bluuebunny/crossref_metadata_2025_split huggingface dataset:
Spontaneous disappearance of an IgA anti‐factor IX inhibitor in a child with christmas disease
Authors: Carroll Robert R. , Panush Richard S. , Kitchens Craig S. 
Published: February 2007

AbstractThe few inhibitors to blood coagulation factor IX in patients with Christmas disease (hemophilia B) that have been studied have been shown to belong to the IgG class of immunoglobulins. We report the first case in which a factor IX inhibitor was of the IgA class. Additionally, he appears to be the youngest patient with hemophilia B to have developed an inhibitor. His inhibitor complicated treatment of the patient for several years because of its anamnestic rise following factor IX concentrate administration. It then spontaneously vanished and has not returned in spite of repeated factor IX complex administration.
Treatment of polycythemia vera with hydroxyurea
Authors: Donovan Paul B. , Kaplan Manuel E. , Goldberg Judith D. , Tatarsky Ilana , Najean Yves , Silberstein Edward B. , Knospe William H. , Laszlo John , Mack Karen , Berk Paul D. , Wasserman Louis R. 
Published: February 2007

AbstractConventional treatment of polycythemia vera (PV) with radioactive phosphorus or alkylating agents is associated with a significant excess of acute leukemia and cancer of the gastrointestinal tract and skin. There is thus a need for a nonmutagenic agent in the treatment of this disorder. Hydroxyurea (HU) was administered to 118 patients with a loading dose of 30 mg/kg/day for 1 week, which was then reduced to 15 mg/kg/day. Initial control of the elevated hematocrit and platelet count was achieved within 12 weeks in over 80% of patients. Long‐term disease control was defined and the accumulative 1‐year failure‐free survival was 73% in the previously untreated patients and 59% in those patients previously treated with other myelosuppressive modalities. The HU was well tolerated and cytopenia, which generally occurred within the first 8 weeks of therapy, was transient and of little clinical significance. However, it is recommended because of this toxicity that HU be administered initially at a dose of 15‐20 mg/kg/day. Three patients developed acute leukemia; two were untreated and one had had myelosuppressive therapy. Hydroxyurea is an effective agent in the treatment of PV, but continued assessment of its mutagenic potential is necessary.
Differentiation of chronic lymphocytic leukemia cells after in vitro treatment with epstein‐barr virus or phorbol ester. I. Immunologic and morphologic studies
Authors: Deegan Michael J. , Maeda Koichi 
Published: February 2007

AbstractLeukemic cells from ten patients with “nonsecretory,” B‐type chronic lymphocytic leukemia (CLL) were cultured alone or in the presence of Epstein‐Barr virus (EBV) or a phorbol ester (12‐0‐tetradecanoylphorbol‐13‐acetate; TPA) for 7 days. At periodic intervals the cell morphology, cytoplasmic immunoglobulin content (direct immunofluorescence) and capacity for immunoglobulin secretion (hemolytic plaque assay) were assessed. A variable but significant number of the TPA‐treated CLL cells from all patients expressed cytoplasmic immunoglobulin of a single light‐chain type at some stage, usually within the first 3 days. EBV induced similar changes in seven of eight cases tested. Untreated cell cultures were negative or contained a few cytoplasmic immunoglobulin‐positive cells. Cells from five of nine cases secreted immunoglobulin of a single light‐chain type. In every instance this was identical to the surface and cytoplasmic immunoglobulin. Cytologic changes were observed in the leukemic cells after treatment with one or both agents in nine of ten cases. The major feature was an increase in cell size associated with immunoblastic or plasmacytoid features. Mitotic figures and binucleate cells were also present. These studies indicate that EBV and TPA are effective at inducing immunoglobulin synthesis and secretion in “nonsecretory” B cell neoplasms and are useful tools for studying the maturation potential inherent in these tumors. The study also suggests that the secreted immunoglobulin is a monoclonal product.
Heparin‐induced thrombocytopenia: A prospective study
Authors: Johnson Robert A. , Lazarus Kenneth H. , Henry David H. 
Published: February 2007

