bluuebunny/crossref_metadata_2025_split huggingface dataset:
Peripheral blood remission of hairy cell leukemia after transfusion hepatitis
Authors: Keefer Michael J. , Weber Maril J. , Bottomley Sylvia S. , Solanki Dilip L. , Hosty Thomas A. 
Published: February 2007

AbstractHairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and transfusiondependent anemia after splenectomy. Each subsequently acquired transfusion hepatitis and in two patients marked hematologic improvement was noted within 2 months. Complete peripheral blood remission occurred within 17 months in all patients although bone marrow infiltration with hairy cells persisted. One patient remains in remission for 12 years; the other two succumbed to infectious illnesses but with normal blood counts. The mechanism by which hepatitis virus induces hematologic recovery in patients with hairy cell leukemia is unknown but may involve augmentation of the interferon system.
Hairy leukemic cells which hyperexpress Ii do not demonstrate Ii genome alterations by restriction endonuclease analysis
Authors: Owerbach David I. , Elliott William L. , Humphreys Robert E. 
Published: February 2007

AbstractThe finding of increased expression and apparently altered processing of Ii in hairy leukemic cells led us to test for Ii genomic alteration by Southern‐type Ii cDNA hybridization to leukemic spleen DNA cleaved with a series of restriction endonucleases. Some insertions, deletions, or point mutations, potentially detectable by this technique, might correlate to alteration in expression and function of Ij (and indirectly, class II antigens). No changes in genomic structure of Ii were detected in DNA isolated from spleens of five patients with hairy cell leukemia, compared with DNA preparations from peripheral blood cells of nineteen healthy blood donors. These experiments were consistent with the view that gross structural alteration of the Ii genome had not occurred in hairy leukemic cells.
Thymic carcinoma and aplastic anemia: Report of a previously undocumented association
Authors: Vicky Thomas C. , Carlos Manivel J. 
Published: February 2007

AbstractA case of thymic carcinoma documented after a 6‐year history of aplastic anemia is reported. The anemia responded initially to steroids and antithymocyte globulin. The carcinoma may have arisen in a previous undetected thymoma; alternatively, an underlying abnormality, presumably immunologic, played a role in the development of the anemia and the thymic carcinoma.
Thrombotic thrombocytopenic purpura in a human immunodeficiency virus (HIV)‐seropositive homosexual man
Authors: Jokela Janet , Flynn Thomas , Henry Keith 
Published: February 2007

AbstractA case of thrombotic thrombocytopenic purpura (TTP) in a human immunodeficiency virus (HIV)‐seropositive homosexual man is reported. The patient improved after corticosteroid and plasma exchange therapy was instituted. The case demonstrated most of the classic features of TTP, including histologic evidence of fibrin thrombi in small blood vessels. Atypical features included reduced number of megakaryocytes in the bone marrow and only partial resolution of thrombocytopenia after therapy was begun. An autoimmune thrombocytopenia has been well‐characterized in HIV ‐antibody‐seropositive homosexual men. Physicians caring for HIV‐seropositive persons should also be aware of a possible association with TTP.
The greekaγβ+‐hpfh observed in a large black family
Authors: Huang H. J. , Stoming T. A. , Harris H. F. , Kutlar F. , Huisman T. H. J. 
Published: February 2007

AbstractSeveral members of a Black family with a heterozygosity for an AγGβ+‐HPFH, shown in 1969 to have relatively low levels of Hb F and a low glycine to alanine ratio in the γ chain of this Hb F, were reinvestigated. Thirteen of 30 available family members in two generations had the heterozygous form of this condition, which was characterized by a decreased level of Hb A2, an average Hb FAD value of 13.3%, an equal distribution of Hb F over the red cells, and normal hematological values. The γ chain composition of isolated Hb F was determined by reversed phase high performance liquid chromatography for all 13 heterozygotes and showed an average Aγ value of 84.5 %. Hybridization with synthetic oligonucleotides, specific for normal and mutant sequences at positions 111–129 5′ to the Aγ globin gene, identified a G ± A base substitution at position 117, similar to that seen in subjects with the Greek Aγ‐HPFH. Our data support conclusions by others [15,16,30] that this replacement is causative of the increased Aγ chain synthesis in this condition. Haplotype analysis supported the suggestion that the G ± A substitution occurred as an independent event in this Black family.
Application of DNA polymorphisms for prenatal diagnosis of β thalassemia in chinese
Authors: Chan Vivian , Chan T. K. , Ghosh A. , Wong L. C. , Ma H. K. , Kan Y. W. , Todd D. 
Published: February 2007

