bluuebunny/crossref_metadata_2025_split huggingface dataset:
Interaction of protamine sulfate with thrombin
Authors: Cobel‐Geard Richard J. , Hassouna Houria I. 
Published: February 2007

AbstractProtamine sulfate (salmine), a basic protein with a molecular weight of 4,626 ± 109, is a known antiheparin agent which in the absence of heparin demonstrates an anticoagulant activity. To date, much work has been done to elucidate the interaction of heparin with thrombin and its physiologic inhibitor, Antithrombin III (ATIII). Little is known, however, about the mechanism of anticoagulant action of protamine sulfate and its mode of thrombin inactivation. We provide information about the interaction of protamine sulfate with purified, labeled thrombin and ATIII through binding experiments in which protamine is shown to inhibit the inactivation of thrombin by ATIII. Furthermore, we show in clotting assays that protamine sulfate has an inhibitory effect on thrombin in the conversion of fibrinogen to fibrin, and that this inhibition is concentration dependent, partial, and reversible.
Abnormalities of the spleen and liver in patients with hemophilia
Authors: Meyer William H. , Levin Jack , Ness Paul M. , Bell William R. , Douglass Kenneth H. , Camargo Edwaldo E. , Zinkham William H. 
Published: February 2007

AbstractPrevious studies have demonstrated functional and histologic abnormalities of the liver, and, more recently, splenomegaly in patients with hemophilia. Since these observations usually were derived from hemophiliacs who had received intensive replacement therapy, the question was posed as to whether the frequency of splenic and hepatic abnormalities was secondary to the amount of therapy utilized. In this study, a variety of tests were employed to evaluate spleen and liver size and function to determine if abnormalities in these organs correlated with the intensity of the transfusion program. The study group was comprised of 25 hemophiliacs (mean factor replacement—18,361 U/year; median factor replacement—12,920 U/year). Over 70% of our patients had elevations of aspartate and alanine aminotransferase. Immunoglobulin and complement levels were normal in most subjects. Ninety‐six percent had evidence of exposure to hepatitis B virus. Liver‐spleen imaging suggested significant hepatic abnormalities in most of the patients as evidenced by inhomogeneity of tracer uptake in the liver in 33% and relatively increased colloid uptake in the spleen in 90%. Splenomegaly (palpable spleen or enlargement on liver‐spleen imaging) was detected in 40% of our patients, and tended to occur in the more frequently transfused patients. These findings indicate that significant abnormalities of the spleen and liver can occur in hemophiliacs who have received moderate amounts of replacement therapy and that liver‐spleen imaging may be a useful method for monitoring the development of hepatic and splenic abnormalities.
Transient erythroblastopenia of childhood: Varied pathogenesis
Authors: Freedman Melvin H. , Saunders E. Fred 
Published: February 2007

AbstractThe mechanism of anemia in four patients with transient red cell aplasia of childhood (“erythroblastopenia”) was studied at the time of diagnosis by assessing the colony growth of marrow erythroid progenitors in methylcellulose tissue cultures. Marrow from Patient 1 yielded high normal numbers of BFU‐E colonies that were completely abolished on addition of autologous serum or IgG. Patient 2 had normal BFU‐E growth that markedly declined when autologous serum or IgM was added to the cultures, but growth remained unchanged with added autologous IgG or peripheral blood mononuclear cells (PBMC). Marrow from Patient 3 yielded low CFU‐E and BFU‐E numbers with standard plating techniques, but colonies strikingly increased when marrow fractions from an albumin density gradient were cultured. PBMC from Patient 3 suppressed control marrow CFU‐E and BFU‐E, but serum had no effect. Patient 4 had normal CFU‐E and BFU‐E that increased with autologous serum and remained unchanged with autologous PBMC. We conclude that the red cell aplasia in Patients 1, 2, and 3 was due to suppressed erythropoiesis via IgG, IgM, and cell‐mediated inhibition, respectively. In contrast, in Patient 4 no immune mechanism was demonstrated. Whereas transient red cell aplasia has a uniform clinical presentation, there are at least four pathogenetic mechanisms that can be detected in vitro.
Migration of human leukocytes from soft agarose droplet: A simplified method for studying chemotaxis and spontaneous migration
Authors: Barak Yigal , Karov Yocheved , Levin Stanley 
Published: February 2007