AbstractThrombocytopenia is a well‐described complication of heparin therapy. Few studies describe the incidence of thrombocytopenia when low‐dose heparin (10,000–15,000 units/day) is used for prophylaxis of deep venous thrombosis. In our study, ten of 66 courses (15%) of heparin prophylaxis in coronary care unit patients were accompanied by a mild thrombocytopenia with platelet counts below 150 ± 103/mm3. In all cases the platelet count returned to normal despite continued heparin therapy. Patients who became thrombocytopenic had significantly lower initial platelet counts. No cases of severe thrombocytopenia were seen (platelet count below 100 ± 103/mm3). No patient developed thrombosis, bleeding or elevated fibrin split products. Mild thrombocytopenia occuring after 2–5 days of low‐dose heparin is common, but clinically insignificant.
Pharmacokinetics and tolerance of intravenous and intramuscular recombinant alpha2 interferon in patients with malignancies
Authors: Shah I. , Band J. , Samson M. , Young J. , Robinson R. , Bailey B. , Lerner A. Martin , Prasad A. S. 
Published: February 2007

AbstractFifteen patients with advanced hematopoietic and other malignancies were treated with recombinant DNA produced Alpha2 Interferon (IFN) (Schering) by intravenous (IV) and intramuscular (IM) routes at weekly intervals in escalating doses from 1 × 106 IU to 100 × 106 IU in order to determine the tolerance and pharmacokinetics. The most common side effects included fever, chills, myalgia, and arthralgia. At doses of 60 × 106 or above, severe but reversible hypotension was observed in five patients receiving interferon by intravenous route. Patients receiving interferon by intramuscular route had fever, chills, and myalgias but did not develop hypotension at the same dosage. Two patients with non‐Hodgkin lymphoma showed objective evidence of regression. Our data suggest a biphasic pattern of elimination with terminal half‐life ranging from 1.9 to 2.9 hours and peak titer of 16,000 units and under for IV interferon, and terminal half‐life of 6 hours with peak titers of 600 units for intramuscular interferon. However, interpatient variability precludes a definite conclusion.Although the areas under the serum concentration vs time curves were similar, the intravenous route provided higher but unsustained levels of interferon than the intramuscular route.
Preparation of low‐affinity red cells with dimethylsulfoxide‐mediated inositol hexaphosphate incorporation: Hemoglobin and ATP recovery using a continuous‐flow method
Authors: Franco R. S. , Wagner K. , Weiner M. , Martelo O. J. 
Published: February 2007

AbstractIncorporation of IHP into red cells decreases oxygen affinity as a result of the binding of this compound to the 2,3‐DPG site of hemoglobin. This investigation describes a continuous‐flow method which utilizes the osmotic pulse technique to transport IHP into RBC. Using this procedure, it is possible to obtain a significant increase in P50 while maintaining in vitro cellular integrity. For example. IHP incorporation sufficient to cause an increase in the P50 of 20 mm Hg may be achieved with recovery of approximately 75% of the hemoglobin and with maintenance of ATP levels compatible with good viability. The continuous‐flow method allows uniform treatment of large, unit‐size volumes of red cells with a relatively small quantity of reagents. The final cell product is macrocytic/hypochromic with an increased number of stomatocytes.
Thrombotic thrombocytopenic purpura: Clinical course and response to therapy in eight patients
Authors: Evans Terry L. , Winkelstein Alan , Zeigler Zella R. , Shadduck Richard K. , Mangan Kenneth F. 
Published: February 2007

AbstractThe clinical manifestations, pathologic findings, and responses to therapy were reviewed in eight patients with thrombotic thrombocytopenia purpura (TTP). Only one exhibited all five cardinal manifestations; five showed a triad of anemia, thrombocytopenia, and neurologic abnormalities. Microangiopathic red cell changes on peripheral blood smear and severe thrombocytopenia were present in all. The serum LDH levels were initially elevated in all eight; this enzyme determination was extremely useful for following the course of the disease and its response to therapy. Pathologic evidence of TTP was most consistently found in lymph nodes, spleen, and bone marrow biopsies. All patients were treated with a combination of therapeutic modalities including plasma exchange with replacement by fresh frozen plasma. Using this approach, 7/8 entered a complete remission; however, disappearance of all clinical manifestations was not seen in two patients prior to splenectomy.
Membrane expansion as a mechanism explaining the antisickling action of ticlopidine observed in vitro
Authors: Sablayrolles Magali , Wajcman Henri , Castaigne Jena‐Paul , Labie Dominique 
Published: February 2007