AbstractForty‐seven Chinese suffering from β thalassemia major and their parents were studied to establish linkage of the βthal and βA genes with 11 restriction site polymorphisms. There is marked linkage disequilibrium at the BamH I site 3′ to the β globin gene, such that, in 31% of pregnancies, absence of the site in the fetus can exclude β thalassemia major. Using four restriction sites (Hinc II β, Ava II β, Hind III β, and BamH I β), prenatal diagnosis is feasible in all families. In 46% of all cases, a definitive diagnosis can be made, and in the remaining cases, a 50% chance of exclusion is possible. Fetal blood globin chain analysis would be required for the failures. Our experience in nine successive β thalassemia prenatal diagnosis is also reported.
A possible mechanism of increased sodium influx in red cells with abnormal membrane lipid levels induced by phospholipase A2
Authors: Hashimoto Masashi , Yawata Yoshihito 
Published: February 2007

AbstractMembrane transport—sodium (Na+) influx and calcium (Ca2+) uptake—was examined in human mature red cells treated with phospholipase A2 (PLase A2) from snake venom. PLase A2‐induced conversion of phosphatidyl choline (PC) to lysophosphatidyl choline (L‐PC) was associated with a marked increase in Na+ influx and Ca2+ uptake. After L‐PC was removed from the cell membrane of the PLase A2‐treated red cells in the presence of albumin, an additional increase in Ca2+ transport was observed. These results indicate that membrane lipid abnormalities, such as increased L‐PC and/or a loss of total lipids, appear to induce increased membrane transport.
Natural variation in passive sodium permeability in human erythrocytes
Authors: Lee Jong Y. , Prineas Ronald J. , Hallaway Philip E. , Eaton John W. 
Published: February 2007

AbstractThe rate of influx of 22Na+ into human erythrocytes (RBC) varies greatly depending upon the donor. A high rate of influx may be related to a congenital predisposition to essential hypertension. In Northern Europeans, we find a threefold difference in the rate of 22Na+ influx between those with the least (LP) and most highly permeable (HP) RBC (from <0.15 to >0.60 mmol Na+/liter RBC/hr). In order to further define determinants of these apparently hereditary differences in passive membrane Na+ transport, we identified two groups of normal laboratory and hospital personnel differing markedly (> twofold) in RBC 22Na+ influx rate. We find that the loop diuretics furosemide and bumetanide decrease by about 50% the influx of 22Na+ into HP RBC, but have a lesser influence on LP RBC. Impermeant polyanions such as citrate and pyrophosphate also specifically diminish 22Na+ influx into HP, but not LP, RBC. Therefore, the exaggerated 22Na+ influx into HP RBC probably occurs through a discrete pathway (perhaps via “Na/K/Cl cotransport”), which appears to be almost absent in LP RBC. The differences between HP and LP RBC most likely do not involve polymorphisms of RBC anion transport per se. The rate of RBC anion (35SO42–) transport is the same in HP and LP RBC and is equally inhibited by furosemide and (to a lesser extent) bumetanide. Furthermore, the potent inhibitor of RBC anion transport, DIDS (diisothiocyanostilbene disulfonate) does not affect RBC Na+ permeability in either group. Nonetheless, the preferential reduction of Na+ permeation of HP RBC by loop diuretics may be of help in experimentally distinguishing HP from LP phenotypes. This information may be crucial in unraveling the structural basis of intrinsic differences in cell membrane Na+ permeability and their possible relationship to essential hypertension.
Diminished lymphocyte and granulocyte γ‐glutamyltranspeptidase activity in acute lymphocytic leukemia and response to chemotherapy
Authors: Russo Sandra A. , Harris Michael B. , Greengard Olga 
Published: February 2007

