of knees were either not restricted or mildy restricted as to motion. Of those knees that were not restricted at the outset, 96% remained not restricted. Of the knees that were mildy restricted, only 58% remained stable or improved over the 6 years, and of the knees that were moderately to severely restricted, 75% improved or remained stable. It thus appears that a demand therapy program can maintain normal range of motion in a knee joint. However, if the patient's joint has progressed to mild disease, the ability to maintain or improve the joint with demand therapy appears to be decreased.
Use of 75se‐labeled methionine to study the sequestration of senescent red blood cells
Authors: Smedsrød Bård , Aminoff David 
Published: February 2007

AbstractLabeling red blood cells with Na251CrO4 enabled us to study certain aspects of red cell survival and sequestration from the circulation. As a random labeling procedure, however, the 51Cr method has certain limitations. Therefore, we developed a cohort labeling method using 75Se‐methionine as a two‐rat procedure. This gives us a clear pulse‐labeled population of rat red cells to study the dynamics of sequestration. With this labeling procedure, it was possible to demonstrate that 1) there is an increase in the density of red cells with age, 2) a significant sequestration of red cells from the circulation is apparent at the end of 48 days and essentially is complete at the end of 60 days, 3) there is a corresponding uptake of senescent red cells in the spleen, which peaks at 55 days, and 4) the 60‐day end point is sharper and is more definitive when the “specific activity” (cpm per red blood cell) of the labeled red cells in the spleen is compared to that of the red cells still in the circulation. Asialo red cells, obtained by removal of sialic acid with sialidase, frequently have been used as a model for the study of sequestration of senescent red cells. With the technique herein described, it was possible to show that while asialo red cells will inhibit the uptake of labeled asialo red cells, they have no effect on the sequestration of senescent red cells. Presumably, different sites and mechanisms of sequestration are involved.
Steroid‐induced hypersegmentation in neutrophiles
Authors: Eichacker Peter , Lawrence Christine 
Published: February 2007

AbstractThe effect of glucocorticoid administration on the nuclear segmentation of circulating granulocytes was studied in 13 patients with hematologic disorders and in two normal volunteers. In seventeen of eighteen courses, the granulocyte nuclear lobe count increased from 3.24 ± 0.12 (SD) to 3.41 ± 0.12 by the third day of treatment (P < .001). Nuclear segmentation increased even further with continued therapy during a week of observation. Thus, like deficiencies of folate and of vitamin B12, glucocorticoid therapy causes neutrophil hypersegmentation. The cause of this effect is not known.
Increased heinz body formation and impaired erythrocyte pentose phosphate shunt function during pregnancy
Authors: Lachant Neil A. , Gottlieb Arlan J. , Difino Santo M. , Landaw Stephen A. , Tanaka Kouichi R. 
Published: February 2007

AbstractThe erythrocytes of 90 pregnant women were evaluated for the presence of in vivo or in vitro oxidant damage. The reduced glutathione (P < 0.005) and the membrane reduced sulfhydryl (P < 0.001) concentrations were decreased in fresh erythrocytes. Following incubation with acetylphenylhydrazine, Heinz body formation was significantly increased (P < 0.001). Both the increase in Heinz body formation and the reduction in membrane reduced sulfhydryl content correlated strongly with duration of pregnancy. Glucose consumption was significantly decreased before, but not after, new methylene blue stimulation. Pentose phosphate shunt activity was impaired both before (P < 0.05) and after (P < 0.001) stimulation. No changes were observed in pentose phosphate recycling. The only alteration observed in the activity of the enzymes of the pentose shunt was an elevation of 6‐phosphogluconate dehydrogenase activity. Although the clinical significance of these findings remains to be determined, medications with an oxidant potential should be used judiciously during gestation.
Philadelphia‐chromosome‐negative chronic myelogenous leukemia with lymphoid stem cell blastic transformation
Authors: Kessler John F. , Grogan Thomas M. , Greenberg Bernard R. 
Published: February 2007