AbstractTiclopidine, a platelet antiaggregant, has shown some efficacity in a clinical trial in patients with sickle cell disease. We have studied this agent in vitro to evaluate its effects on sickle erythrocyte. Ticlopidine effects sickling in vitro not by direct interaction with hemoglobin, but via strong binding to the red cell membrane. The density of the whole cell population is decreased when cells are treated with 0.1 mM ticlopidine, which is higher than the concentrations of 1 μM potentially achievable in vivo. Since hemoglobin concentration influences the delay time for gelling, its decrease in the red cell could have a beneficial effect. Such a partial inhibition of the polymerization is shown by oxygen equilibrium studies at various ionic strengths.
Changes in cellular ferritin content during myeloid differentiation of human leukemic cell lines
Authors: Fibach Eitan , Konijn Abraham M. , Rachmilewitz Eliezer A. 
Published: February 2007

AbstractThe human promyelocytic cell lines HL‐60 can be induced to undergo differentiation to either granulocyte‐ or macrophagelike cells. We followed the changes in the synthesis and content of ferritin in this and other cell lines during differentiation. Ferritin content of HL‐60 cells ranged from 11 to 81 fg/cell, depending on the clone tested. Following exposure to dimethylsulfoxide (DMSO) or retinoic acid (RA) an increase in ferritin and a decrease in total protein synthesis was observed, resulting in increased ferritin content, reaching a peak after 2 days. This increase occurred prior to the appearance of the typical morphological and functional characteristics of mature granulocytes. A correlation was found between concentrations of DMSO effective in inducing differentiation and the increase in ferritin content. Other inducers of granulocyte differentiation had a similar effect, while 12‐0‐tetradecanoylphorbol‐13‐acetate (TPA), an inducer of macrophage differentiation, had not. Another human cell line (U‐937), which was induced into monocytelike cells by RA, showed a twofold increase in ferritin content following differentiation. Addition of iron to the culture medium increased ferritin content of both differentiating and nondifferentiating cells, but the former responded to lower concentrations of iron. The increase in ferritin during differentiation, however, was not related to an accelerated iron uptake. The present results suggest that changes in the intracellular ferritin of the developing myeloid cells may play a regulating role in the process of maturation of these cells.
Evidence for two isozymes of leukocyte alkaline phosphatase in leukemic leukocytes
Authors: Miller Donald M. , Yang Angela , Liepman Marcia 
Published: February 2007

AbstractLeukocyte alkaline phosphatase (LAP) is a granulocyte enzyme whose level of expression is markedly altered in various disease states. We have characterized LAP from normal cells and leukemic cells with a high level of LAP activity in order to determine whether increased enzyme levels are caused by increased levels of the same enzyme or induction of a different alkaline phosphatase.Leukocyte alkaline phosphatase was purified from normal granulocytes and from leukemic cells of a patient with chronic granulocytic leukemia (CGL) in blast phase with an elevated LAP level. LAP was partially purified utilizing diethylaminoethyl (DEAE)‐cellulose ion exchange chromatography, gel filtration, and preparative electrophoresis.The sample prepared from normal granulocytes contained a single protein with LAP activity having a molecular weight of 61,000 as determined by SDS gel electrophoresis. The sample from the CGL blast‐phase cells, however, demonstrated two proteins with alkaline phosphatase activity, one with a molecular weight of 61,000 (LAPs) and one with a molecular weight of 45,000 (LAPf). Differential heat inactivation and distinct isoelectric points of the two isozymes suggest that they are different proteins.We interpret our data to suggest two closely related LAP alleles whose expression is controlled independently. This may represent either genetic heterogeneity or induction of “tumor marker” gene expression.
Spurious thrombocytopenia produced by the interaction of rheumatoid factor with antiplatelet antibody
Authors: Poskitt Thomas R. , Poskitt Paula K. F. 
Published: February 2007

AbstractA patient had spurious thrombocytopenia resulting from a mechanism not previously described. Whereas in prior reports the in vitro phenomenon of platelet clumping has been effected by either EDTA‐dependent or temperature‐dependent antibodies capable of direct platelet agglutination, neither the IgG nor the IgM fractions of this patient's serum demonstrated such activity. However, agglutination was produced by incubating allogeneic platelets with the IgG fraction followed by a room temperature incubation with the rheumatoid factor‐positive IgM fraction. The data support a new mechanism for spurious thrombocytopenia resulting from the interaction of a cold‐reactive rheumatoid factor with antiplatelet antibody.
Platelet activity in mitral valve prolapse: A study of platelet aggregation, malondialdehyde production, and plasma β‐thromboglobulin
Authors: Arocha Francisco , Diez‐Ewald Maria , Durango Ana I. , Sulbarán Tulio 
Published: February 2007