Abstractγ‐Glutamyltranspeptidase (GGT) acitivity (per mg protein) in blood lymphoid cells of 27 children with acute lymphoblastic leukemia (ALL) (1.05 ± 0.15) was significantly below that of controls (2.25 ± 0.30), became normalized during chemotherapy‐induced remission (2.47 ± 0.26), and was low again (1.59 ± 0.62) in relapsed subjects. Individual variations in the GGT activity of the blood lymphoid cell fraction (per mg protein) bore a significant inverse correlation to the number of white blood cells (WBC) as well as of blasts per ml blood. Blasts had minimal GGT activity; however, partial GGT deficiency was also exhibited by the microscopically normal circulatory lymphocytes of several patients prior to treatment and in relapsed subjects whose blood was still devoid of blasts.Significantly diminished GGT activity (per mg protein) was found in the blood granulocytes of ALL subjects. This deficit, restored during remission and present again at relapse, varied in magnitude but showed no statistically significant correlation to the different patients' degree of neutropenia. In about one‐third of the newly diagnosed or relapsed pre‐B ALL children, the circulatory granulocytes' GGT activity was only 10–20% of normal. The results suggest that 1) the presence or absence of this sign of functional maldifferentiation in granulocytes is a factor in the heterogeneity of disease manifestation among subjects with apparently the same type of ALL and that 2) measurement of GGT in the circulatory granulocytes, as well lymphocytes, may be useful for monitoring the efficacy of chemotherapy.
Acute myelomonocytic leukemia (AML‐M4) and translocation t(6;9) (p23;q34): Two additional patients with prominent myelodysplasia
Authors: Horsman Douglas E. , Kalousek Dagmar K. 
Published: February 2007

AbstractTwo patients with acute myelomonocytic leukemia (AML‐M4) and a specific chromosomal translocation t(6;9)(p23;q34) are reported and compared to 21 AML patients with the same translocation collected from the literature. Our observations suggest that AML with t(6;9)(p23;q34) is characterized by myelodysplasia, basophilia, and a variety of blast cell morphologies (M1, M2, M4) with a greater proportion of the cases than previously appreciated being examples of acute myelomonocytic leukemia (AML‐M4). The consistent association of myelodysplasia provokes the proposal that this subtype of de novo AML is a result of an acute stem cell disorder. The poor outcome with standard AML chemotherapy experienced in this group of relatively young patients necessitates consideration of alternative therapeutic strategies such as early bone marrow transplantation.
Immunophenotyping of acute myeloid leukemia by immuno‐alkaline phosphatase (APAAP) labeling with a panel of antibodies
Authors: Davey Frederick R. , Erber Wendy N. , Gatter Kevin C. , Mason David Y. 
Published: February 2007

AbstractA panel of eight antibodies was used by the alkaline‐phosphatase/anti‐alkaline phosphatase (APAAP) method to stain peripheral blood films, bone marrow smears, and cytocentrifuge preparations from 29 cases of acute myeloid leukemia. These findings were correlated with the French‐American‐British (FAB) classification. Leukemic cells from six cases of myeloblastic leukemia (FAB;M1) were predominantly labeled by the antimyeloperoxidase monoclonal antibody (MPO‐7). Leukemic cells from the majority of eight cases of myeloblastic leukemia with maturation (FAB;M2) and progranulocytic leukemia (FAB;M3) stained with monoclonal antibodies MPO‐7, NP57 (anti‐elastase), and EBM11 (antimonocyte/macrophage). Leukemic cells from six cases of myelomonocytic (FAB;M4) and five cases of monocytic (FAB;M5) leukemia were most often labeled with antibodies MPO‐7, NP57, and EBM11 as well as monoclonal antibodies Y1/82A (anti‐monocyte) and KB90 (against the p150, 95 molecule, CD11c; a monocyte/granulocyte marker), but not with monoclonal antibody C17 (antiglycoprotein IIb/IIIa) and/or monoclonal antibody Y2/51 (antiglycoprotein IIIa). Erythroblasts from a single case of erythroleukemia (FAB;M6) were not labeled with any of the antibodies from this panel. Leukemic cells from two cases of acute megakaryocytic leukemia (FAB;M7) stained strongly with the monoclonal antiglycoprotein IIIa/IIb antibody (C17) and antiglycoprotein IIIa antibody (Y2/51). Staining by the APAAP method with this panel of antibodies was easy to perform, required no expensive instrumentation, and provided useful information in the classification of acute myeloid leukemia.
Impaired antioxidant defense in hemoglobin E‐containing erythrocytes: A mechanism protective against malaria?
Authors: Lachant Neil A. , Tanaka Kouichi R. 
Published: February 2007

AbstractRed blood cell (RBC) antioxidant defense was investigated in eight individuals with hemoglobin E (Six EE and two E‐B+ thalassemia) and compared to that in six individuals with thalassemia and ten normal subjects. Individuals with hemoglobin E had increased incubated Heinz body formation (68% ± 18%; p < 0.001) compared to normal and thalassemic RBC (10% ± 2% and 11% ± 5%, respectively). Stimulated pentose phosphate shunt activity was increased in the thalassemic and decreased in the hemoglobin E RBC as compared to normal. The 2,3‐diphosphoglycerate (DPG) content of the EE RBC was increased to 5.59 ± 0.69 μmol/ml RBC as compared to normal (4.51 ± 0.77; p < 0.001). In the EE RBC, there was a direct correlation between Heinz body formation and DPG content (r = 0.73). Ascorbic and dehydroascorbic acid (0.1 and 1.0 mM) were able to decrease the degree of Heinz body formation in the hemoglobin E RBC. Ascorbic acid (0.1 mM) prolonged the response of the pentose shunt. Thus impaired antioxidant defense may account for the persistence of the hemoglobin E gene in areas where malaria is endemic. Oxidant medications should be used with caution in individuals of Southeast Asian origin.
Effect of heparin on platelet count and platelet aggregation
Authors: Shojania A. Majid , Turnbull Gail 
Published: February 2007