AbstractA patient is described who had blastic transformation of Ph1 negative chronic myelogenous leukemia (Ph1 – CML). Characterization of the leukemic cells revealed a population with a lymphoid stem cell phenotype (cALL–, TdT +, Ia+, cIgM –). This particular phenotype may be responsible for the refractoriness to vincristine and prednisone and the rapid downhill course.
Platelet transfusion therapy in acute leukemia: Lack of effect of splenomegaly on transfusion requirements and risk of hemorrhage
Authors: Witzig Thomas E. , Ducatman Barbara S. , Wick Mark R. , Letendre Louis , Moore S. Breanndan , Ilstrup Duane M. , Taswell Howard F. 
Published: February 2007

AbstractPlatelet transfusions are an important supportive measure during treatment for acute nonlymphocytic leukemia (ANLL). The presence of splenomegaly may produce decreased posttransfusion platelet increments leading some to recommend an increased dose of platelets per transfusion in this situation. Forty‐nine newly diagnosed patients with ANLL were evaluated during 1980 and 1981, and 24% had palpable splenomegaly. Although treated with usual doses of platelets per transfusion, there was no detectable statistical increase in transfusion requirement or incidence of hemorrhage in patients with splenomegaly. Experimental evidence indicates that the splenic platelet pool enlarges with splenomegaly, but the life span of circulating platelets is not significantly changed. Furthermore, the splenic platelet pool is in dynamic equilibrium with the circulating platelet pool thus allowing these platelets to participate in hemostasis. Although posttransfusion increment in platelet count may be less, it appears that platelet transfusion therapy need not be altered solely because of splenomegaly.
Concentration‐dependent sedimentation properties of ferritin: Implications for estimation of iron contents of serum ferritins
Authors: Niitsu Yoshiro , Adachi Chikara , Takahashi Fumio , Goto Yoshiro , Kohgo Yutaka , Urushizaki Ichiro , Listowsky Irving 
Published: February 2007

AbstractSerum ferritins from various sources sedimented at lower densities than tissue ferritins in sucrose gradient centrifugation systems. The sedimentation patterns of ferritins, however, were shown to be dependent on the concentration of the protein; as the concentration decreased the protein appeared to sediment at lower densities. Thus, at the low concentration levels usually used for analysis of serum ferritin, tissue ferritins also sedimented in the same lower density regions. Iron labeling experiments indicated that the sedimentation changes upon dilution were not due to release of iron or was there any indication that the protein dissociated into subunits. The anomalous sedimentation behavior of serum ferritin should therefore not be interpreted in terms of its iron content. The disclosure that serum ferritins may have full complements of iron is counter to the prevalent view that serum ferritins are low iron forms and has potential implications with regard to the sources and possible function of this protein in the circulation.
High‐dose intravenous igg in the management of pregnancy in women with idiopathic thrombocytopenic purpura
Authors: Besa Emmanuel C. , Macnab Malcolm W. , Solan Andrew J. , Lapes Melvin J. , Marfatia Usha 
Published: February 2007

AbstractIdiopathic thrombocytopenic purpura (ITP) may develop during pregnancy or affect later pregnancies, causing serious risks of bleeding to the mother and fetus. High‐dose intravenous immunoglobulin (IGIV) has caused an immediate and predictable rise in platelet count during the infusion in both adults and children with chronic or acute ITP. The rapid rise in platelet counts may be important in preparing pregnant women with ITP for surgery or delivery. We report our experience in managing two women at weeks 29 and 37 week of gestation who required splenectomy and/or cesarean section. Both patients demonstrated an increase in platelet counts, underwent surgery without excess bleeding, and had normal infants with normal platelets, and with mild thrombocytopenia at delivery.
Platelet sensitivity to antiaggregatory prostaglandins (PGE1, D2, I2) in patients with peripheral vascular disease
Authors: Fitscha P. , Kaliman J. , Sinzinger H. 
Published: February 2007