AbstractPlatelet aggregation, malondialdehyde (MDA) production, and recovery after aspirin (ASA) administration and plasma levels of beta‐thromboglobulin (BTG) were determined in 40 asymptomatic patients with mitral valve prolapse (MVP) and 17 normal subjects. Platelet aggregation was similar in patients and controls, although two patients presented spontaneous aggregation. Production of MDA and plasma levels of BTG were higher in MVP than in controls; however, recovery after ASA was similar in both groups. The results further indicate that platelet hyperactivity is present in a significant number of asymptomatic patients with MVP.
A marked increase of calcium uptake in the ATP‐depleted red cells of patients with iron deficiency
Authors: Shimoda Masaoo , Yawata Yoshihito 
Published: February 2007

AbstractCalcium (Ca) uptake was markedly increased in ATP‐depleted red cells of patients with iron deficiency anemia (IDA) compared to ATP‐depleted normal red cells. The extent of increased Ca uptake was related to the severity of iron deficiency as judged by decreased mean cell volume. Moreover, the increased Ca uptake returned to normal levels after oral iron supplementation therapy. The net calcium content of fresh red cells from iron‐deficient individuals was the same as in red cells from normal subjects. Sodium influx and ferric ion uptake appeared to be virtually unaffected in the iron deficient red cells.
Low molecular weight iron in guinea pig reticulocytes
Authors: Pollack Simeon , Campana Theresa , Weaver Janet 
Published: February 2007

AbstractThe low molecular weight iron found in the guinea pig reticulocyte has been partially characterized. On thin layer chromatography it is distinguishable from the iron complexes of a variety of nucleotides, sugars, and amino acids. On paper chromatography it comigrates with a 250‐nm absorbing, orcinol‐positive material. The eluted count peak contains phosphorus. Approximately 1 μg of iron is recovered from 1 ml of hemolyzed red cells. Preparation under nitrogen improves recovery of low molecular weight iron, suggesting that the iron is in the ferrous oxidation state.
The procoagulant effect of zinc on fibrin clot formation
Authors: Marx Gerard , Eldor Amiram 
Published: February 2007

AbstractThe influence of Zn+2 on fibrin clot formation was investigated by measuring its effect on the clotting times of fibrinogen exposed to thrombin. It was observed with either human or bovine thrombin that 0.01‐0.1 mM Zn+2 induced significant reductions of clotting times in a concentration‐dependent manner. The procoagulant effect of Zn+2 occurred in the presence of Ca+2 but was inhibited by metal chelating agents. Higher levels of Zn+2 (> 0.2 mM final concentration) were required to accelerate thrombin‐induced clot formation in the presence of citrate or oxalate. Similarly with oxalated human plasma, > 0.2 mM Zn+2 decreased the clotting time. Cations such as Mg+2 and Mn+2 caused little change in clotting times. As an extension of these findings, we examined the effect of Zn+2 on the in hibition of thrombin by antithrombin‐III (AT‐III). The presence of as little as 0.006 mM Zn+2 in an incubating mixture of thrombin and AT‐III severely reduced the inhibitory activity of AT‐III towards thrombin. It was observed that the relative intrinsic fluorescence emission of human thrombin decreased upon exposure to Zn+2 but was unaffected by Mg+2 or Mn+2. It is suggested that Zn+2 can form a complex with thrombin, which results in altered reactivity towards fibrinogen and decreased inhibition by AT‐III.
Evidence for rapid rapid mobilization of platelets from the spleen during intensive plateletpheresis
Authors: Lee Edward J. , Schiffer Charles A. 
Published: February 2007