AbstractStudies of in vitro chemotaxis and spontaneous migration of human leukocytes using the accepted method with the Boyden‐chamber‐filter are troublesome, because of the need for specially constructed vessels as well as the difficulties caused by the use of membrane filters. We describe a new and simplified method for measuring human leucocyte chemotaxis, which is a modification of the recently described underagarose migration method and which is based upon spontaneous migration of cells from a soft agarose droplet and in response to a chemotactic gradient. We examined suspensions of leukocytes, purified granulocytes, and mononuclear cells from 10 healthy normal adults and from 10 samples of cord blood using E Coli O111B4 endotoxin‐activated human serum as attractant. Our results showed that the mean chemotactic indices (C.I.‐chemotaxis/migration) for purified granulocytes and for mononuclear cells from normal individuals were 3.0 ± 1.2 and 2.7 ± 1.5, respectively. Chemotaxis was significantly reduced when unwashed leukocytes were studied, indicating a detrimental effect of autologous plasma on leukocytic response to a chemotactic stimulus in this system. Cord blood cells showed normal spontaneous migration, but significantly decreased chemotaxis. This preliminary report shows that the technique is simple, rapid, and reproducible, and can detect abnormalities of chemotaxis in both granulocytes and mononuclear cells.
Red cell adenylate kinase deficiency associated with hereditary nonspherocytic hemolytic anemia: Clinical and biochemical studies
Authors: Miwa Shiro , Fujii Hisaichi , Tani Kenzaburo , Takahashi Keisuke , Takizawa Takenori , Igarashi Takashi 
Published: February 2007

AbstractWe report here a case of red cell adenylate kinase (AK) deficiency associated with hereditary hemolytic anemia. The proband is a 10‐year‐old Japanese girl. Her physical and mental development was normal. She has shown moderate to mild hemolytic anemia since the neonatal period and hepatosplenomegaly. The red cell AK activity was 44% of normal. Contents of red cell glycolytic intermediates and adenine nucleotides were normal when compared with a comparable reticulocyte‐rich control. Glucose consumption and lactate formation were normal. Hexose monophosphate shunt activity was somewhat lower than that of a comparable reticulocyte‐rich control. There were no significant differences in the contents of adenine nucleotides between the younger and older red cells of the patient. Enzymatic characterization by hemolysate revealed that the patient's AK had an increased Michaelis constant for adenosine diphosphate and slight thermal instability. The patient's enzyme migrated approximately half‐way between the AK 1 and AK 2 position on starch‐gel electrophoresis. The mode of inheritance of this case is obscure. The mechanism of hemolysis might be a structural gene mutation that caused altered electrophoretic and kinetic properties.
Mechanism of defibrination in humans after envenomation by the eastern diamondback rattlesnake
Authors: Kitchens Craig S. , van Mierop Lodewyk H. S. 
Published: February 2007

AbstractWe prospectively studied the hemostatic system of ten persons bitten by the Eastern diamondback rattlesnake (Crotalus adamanteus) during 1978–1980. Blood was drawn when the patients arrived in the emergency room and every 6 hr thereafter. All envenomated victims developed incoagulable blood (defined by a thrombin time ≥ 120 sec, normal < 20 sec). Platelet counts and plasma levels of antithrombin III and factors II and VIII were not drastically altered, which distinguished this disorder from classic disseminated intravascular coagulation. Fibrinogen levels were markedly decreased (mean coagulable level of 0 mg/dl and antigenic levels of 99 mg/dl). Plasminogen levels were 20% of normal, alpha‐2‐plasminogen inhibitor was 17% of normal, and plasminogen activator was 20 times normal. Levels of fibrin degradation products peaked at a mean of 7,680 μg/ml. The magnitude and duration of the coagulopathy were proportional to the clinical severity of envenomation. Treatment with antivenin blunted the coagulopathy. Because venom from the Eastern diamond‐back rattlesnake does not directly activate plasminogen, we conclude that coagulopathy following envenomation by that reptile appears to be due to partial proteolysis of fibrinogen with secondary activation of plasminogen by released plasminogen activator, probably of endothelial orgin.
Hereditary disorders of red cell enzymes in the embden‐meyerhof pathway
Authors: Miwa Shiro 
Published: February 2007