AbstractPlatelet sensitivity to antiaggregatory prostaglandins (PGI2, PGE1, PGD2) was studied in 143 patients (122 male) with angiographically proven peripheral vascular disease and compared with age‐matched clinically normal controls. Patients had a significantly lower platelet sensitivity to PGI2, PGE1, and PGD2, than controls. Clinical stages had no significant influence on the platelet sensitivity to PGI2 and PGE1. Patients with stage IIa had a lower sensitivity to PGD2 than patients with stage IV, the difference not being significant. Analyzing the influence of risk factors like diabetes, hyperlipoproteinemia, or smoking, there seemed to be an inverse relation between risk factors and platelet sensitivity to PGI2 and PGE1. Smokers especially, together with smokers exhibiting an additional risk factor, exhibited the highest prostaglandin consumption (PGI2, PGE1) and therefore the lowest platelet sensitivity. However, it has to be emphasized that the differences were not significant.There was a significant correlation between platelet sensitivity to PGI2 and PGE1, whereas this was not the case between the respective sensitivities to PGI2, and PGD2. This supports the hypothesis that both these prostaglandins (PGI2, PGE1) share the same receptor on the platelet surface, whercas PGD2 has its own receptor.
Hematologic genetic disorders among Southeast Asian refugees
Authors: Monzon Carlos M. , Fairbanks Virgil F. , Burgert E. Omer , Sutherland John E. , Elliot Stephen C. 
Published: February 2007

AbstractResettlement of Southeast Asian refugees has introduced into the Western Hemisphere many persons of all major ethnic groups from Indochina. They represent several distinctive cultural, genetic, and linguistic groups, and the prevalence of genetic traits among them varies accordingly. We studied 778 Southeast Asian persons resettled in the upper Midwest who belonged to 182 unrelated families from the five major Southeast Asian ethnic groups. High prevalences of hemoglobin E, α‐ and β‐thalassemia disorders, and glucose‐6‐phosphate dehydrogenase deficiency were found. The prevalences of these four conditions in the refugees are among the highest known in the world. For these groups, iron deficiency is an uncommon cause of microcytosis; instead, the most frequent causes are hemoglobin E and α‐thalassemia‐1. Very serious thalassemic disorders occur with unusually high frequency in the refugees, especially in the Tai‐Dam.
Adenosine deaminase (ADA) in leukemia: Clinical value of plasma ADA activity and characterization of leukemic cell ADA
Authors: Morisaki Takayuki , Fujii Hisaichi , Miwa Shiro 
Published: February 2007

AbstractAdenosine deaminase (ADA) activity was measured in plasma, erythrocytes, and mononuclear cells from 18 patients with acute and chronic leukemia. High levels of ADA activities were found in plasma, erythrocytes, and mononuclear cells from patients with acute leukemia, especially acute lymphoblastic leukemia, and blastic crisis of chronic myeloid leukemia. Serial determination of plasma ADA activities was done in 9 patients with acute leukemia. All patients untreated or in relapse had an elevation of plasma ADA activity, which decreased to normal or subnormal levels during complete remission. On starch gel electrophoresis, plasma ADA in leukemic patients separated into two bands. The major band showed a mobility identical to that of normal red cells and mononuclear cells, and the minor band corresponded to that of normal plasma ADA. Enzymatic and immunological studies were performed on ADA from leukemic cells of acute myeloid and lymphoblastic leukemia. There were no differences in Michaelis constant for adenosine, thermostability, electrophoretic mobility, immunological reactivity, and specific activity between ADA of leukemic cells and normal mononuclear cells. These results strongly suggest that the increased ADA activity in leukemic cells is caused by an increased synthesis of a structurally normal enzyme and that increased plasma ADA activity in leukemic patients reflects an increment of leukemic cells in bone marrow. Therefore, serial determination of plasma ADA activities seems to provide a good indicator of the total mass of leukemic cells in bone marrow.
Defective complement activity in chronic lymphocytic leukemia
Authors: Heath Margo E. , Cheson Bruce D. 
Published: February 2007