AbstractThe in vitro effect of heparin on platelet aggregation was studied in three groups: in 26 subjects recently treated with heparin, in 18 subjects on maintenance hemodialysis, and in 20 normal controls. With the aid of Technicon H6000, platelet counts and platelet aggregations were compared in whole blood samples collected in ethylenedi‐aminetetraacetic acid (EDTA) and in heparinized tubes. Although there was no significant difference between platelet count of heparinized and EDTA blood in the control group, the dialysis group and the group recently treated with heparin showed significantly lower platelet counts and more platelet aggregation in heparinized tubes than in EDTA tubes.We speculate that the majority of subjects exposed to heparin develop an antibody or a proaggregator which can aggregate or agglutinate platelets in the presence of heparin and causes destruction of platelets; but only in a small percentage of subjects receiving heparin is this reaction severe enough to cause thrombocytopenia.
Carrier detection in the hemophilias
Authors: Lillicrap D. P. , White B. N. , Holden J. J. A. , Giles A. R. 
Published: February 2007

AbstractThe subject of carrier detection in the hemophilias has received new impetus in the past several years. Treatment complications arising from clotting factor concentrates have become more evident and earlier prenatal diagnosis and new genetic markers for the clotting factor genes have focused interest on this area. Until now, carrier diagnosis has relied upon standard pedigree analysis and clotting factor assays. The results obtained using these methods are probabilistic, and the coagulation tests are unavoidably influenced by the effects of random X chromosome inactivation and the inherent variability of the methods involved. With the cloning and characterization of both factor IX and factor VIII genes, has come the capability of using gene analysis to diagnose the carrier state. This usually involves the detection of restriction fragment length polymorphisms (RFLPs) and their use as linked markers for the defective clotting factor gene. In hemophilia A, the combined use of three intragenic RFLPs and two closely linked, highly polymorphic extragenic markers will make carrier information available to approximately 90% of kindred. In hemophilia B, phenotypic analysis has been complicated by the more heterogeneous expression of the gene defect. To date, five intragenic and one closely linked RFLP have been reported, as well as two protein polymorphisms detectable by monoclonal antibody immunoassays. With the combined use of these genetic markers it is likely that accurate carrier assignment will be available to more than 80% of hemophilia B families.
Evaluation of factor VII antigen in factor VII congenital deficiencies with a new ELISA assay
Authors: Tirindelli M. C. , Mariani G. , Mazzucconi M. G. , Iacopino G. , Carbonaro M. , Ghirardini A. , Motta M. , Mandelli F. 
Published: February 2007

AbstractAn evaluation of a new Enzyme Linked Immunosorbent Assay (ELISA) factor VII:Ag assay in factor VII congenital deficiencies was carried out. This assay was compared to factor VII:C assay and the Inhibitor Neutralization Assay (INA) for factor VII:Ag, both in normals and F VII‐deficient patients. The correlations between ELISA F VII:Ag and VII:C, as well as the one between INA F VII:Ag and VII:C, were good (r = .86 and .81, respectively). The correlation between the two immunologic methods of assay in normals was fairly good (r = 0.73, P < 0.001); whereas in mild F VII deficiencies (heterozygotes for F VII deficiencies), the two methods correlated very well (r = .96). In the severe deficiencies, ELISA F VII:Ag assay allowed the evaluation of F VII protein levels below 1 u/dl.The sensitivities of the INA and ELISA assays were evaluated by creating artificially prepared plasmas containing serial amounts of purified F VII: INA was unable to pick up F VII protein levels lower than 75 ng/ml, whereas the ELISA assay could detect up to 5 ng of F VII.
Acquired factor XI inhibitors in congenitally deficient patients
Authors: Schnall S. F. , Duffy T. P. , Clyne L. P. 
Published: February 2007