AbstractThe role of the spleen as a reservoir capable of adding platelets to the circulating blood cells has been well documented in animals but poorly documented in humans. As part of a program of platelet cryopreservation, we have intensively plateletpheresed a group of patients with acute leukemia in remission who had undergone splenectomy. The results of the 64 plateletphereses in these 11 patients (average platelet yield 4.2 × 1011 ± 2.4, range 1.2‐12.4) were compared with 50 consecutive plateletphereses in nonsplenectomized leukemia patients (average yield 4.9 × 1011 ± 3.2, range 2.1‐18.3) and 50 consecutive plateletphereses in normal donors (average yield 3.8 × 1011 ± 1.4, range 1.7‐7.6). There was no difference in the prepheresis platelet counts among the three groups. Plateletphereses were done in 1.5‐3 hr by intermittent flow differential cell centrifugation technique. The expected platelet yield was calculated by multiplying the pre‐platelet count –(pre‐hct/post‐hct × post plt count) by the estimated blood volume (70 ml/per kg body weight). The mean ratios of the observed platelet yield to expected platelet yield were similar for the nonsplenectomized leukemia patients (mean ratio = 1.32 ± 0.50, range 0.73‐3.04) and normal donors (mean ratio = 1.31 ± 0.49, range 0.52‐2.9), implying mobilization of platelets from outside of the blood pool. In contrast, in the splenectomy group, the mean ratio (0.76 ± 0.32, range 0.31‐2.9) was significantly lower (p < 0.001). These data indicate that there is replenishment of the circulating platelet pool by the spleen in response to the rapid removal of platelets.
Treatment of patients with acute nonlymphocytic leukemia in relapse: A leukemia intergroup study
Authors: Goldberg J. , Grunwald H. , Vogler W. R. , Winton E. , Browman G. , Walker I. , Benger A. , Miller K. B. , Rai K. , Gottlieb A. J. , Brennan J. K. , Bennett J. M. , Chervenick P. , Joyce R. , Azarnia N. , Priore R. L. , Preisler H. D. 
Published: February 2007

AbstractForty patients with acute nonlymphocytic leukemia (ANLL) in first relapse were treated at eight member institutions of the Leukemia Intergroup with a 10‐day continuous intravenous infusion of cytosine arabinoside and an anthracycline antibiotic administered on days 1, 2, and 3. Twenty of the 40 patients achieved a complete response. Seven of the patients who did not enter remission were drug‐resistant failures, while 13 patients failed to enter remission for reasons other than persistent leukemia. Pretreatment parameters such as age, presence of infection, platelet count, and liver function tests were important predictors of survival. The percent bone marrow cellularity, the percent circulating abnormal (leukemic) cells, and the height of the white blood cell count prior to treatment were helpful in distinguishing patients who would enter remission from those who would not enter remission because of persistent leukemia.
Recurring thromboembolic disease and pulmonary hypertension associated with severe hypoplasminogenemia
Authors: Lottenberg Richard , Dolly F. Ray , Kitchens Craig S. 
Published: February 2007

AbstractIn a patient with pulmonary hypertension and a history of recurrent venous thrombosis, plasma concentrations of all known coagulant and inhibitor proteins were normal except for severe deficiency of plasminogen. Repeated analyses showed the circulating plasma plasminogen level to be 30% of normal by either functional or immunologic methods. We sought evidence for either increased activation of plasminogen or for dysplasminogen. There was no evidence for the former. Purified plasminogen studies disclosed a normal number of active sites and normal activation. Generated plasmin had normal catalytic activity. Isoelectric focusing disclosed normal distribution of isoforms. Affinity chromatography with lysine‐sepharose showed the presence of the two variant forms; however, an increased proportion of the protein eluted in the first peak. Danazol administration induced an increase in circulating plasminogen, but the differences in affinity chromatography elution profile remained. We conclude that this patient has a deficiency of normally functioning plasminogen, probably due to decreased synthesis.
Perfluorocarbon compounds: Effects on the rheological properties of sickle erythrocytes in vitro
Authors: Reindorf C. A. , Kurantsin‐Mills J. , Allotey J. B. , Castro O. 
Published: February 2007

AbstractThe effects of oxygenated perfluorotributylamine (Fluosol‐43) on the rheological properties of sickle (HbSS) erythrocytes have been determined by means of microviscometry and positive pressure cell filtration. Incubation of deoxygenated sickled erythrocytes (pO2 = 30 mmHg) with oxygenated Fluosol‐43 reduced the percentage of sickled erythrocytes from about 63 to 33%. Deoxygenation of 40% suspension of sickle erythrocytes in autologous plasma increased the viscosity by about 160% at shear rate of 1.15 sec−1. Incubation of the deoxygenated sickled erythrocytes with oxygenated Fluosol‐43 significantly reduced the viscosity at the low shear rates. Filtration of 0.2% suspension of deoxygenated sickle erythrocytes through capillarysized Nuclepore filters showed high resistance at low flow rates. Oxygenated Fluosol‐43 increased the deformability of HbSS erythrocytes and thereby reduced the resistance at flow rates less than 1 ml/min. These data suggest that perfluorocarbons may be useful in reducing the propensity of hemoglobin S polymeriztion and sickling and thereby prevent tissue infarction in vaso‐occlusive crisis. Therefore, the concept of examining the potential application of perfluorochemicals for alleviating severe vasoocclusive events may be useful.