bluuebunny/crossref_metadata_2025_split huggingface dataset:
Unmasking of cryptic natural killer (NK) cell recognition sites on chronic lymphocytic leukemia lymphocytes
Authors: Spitz Daniel L. , Zucker‐Franklin Dorothea , Nabi Zeenat F. 
Published: February 2007

AbstractThe sensitivity of chronic lymphocytic leukemia (CLL) lymphocytes to attack by natural killer (NK) cells has remained questionable. To clarify this issue, freshly isolated lymphocytes of 37 patients with B‐CLL, five with WDLL and two with HCL, were tested with a standard cytotoxicity assay with NK cells from normal donors. All these targets were resistant to cytolysis by the effectors. Freeze‐fracture analysis of CLL cell plasma membranes revealed that they have a larger number of intramembranous particles (IMP) associated with the external leaflet (E‐face) than have normal lymphocytes. Unlike other neoplastic cells, exposure of CLL lymphocytes to phorbol esters or treatment with neuraminidase did not render them vulnerable to attack by NK cells, nor did 5 days of culture have an effect. Incubation of CLL lymphocytes with anti‐lg‐μ (24–72 hr) or with 0.1% pepsin (15 min) resulted in 15% and 27% cytolysis, respectively. B‐lymphocytes from the blood of healthy donors were not killed when treated similarly: These data establish that freshly isolated B‐CLL lymphocytes are resistant to NK cytolysis but that in contrast to normal B‐cells, they possess cryptic NK‐recognition structures, which may be uncovered by surface modulation.
Spontaneous mutation in the male gamete as a cause of hemophilia A: Clarification of a case using DNA probes
Authors: Howard Phillip L. , Hoag Jennifer B. , Bovill Edwin G. , Heintz Nicholas H. 
Published: February 2007

AbstractThe carrier status of two sisters of the mother of a hemophilic boy was clarified by the use of DNA probes in a family with a single case of hemophilia A and no family history of the disease. The extragenic polymorphic site demonstrated by probe DX13 (locus DXS 15) and the intragenic polymorphic site demonstrated by Bgl I digestion and a factor VIII partial cDNA probe indicated that the mother of the index case carried a mutation in the X‐chromosome received from her nonhemophilic father rather than the X‐chromosome received from her mother. In spite of equivocal coagulation data, the mother's two sisters were shown not to be carriers of hemophilia A.
Hemoglobin J Guantanamo [α2β2 128 (H6) Ala→Asp] in association with hemoglobin C and α‐thalassemia in a family from Benin
Authors: Wajcman H. , Baudin‐Chich V. , Kister J. , Feo C. , Gombaud‐Saintonge G. , Bohn B. , Marden M. , Pagnier J. , Poyart C. , Dodé C. , Galacteros F. , Blouquit Y. , Cynober T. , Tchernia G. 
Published: February 2007

AbstractHemoglobin J (HbJ), Guantanamo, which had been described but once in the literature, was found in a tamily originating from Benin; this second case was found to be in association with hemoglobin C (HbC) and α‐thalassemia. High‐performance liquid chromatography (HPLC) procedures and microsequencing were used for characterization of the aminoacid substitution. The main hematological disorder, in relation with the instability of Hb J Guantanamo, seems to be a worsening of the rheological properties of the red blood cells (RBC), as demonstrated by ektacytometric studies. Oxygen‐binding properties of the RBC were almost normal, but a slight decrease in cooperativity and lowered Bohr and 2,3‐diphosphoglycerate (DPG) effects were observed for pure stripped Hb J Guantanamo. The expression of the electrophoretic charge difference was partly masked, as is often observed when the structural abnormality is situated in or near a contact area.
Two homozygous cases of erythrocyte pyruvate kinase (PK) deficiency in Japan: PK sendai and PK shinshu
Authors: Tani Kenzaburo , Fujii Hisaichi , Takahashi Keisuke , Ogura Hiromi , Kanno Hitoshi , Hayasaka Kiyoshi , Narisawa Kuniaki , Nakahata Tatsutoshi , Akabane Taro , Morisaki Takayuki , Tsutsumi Hisashi , Miwa Shiro 
Published: February 2007

AbstractTwo new erythrocyte pyruvate kinase (PK) variants with severe nonspherocytic hemolytic anemia are presented. These cases are both considered to be homozygous because of the consanguineous marriages in their parents. Their erythrocyte PK's were characterized by the recommended methods of the International Committee for Standardization in Haematology (ICSH). These two variants have been named PK Sendai and PK Shinshu. PK Sendai showed a high K0.5S(phosphoenolpyruvate), was remarkably inhibited by ATP, and was thermolabile, while PK Shinshu demonstrated remarkably low enzyme activity and required a high level of fructose 1,6‐diphsphate for activation.
Significance and limits of cerebrospinal fluid beta‐2‐microglobulin measurement in course of acute lymphoblastic leukemia
Authors: Musto Pellegrino , Tomasi Paolo , Cascavilla Nicola , Ladogana Saverio , La Sala Antonio , Melillo Lorella , Nobile Michele , Castoldi Gianluigi , Carotenuto Mario 
Published: February 2007