AbstractRecent advances about hereditary disorders of red cell enzymes in the Embden‐Meyerhof glycolytic pathway and Rapoport‐Luebering cycle are discussed with a stress on pyruvate kinase deficiency, because it is the most common and most intensively studied disorder among them. Broad genetic heterogeneity exists in all the known erythroenzymopathies. Recently, the primary structure of normal human red cell phosphoglycerate kinase has been determined and single amino acid substitutions of four mutant phosphoglycerate kinases have been clarified by Yoshida et al. These studies allowed analysis of structure‐function relationships at the molecular level to be carried out more precisely than was previously possible. It is the consensus of the investigators working in this field that the pathogenesis in three‐quarters of the congenital nonspherocytic hemolytic anemia patients remains unknown even after adequate red cell enzyme studies and isopropanol test for unstable hemoglobin have been done. This simply means that much studies remain to be worked out in this field.
Studies on abnormal polymorphonuclear neutrophils in acute myelogenous leukemia: Clinical significance and changes after chemotherapy
Authors: Suda T. , Onai T. , Maekawa T. 
Published: February 2007

AbstractIn patients with acute myelogenous leukemia (AML), 34 out of 35 patients (97%) had cytochemically abnormal polymorphonuclear neutrophils (PMN). Some interesting regularity was noted in the appearance of cytochemical abnormalities of the PMNs. When peroxidase (PO) activity was low, both alkaline phosphatase (AP) and chloroacetate esterase (CAE) activities were also low (group A). When PO activity was normal, if AP activity was low, CAE activity tended to be high (group B) and if AP activity was high, CAE activity tended to be low (group C). Complete remission rate of group B (65%) seemed to be better than that of group C (20%) and group A (25%) although the differences was not statistically significant. In these patients, the number of peripheral leukemic blasts and PMNs showed a significant positive correlation (r = 0.69, p < 0.005). Furthermore, patients with higher circulating PMN counts (more than 2 × 109/L) had a significantly worse prognosis than patients with lower counts (p < 0.05). These rather unexpected findings strongly suggest that cytochemically abnormal PMNs are progeny of AML blasts. The change in the proportion of abnormal PMNs associated with chemotherapy were studied in 21 patientas during the remission induction period. Abnormal PMNs disappeared from the circulation in patients who achieved complete remission. In contrast, abnormal PMNs persisted throughout the remission induction period in patients who failed to achieve complete remission.
Serum‐free light chain analysis by crossed immunoelectrophoresis: Correlation with plasmapheresis in light chain disease nephropathy
Authors: McLeod Bruce C. , Viernes Ann L. , Sassetti Richard J. 
Published: February 2007

AbstractThe technique of crossed immunoelectrophoreis (X‐IEP) has been used to quantify free monoclonal light chains (LC) directly in the serum of patients with light chain disease, without preliminary gel or membrane filtration of serum to separate whole immunoglobulin. LC concentration is proportional to the area under an immunoprecipitin peak formed by LC in the patient's serum and an anti‐LC antibody of appropriate specificity. Light chains of beta electrophoretic mobility can be processed in the standard X‐IEP technique at pH 8.6. Light chains of gamma mobility must be processed in a modified technique using a pH of 5.0 and a carbamylated antiserum in the second dimension gel. Dose response curves obtained from the method in serial dilution experiments with sera from 18 patients gave correlation coefficients ± 0.99. Replicate measurements of absolute LC concentration on the same specimens were within ± 10%. The method can also detect polymerized light chains. Serum light chain levels were measured during the course of plasmapheresis therapy in three patients with light chain disease and renal failure. Light chain levels were shown to fall after plasmapheresis and to rise rather rapidly in the interval between treatments.
Waldenström macroglobulinemia terminating in acute leukemia: A report of three cases
Authors: Horsman Douglas E. , Card Robert T. , Skinnider Leo F. 
Published: February 2007

AbstractAcute leukemia was observed to develop in three patients with Waldenstöm marcroglobulinemia. Each patient had been treated with cytotoxic drug therapy. Pancytopenia preceded the onset of the terminal leukemia in two of the three cases. The acute leukemias were all of acute myelogenous type. All of the patients died soon after the development of the terminal leukemia. A number of recent reports have documented acute terminal leukemia in patients with macroglobulinemias and multiple myeloma, as well as other malignant and nonmalignant diseases. The role of the cytotoxic drugs, especially chlorambucil, cyclophosphamide, and melhalan, in leukemogenesis has been raised by these recent reports.
Heinz‐body anemia: “Bite Cell” variant— A light and electron microscopic study
Authors: Ward Patrick C. J. , Schwartz Burton S. , White James G. 
Published: February 2007