AbstractPatients with chronic lymphocytic leukemia (CLL) are at an increased risk for infections with bacteria which require complement for osponization. We explored the possibility that patients with CLL have a defect in binding the potent opsonin C3b to bacteria. Bacteria selected for these experiments included Streptococcus pneumoniae type 3, which binds C3 by activating the classical complement pathway (CCP), type 25, which can bind normal amounts of C3b by the alternative complement pathway (ACP), type 14, which can activate both the CCP and ACP, and Staphylococcus aureus and Escherichia coli, both of which activate the CCP.Bacteria were treated with normal serum or serum from 15 patients with CLL, and the bound C3b was quantified spectrophotofluorometrically. Despite normal serum concentrations of C3, C4, Factor B, C‐reactive protein, and total hemolytic complement activity, all 15 CLL sera bound reduced amounts of C3b to at least one bacterial species; 9 to S pneumoniae type 3, 8 to types 14 and 25, 11 to S aureus, and 13 to E coli. Mixing normal serum with CLL serum restored C3b binding to all bacteria, suggesting a deficiency rather than an inhibitor of activity. Serum from ten hypogammaglobulinemic CLL patients bound less C3b (62.7 ± 5% of normal) (X̄ ± SEM) than those with normal immunoglobulin levels (81.9 ± 5%) (p < 0.005). Nevertheless, the addition of specific antibacterial antibodies to CLL serum did not enhance C3b binding to any of the bacteria. Serum from patients with a history of a bacterial infection bound less C3b (62.3 ± 5%) than those without a history of infections (76.1 ± 6%) (p < 0.05). Thus, there is a defect in either the activation or activity of C3 in CLL serum which may contribute to the increased incidence of infections in these patients.
Immune hemolytic anemia following administration of antithymocyte globulin
Authors: Prchal Josef T. , Huang Shu T. , Court Wayne S. , Poon Man‐Chiu 
Published: February 2007

AbstractAntithymocyte globulin is commonly used as a therapy for rejection of transplanted organs. Its use can be associated with many side effects. We report a significant hemolytic anemia following therapy with horse‐derived antithymocyte globulin. Several commercial Coombs' sera failed to yield a positive Coombs' test with the patient's erythrocytes; however, we were able to detect horse immunoglobulin on the patient's cells as well as on control red cells incubated with this particular preparation of antithymocyte globulin.
Identification of erythroid colony progenitors in a subset of human peripheral null lymphocytes devoid of Fc receptors
Authors: Rosenthal C. Julian , Hassan Mozaffar , Rieder Ronald F. , Brissette William , Parsa Ismail 
Published: February 2007

AbstractThis study was aimed at purifying the progenitors of erythroid burst units (BFU‐E) from human peripheral blood. Human mononuclear leukocytes from five normal donors were fractionated into several mononuclear cell subpopulations, including null lymphocytes with (null Fc+) and without (null Fc−) receptor for the Fc fragment of immunoglobulin G, through a succession of rosetting procedures and discontinuous Ficoll‐Hypaque gradient centrifugations. The fractionated cells were separately cultured for 14 days in plasma clots in the presence of erythropoietin. Among fractionated cell subpopulations large and numerous BFU‐E derived colonies grew only from the Fc− null lymphocyte subpopulation. This fraction, representing less than 4% of all mononuclear cells, also contains cells (42 + 11%) capable to differentiation towards the B‐cell and plasma‐cell lineages. The Fc+ null Imphocytes, representing less than 9% of all mononuclear cells, contained 15.2 + 3.3% cells capable of differentiation toward the T‐cell lineage. The whole null lymphocyte subpopulation generated half the number of BFU‐E colonies expected from its content in Fc− null lymphocytes. These data demonstrate that the progenitor of erythroid cells (BFU‐E) resides in a small heterogenous null Fc− subpopulation of circulating lymphocytes and suggest that its in vitro differentiation, though generally subjected to inhibitory and enhancing influences from other circulating cell subpopulations, does not necessarily require interaction with other peripheral blood cells.
Red cell superoxide dismutase and sickle cell anemia symptom severity
Authors: Schacter Lee P. , Delvillano Bert C. , Gordon Erlinda M. , Klein Bruce L. 
Published: February 2007

AbstractPatients with sickle cell anemia vary in the severity of their symptoms but the basis of this variability is unknown. We have tested the hypothesis that this variability is related to differences in the activity of the antioxidant superoxide dismutase (SOD). The amount of superoxide dismutase I (SOD) enzyme activity in red cells of patients with different degrees of symptom severity and healthy black and white controls was measured and correlated with symptom severity in SCA patients. Blacks with normal (AA) hemoglobin had significantly (p < 0.001) more SOD activity (1.82 U/mg Hb) than white controls (1.44 U/mg Hb). Patients with moderate or severe symptoms had less SOD activity (1.16 and 0.95 U/mg Hb, respectively) than control blacks or SCA patients with mild symptoms (1.62 U/mg Hb). The correlation of SOD activity and symptom severity was not a function of age or sex and was unrelated to reticulocyte count or fetal hemoglobin level.
Complications of heparin administration in normal individuals
Authors: Schwartz Kenneth A. , Royer George , Kautman Donald B. , Penner John A. 
Published: February 2007