AbstractCerebrospinal fluid beta‐2‐microglobulin (CSF‐β2m) was measured longitudinally in 48 patients affected by acute lymphoblastic leukemia (ALL). Thirteen developed a central nervous system (CNS) involvement during the course of the disease; although moderately higher mean CSF‐β2m levels were found in these subjects, no significant statistical differences were observed in comparison with patients without this complication and compared with the control group. No correlations were found between β2m and other biochemical parameters in CSF. Furthermore, CSF‐β2m levels appeared to be influenced by previous combined chemoradiotherapeutic treatment for CNS prophylaxis, presence of meningeal non‐neoplastic infiltrates, patients' ages, amount of CSF blasts, and their immunological phenotype. In particular, only clearly B‐committed leukemic cells, when tested, showed a strong surface expression of β2m, as demonstrated by immunocytochemical detection of this protein on cell membrane. However, in specific cases, CSF β2m measurement and CSF/serum β2m ratio were helpful in diagnosing and monitoring isolated CNS disease. Such findings suggest that CSF‐β2m assay may be a useful tool in the management of CNS involvement in the course of ALL in only selected patients, as several factors can modify the outcome.
Successful outcome of severe aplastic anemia following epstein‐barr virus infection
Authors: Grishaber Judy E. , McClain Kenneth L. , Mahoney Donald H. , Fernbach Donald J. 
Published: February 2007

AbstractSevere aplastic anemia has been reported in children following Epstein‐Barr virus (EBV) infection. The pathophysiology of disease is not known, but an immunologic mechanism is suspected because of the rapid response to steroids. A patient with severe aplastic anemia following EBV infection experienced bone marrow recovery after treatment with high‐dose prednisone. After reviewing the literature it is concluded that steroid therapy is indicated in this select group of patients.
CD5 positive immunoregulatory B cell subsets
Authors: Paglieroni Teresa , Caggiano Vincent , Mackenzie Malcolm 
Published: February 2007

AbstractB‐chronic lymphocytic leukemia (B‐CLL) is a heterogeneous disease often expressed as a clonal expansion of CD5+ B cells. We report the characterization of CD5+ B cells from two unique B‐CLL patients. Cells from patient 1 coexpressed CD5 (leu‐1), CD19 (Leu‐12), CD20 (B1), and HLA‐DR; they were CD10 (J5), CD21 (B2), CD22 (Leu‐14), CD25 (IL2‐R1), PCA‐1, surface, and cytoplasmic lg negative. They suppressed normal peripheral blood lymphocyte (PBL) pokeweed mitogen (PWM) ‐stimulated Immunoglobulin (lg) synthesis >80%. Cells from patient 2 were CD5 (Leu‐1), CD19 (Leu‐12), CD20 (B1), CD21 (B2), CD22 (Leu‐14), HLA‐DR, IgM, and kappa positive. They were negative for CD10 (J5), CD25 (IL2‐R1), and PCA‐1. These cells did not suppress normal PBL PWM‐stimulated lg synthesis but produced a monoclonal IgM kappa protein with rheumatoid factor‐like activity. These observations suggest that there are different CD5+ B cell subsets, one immunosuppressive and the other autoreactive.
Hemoglobin constant spring defined by specific oligonucleotide hybridization and hemoglobin D Punjab (β121→Gln) in a Batak Indonesian family
Authors: Kosasih Eddi N. , Cai Shi‐Ping , Kan Yuet W. , Lie‐Injo Luan Eng 
Published: February 2007

AbstractA Batak Indonesian from North Sumatra with hemoglobin (Hb) D Punjab (α2 β2 121→Gln) and hemoglobin Constant Spring (Hb CoSp) is described. The 24‐year‐old man did not have clinical symptoms, and his hematological indices were normal. However, he had a persistent slight elevation of fetal hemoglobin level. His mother and his brother were heterozygous for Hb D Punjab; his father had Hb CoSp trait. A sister did not have any abnormal hemoglobin. To show the exact molecular defect leading to the synthesis of Hb CoSp in this family, genomic DNA from the father was analyzed by hybridization with synthetic oligonucleotides. Genomic DNA was digested with Sst I and Hind III producing a 1.05‐kb fragment from the 3′ end segment of the α2‐globin gene, including the termination codon. Two nonadecamers were synthesized to serve as probes: one, entirely homologous to the normal 3′ end of α2A‐globin gene sequence, including the termination codon TAA, the other different from it by a replacement of the T in the termination codon TAA with C, changing it to CAA, the codon for the amino acid glutamine. DNA from normal controls gave a positive signal with the normal α2TAA oligonucleotide probe but negative with the α2CAA probe. The father of propositus who had Hb CoSp trait gave a positive signal with the normal α2TAA oligonucleotide probe as well as with the α2CAA oligonucleotide probe, showing him to be heterozygous for the α2CAA‐globin gene. This result shows that the Hb CoSp in the Batak family is indeed due to a replacement of T by C in the TAA termination codon of the α2‐globin gene changing it to CAA the codon for glutamine. This explains the resulting readthrough of the untranslated sequence of the mRNA.
IgD multiple myeloma: A cure at 21 years
Authors: Kyle Robert A. 
Published: February 2007