AbstractLight and scanning electron microscopic studies of blood from five patients with drug‐induced oxidant hemolysis are presented. None of the patients had a previous history of hemolytic disease and laboratory studies indicated no evidence of either glucose‐6‐phosphate dehydrogenase (G6PD) deficiency or unstable hemoglobinopathy. Although the red cell deformities in our patients overlapped to some extent with those reported in patients with microangiopathic hemolytic anemia (MAHA) and in patients with G6PD deficiency undergoing oxidant hemolysis, striking differences were also observed. Cell fragments, commonly found in patients with MAHA, and eccentrocytes, frequently found in patients with G6PD deficiency undergoing oxidant hemolysis, were seldom found in blood samples from the five patients in this study. Bite cells were extremely common in our patients. They are rare in patients with either of the above disorders. An awareness of the morphologic abnormalities detailed in this report may help characterize the nature of a hemolytic process so that appropriate therapy can be initiated.
Leukocyte alkaline phosphatase: Another organ‐specific alkaline phosphatase
Authors: Miller Donald M. , Yang Angela , Liepman Marcia 
Published: February 2007

AbstractWe have used enzyme specific inhibitors and heat inactivation to distinguish Leukocyte alkaline phosphate (LAP) from other organ‐specific alkaline phosphatases as well as to compare LAP from normal granulocytes and leukemic cells with elevated LAP. The heat inactivation and inhibition curves of LAP are quite different from those of other organ‐specific alkaline phosphatases. The inhibition curves and heat inactivation characteristics of LAP from normal granulocytes and that obtained from chronic granulocytic leukemia (CGL) blast phase cells with elevated LAP are identical. These data suggest that LAP is distinct from other organ‐specific alkaline phosphatases, particularly placental alkaline phosphatase. We also conclude that the LAP present in cells with elevated levels is very similar or identical to that of normal granulocytes.
Splenectomy for thrombocytopenia in chronic lymphocytic leukemia
Authors: Merl Stuart A. , Theodorakis Michael E. , Goldberg Jack , Gottlieb Arlan J. 
Published: February 2007

AbstractThe role of peripheral platelet destruction as a reversible etiology of thrombocytopenia in chronic lymphocytic leukemia (CLL) was evaluated in nine patients with CLL and refractory thrombocytopenia who underwent splenectomy. The patients' ages ranged from 54 to 74 years. Progressive thrombocytopenia refractory to antineoplastic agents and corticosteroids had been present for a mean of 23.4 months. The platelet counts were 4,000–57,000/μl, and were generally higher in those patients with larger spleens. The spleens ranged from 180 to 4050 gm.Seven patients responded completely to splenectomy, achieving platelet counts > 150,000/μl, and in one other patient, the count rose to > 100,000/μl. The platelet count of one patient failed to respond to surgery. Those patients with massive splenomegaly developed higher, more rapidly rising platelet counts postoperatively. No operative mortality was encountered. Median hospitalization was seven postoperative days. All patients experienced an increased sense of well‐being. Median follow‐up time is 9 months.
Acquired partial deletions of the long arm of chromosome 5 in hematologic disorders
Authors: Wisniewski Lawrence P. , Hirschhorn Kurt 
Published: February 2007

AbstractWe have analyzed the data on 105 patients reported with a deletion of part of the long arm of chromosome 5 in the presence of hematologic disease. The major conditions associated with this abnormality are refractory anemia, polycythemia vera, and acute myelogenous leukemia, as well as the occasional occurrence of several other problems.
Postleukemic dysmyelopoiesis
Authors: Foucar Kathy , Vaughan William P. , Armitage James O. , Patil Shivanand , Dick Fred , Karp Judith E. 
Published: February 2007

AbstractThe morphologic and clinical features of four patients who developed significant bone marrow and blood dyspoiesis after successful chemotherapy for acute nonlymphocytic leukemia (ANLL) are described. This postleukemic dyspoiesis developed 1‐6 months after leukemia induction therapy and persisted for 5‐20 months in a relatively stable state. This period of prolonged dyspoiesis was not associated with rising myeloblast counts or clinical evidence of relapse. Dyspoietic abnormalities developed while two patients were receiving maintenance chemotherapy; the other two patients received no maintenance therapy. The dyspoietic changes in these four patients greatly exceeded those noted in a control group of ANLL patients on maintenance chemotherapy. The morphologic features of postleukemic dysmyelopoiesis were similar to those described in preleukemic dysmyelopoietic disorders. Erythroid abnormalities included hyperplasia with ring sideroblasts, megalobalstic changes, and cytoplasmic PAS reactivity. Myeloid abnormalities consisted of left‐shifted granulopoiesis with hyper‐ and hyposegmentation; megakaryocytic abnormalities included hyperplasia with a predominance of hypolobulated forms. Three of the four patients eventually suffered relapse and have died. The fourth patient died of sepsis after 20 months of pancytopenia and hysmyelopoiesis. Theories to explain the development of postleukemic dysmyelopoiesis are presented which emphasize the possibility of drug‐induced leukemia cell differentiation. Cytogenetic studies will be necessary to establish any relationship between ANLL and the subsequent postleukemic dysmyelopoiesis.
Monocyte markers and the common acute lymphoblastic leukemia antigen on chronic lymphocytic leukemia cells
Authors: Ligler Frances S. , Schlam Mark L , Curley Robert , Brodsky Isadore , Benzel John E. 
Published: February 2007