AbstractThe incidence, severity, and pathogenic mechanism of heparin‐associated thrombocytopenia with both bovine and porcine heparin administration were studied in forty normal males randomized to one of four treatment groups: beef lung heparin #1, beef lung heparin #2, porcine gut heparin, and saline control. All of the subjects receiving heparin developed a reversible increase in serum transaminases (SGOT, SGPT). However, other measurements of liver function were normal. Thirty‐three percent of these heparinized normals had decreased platelet counts. The incidence of platelet count decrease was similar for both bovine and porcine heparins, but 4 of the 20 normals receiving bovine heparin had platelet counts less than 150,000/μl. Immune pathogenesis was investigated by analyzing plasma from the volunteers for both platelet antibody and immune complexes. None of the men had increased plateletassociated IgG. Among the ten subjects with decreased platelet counts, IgG immune complexes were detected in three and C1q in seven. The heparin‐associated thrombo‐cytopenia appears not to be mediated by a platelet antibody. More probably it reflects a direct effect of the heparin on platelets.
Plasminogen‐dependent fibrinolytic activity in normal human lymphocytes: Diminished lymphocyte plasminogen activator in chronic lymphocytic leukemia
Authors: Zucker Stanley , Mehling Karen , Rai Kanti 
Published: February 2007

AbstractDiscrepancies in correlations between fibrinolytic activity and metastatic potential of malignant cells has resulted in speculation on the putative role of plasminogen activators (PA) in cancer. In this report we have compared lymphocyte PA from 40 patients with chronic lymphocytic leukemia (CLL) to normal human B‐ and T‐ lymphocytes. Lymphocytes were isolated from peripheral blood by Ficoll‐Hypaque centrifugation. The B‐ and T‐cells were further separated on nylon wool columns. Cell PA activity and cell membrane PA were determined using 3H‐fibrin‐coated plates with added human plasminogen. Lymphocytes did not lyse 3H‐fibrin in the absence of plasminogen. Plasminogen‐dependent fibrinolytic activities of normal B‐ and T‐ lymphocytes were comparable. The addition of protease inhibitors with trypsin or plasmin specificity to lymphocytes significantly inhibited normal PA, thus substantiating the serine protease spectrum of lymphocyte PA. Examination of lymphocytes from > 95% of patients with chronic lymphocytic leukemia revealed a marked decrease in lymphocyte and cell membrane PA as compared to normals. No correlation between Stage of CLL and lymphocyte PA was observed. Likewise, an inhibitor of PA in CLL lymphocytes was not detected. The function of PA in normal B‐ lymphocyte physiology and the potential pathogenetic role of diminished PA in CLL lymphocytes remain to be explored.
Anti‐Pr cold hemagglutination associated with livedo reticularis
Authors: Rose Virgil L. , Kwaan Hau C. 
Published: February 2007

AbstractA report of anti‐Pr cold hemagglutination associated with livedo reticularis is presented. Livedo reticularis is descriptive of the superficial venous system appearing when blood flow is altered in this area. Cold antibody directed against the Pr antigen of the red cell membrane can cause hemagglutination, thereby altering blood flow in cooler areas of the body. This patient had livedo reticularis approximately 8 months prior to detection of the anti‐Pr cold agglutinin.
Salmonella infection in hairy cell leukemia: Report of a case
Authors: Tilly Hervé , Gardembas Martine , Berry Michel , Gray Christian , Monconduit Mathieu , Piguet Hubert 
Published: February 2007

AbstractA patient with hairy cell leukemia who developed mediastinal mass and fever is described. A CT‐guided aspiration of the mass yielded Salmonella tiphymurium. Granulocyte response to infection was intact and could explain the favorable course of this unusual Salmonella infection. Although several reports deal with salmonellosis as a complication of immunodeficiency states, this type of infection is of rare occurence in hairy cell leukemia.