AbstractFour factor XI (F XI)‐deficient patients are described, all of whom formed circulating anticoagulants against F X1. In the three most severely affected patients (F XI 0%–6% activity), the anticoagulant appeared to have been stimulated by plasma infusion. However, in the milder case (25% F XI activity), no infusion had been documented. The findings in these cases emphasize the diversity of F XI inhibitors in congenitally deficient patients. Awareness of the potential development of these inhibitors will be helpful in both daily management and perioperative care of such patients.
Durable response to etoposide‐loaded platelets in refractory immune thrombocytopenic purpura: A case report
Authors: Wood Lucille , Jacobs Peter 
Published: February 2007

AbstractA 23‐year‐old female presented with widespread purpura and ecchymoses as a result of immune thrombocytopenia which was refractory to corticosteroids, splenectomy, and immunosuppressive agents. In view of the extent and serious nature of her bleeding, salvage therapy with etoposide‐loaded platelets was undertaken, which was associated with a lasting complete remission.
Acute respiratory failure in hyperleukocytotic acute myeloid leukemia: The role of perfusion lung scintigraphy
Authors: Nagler A. , Brenner B. , Zuckerman E. , Hazani A. , Israel O. 
Published: February 2007

AbstractA patient with acute hyperleukocytotic myelogenous leukemia who presented with acute respiratory distress is reported. Clinical manifestations included dyspnea, tachypnea, hyperventilation, and cyanosis. Blood gas analysis revealed hypoxemia, hypocapnia, and metabolic acidosis. Chest X‐ray and perfusion lung scanning were normal. Pulmonary leukostastis syndrome (PLS) was later confirmed at autopsy. In a patient with hyperleukocytosis from acute nonlymphocytic leukemia and respiratory distress, a normal perfusion lung scintigraph should make one consider the PLS.
Philadelphia‐positive acute myelomonocytic leukemia with inversion of chromosome 16 and eosinobasophils
Authors: Mecucci Cristina , Noens Lucien , Aventin Ana , Testoni Nicoletta , Berghe Herman Van Den 
Published: February 2007

AbstractCytogenetic studies in a case of acute nonlymphocytic leukemia, type M4‐FAB with eosinophilia, showed an acquired abnormal karyotype characterized by both a t(9;22) Philadelphia translocation and a pericentric Inversion of chromosome 16, inv(16)(p13q22). This unique case shows that the previously described association between inversion of chromosome 16 and myelomonocytic leukemia with bone marrow eosinophilia is true also in Philadelphia‐positive disorders.The present findings are consistent with the existence of a group of chromosome anomalies, namely t(15;17) and inv(16), which are specifically associated with one single stage of morphological differentiation of myeloid leukemic cells.
Divalent cations induce protofibril gelation
Authors: Marx Gerard 
Published: February 2007

AbstractSoluble fibrin oligomers (protofibrils) undergo phase change merely by adding 1–2 mM Ca2+ 25‐100 μM Zn2+. The cation‐induced “protofibrin” clots appear similar to normally formed fibrin gels. Maximal clot turbidity of protofibrin gels increases with cations in a concentration‐dependent manner. Magnesium (less than 0.5 mM) is ineffective in inducing protofibril gelation. Turbidity and degree of polymerization (DP) [indirectly expressed as AT (activation time)] appear to be positively correlated, regardless of whether the divalent cation is Ca2+ or Zn2+. Cross sections of Ca2+‐induced protofibrin fibers are ∼6–18‐fibrin‐monomers‐thick. With both Ca2+ and 40 μM Zn2+, fiber cross section increases to 30–50 monomers thick. Negatively stained Zn2+,‐ and Ca2+‐induced protofibrin gels exhibit banding periodicity of ∼ 240 Å, similar to that of normally generated fibrin gels. Regions of lateral merging of individual segments of the protofibrin leads to increased cross section of the fiber and forms a branch required for gelation. These findings indicate that the rate of coagulation and the ultimate thickness of the fibers both relate to lateral associative processes of protofibrils, which are augmented by physiologic concentrations of2+ and Zn2+.
Acute myeloblastic leukemia presenting as apparent acute otitis media
Authors: Bertrand Yves , Lefrère Jean‐Jacques , Leverger Guy , Bonnet Madeleine , Lacombe Hervé , Lasfargues Gérard 
Published: February 2007

AbstractThree cases of acute myeloblastic leukemia of FAB‐M2 type revealed by an isolated otitis media are reported. This mean of revelation is valuable for early diagnosis and treatment of the disease. A karyotype was performed in two cases and showed an 8;21 translocation. As far as we know, these are the first acute myeloblastic leukemia cases with otitis media reported in which an 8;21 translocation was detected.