AbstractThe mononuclear cells from 42 patients with chronic lymphocytic leukemia (CLL) were analyzed for surface markers using monoclonal antibodies and flow cytofluorimetry. All 42 CLLs were Ig+ HLA.DR+OKT3−OKT4−OKT6−OKT8− and monoclonal with respect to light chain type. Thirty of the 42 tumours expressed the common acute lymphocytic leukemia antigen, and cells from 18 leukemias stained with the antibodies OKM1 and 63D3, which are specific for different antigens characteristic of myeloid cells. Eight of the leukemias failed to stain with PI153, which has been reported to recognize all B‐cell CLLs.
Complement in thrombotic thrombocytopenic purpura
Authors: Garvey M. Bernadette , Freedman John 
Published: February 2007

AbstractThis report describes a patient with recurrent thrombotic thrombocytopenic purpura (TTP)in whom complement activation was observed during the acute episodes. Serum C3, C4, and CH50 were reduced, and there was deposition of C3 on red cells and platelets. These findings returned to normal during clinical remission.
Autologous marrow transplantation for patients with chronic myelogenous leukemia (CML) in blast crisis
Authors: Thomas M. Roy , Robinson William A. , Dantas Mary , Koeppler Hubert , Drebing Carla , Glode L. Michael 
Published: February 2007

AbstractEleven patients with chronic myelogenous leukemia (CML) in blast crisis were treated with chemotherapy, followed by infusion of autologous bone marrow that had been collected during the chronic phase of the disease and cryopreserved at −198°C. The mean age of the nine females and two males in this study was 34 years with an average duration of the chronic phase of the disease of 5.5 years. Seven out of the 11 patients had a splenectomy prior to intensive chemotherapy. The median survival of the first four patients who received 6‐thioguanine, cytosine arabinoside, daunorubicin (TAD) chemotherapy was 2.6 weeks and no patient reachieved the chronic phase of CML. The second group of seven patients received more intensive chemotherapy (MAdHAT), which included melphalan 30 mg/m2 days 1, 2, and 3; Adriamycin 50 mg/m2 intravenously (iv) day 1, hydroxyurea 1500 mg/m2 by mouth for 5–7 days, cytosine arabinoside 100 mg/m2 continuous infusion for 5–7 days, and VM‐26 100 mg/m2 iv on day 3. Six out of these seven patients reachieved chronic phase CML after bone marrow reinfusion. The median survival was 29.9 weeks for all patients and 33 weeks for the six patients who reachieved chronic phase CML. All patients subsequently died of recurrent blast crisis. There was no correlation between the time of bone marrow storage and the duration of subsequent chronic phase CML. These studies have shown that autologous bone marrow transplantation after high‐dose chemotherapy can result in bone marrow engraftment with reestablishment of chronic phase CML, and prolongation of survival.
Difference between young and old patients in characteristics of leukemic cells: Older patients have cells growing excessively in vitro, with low antigenicity despite high HLA‐DR antigens
Authors: Giannoulis Nicos , Ogier Claudio , Hast Robert , Lindblom Bertil , Sjögren Ann‐Marie , Reizenstein Peter 
Published: February 2007

AbstractFifty‐six patients with acute, nonlymphatic leukemia in the initial phase were studied. The poor prognostic signs were excessive in vitro growth, many HLA‐DR‐positive cells, or a low ratio of leukemic cell antigenicity to HLA‐DR positivity and age. The cells from older patients formed more clusters (P < 0.05), and they had less capacity to stimulate normal allogeneic lymphocytes (P < 0.05) than those from younger patients. Cells forming many clusters also were more often (P < 0.01) HLA‐DR‐positive than those forming few clusters. It is suggested that the prognosis in old patients with acute leukemia is poor in part because their leukemic cells have characteristics different from those of young patients.