AbstractA 52‐year‐old man with a plasmacytoma of the body of T‐10 in February 1965 returned 6 months later with multiple myeloma characterized by bone pain, osteolytic lesions, and IgD λ monoclonal protein in the serum, 6.1 g of Bence Jones protein in the urine, and 21% plasma cells in the bone marrow. The M‐protein and bone pain disappeared within 6 weeks after therapy with melphalan and prednisone was started. Therapy was discontinued in December 1974. Immunoelectrophoreses and immunofixations of the serum and urine over the years revealed no monoclonal protein. A mediastinal tumor developed, and the patient died of respiratory insufficiency on October 23, 1986. Autopsy revealed a large bronchogenic carcinoma of the right lung extending to the mediastinum, trachea, and esophagus. There was no evidence of multiple myeloma. This patient had responded rapidly to chemotherapy and had no recurrence of myeloma during a 21‐year follow‐up.
Antifibrinolytic therapy in the management of the kasabach merritt syndrome
Authors: Ortel Thomas L. , Onorato James J. , Bedrosian Camille L. , Kaufman Russel E. 
Published: February 2007

AbstractThe Kasabach Merritt syndrome consists of thrombocytopenia, microangiopathic he‐molytic anemia, and a localized consumption coagulopathy that develops within the abnormal vascular channels of a hemangioma. In general, these patients demonstrate only mild abnormalities of screening clotting tests, but they can potentially develop life‐threatening complications. We present a patient who developed a severe anemia that was refractory to erythrocyte transfusions. Treatment with epsilon‐aminocaproic acid to inhibit fibrinolysis and cryoprecipitate to replenish his deficient circulating fibrinogen interrupted the cycle of his systemic coagulopathy and enabled us to transfuse him to a normal hematocrit.
Defective release of tissue plasminogen activator in patients with sickle cell anemia
Authors: Phillips George , Mitchell Lanetta B. , Pizzo Salvatore V. 
Published: February 2007

AbstractRecent studies suggest that intravascular coagulation occurs in sickle cell anemia. There is also some evidence that decreased fibrinolytic activity may be associated with the disorder. In the current study we measured tissue plasminogen activator levels (t‐PA) in seven asymptomatic patients with sickle cell anemia. The mean level of releasable t‐PA was only 0.01 lU/mL ± 0.005 (SEM) as compared to 118 healthy volunteers with levels of 0.70 ± 0.10 (SEM). Despite the small sample size, the difference between the two groups was statistically significant (P < .001). These data suggest that defective release of t‐PA may contribute to a hypercoagulable state in sickle cell anemia.
Evidence for posttranslational control of fetal hemoglobin synthesis
Authors: Qiu C. C. , Abraham E. C. 
Published: February 2007

AbstractSubunit competition experiments were conducted by recombining various amounts of α‐subunits with an equimolar mixture of γ‐and βA‐subunits. The results showed that γ‐subunits combine with α‐subunits less readily than β‐subunits. Thus, in the presence of α‐chain deficiency (α‐thalassemia) the formation of HbA is preferred over HbF, resulting in a decrease in the level of the latter.
Platelet antibody: Review of detection methods
Authors: Schwartz Kenneth A. 
Published: February 2007

AbstractThe driving force behind development of in vitro methods for platelet antibodies is identification of plasma factors causing platelet destruction. Early methods relied on measurement of platelet activation. Current methods are more specific and use a purified antibody against immunoglobulin or complement, which is usually labeled with 125I or tagged with an enzyme or fluorescein. Comparisons of guantitation of platelet‐associated IgG show wide variability between different methods. The disparate results can be related both to differences in binding of secondary antibodies to immunoglobulin in solution compared to immunoglobulins attached to platelets and to the improper assumption that the binding ratio between the secondary detecting and primary antiplatelet antibody is one. Most assays can 1) identify neonatal isoimmune thrombocytopenia and posttransfusion purpura, 2) help to differentiate between immune and nonimmune thrombocytopenias, 3) help to sort out the offending drug when drug‐induced thrombocytopenia is suspected, and 4) identify platelet alloantibodies and potential platelet donors via a cross match assay for refractory patients. However, the advantages of quantitative assays over qualitative methods with respect to predictions of patients clinical course and response to different treatments remain to be investigated.
Transient ischemic attack in a patient with congenital protein‐c deficiency during treatment with stanozolol
Authors: de Stefano Valerio , Leone Giuseppe , Teofili Luciana , Ferrelli Rita , Pollari Giuseppe , Antonini Vincenzo , Bizzi Bruno 
Published: February 2007

AbstractA patient with congenital protein‐C deficiency was treated with stanozolol for 8 weeks to increase circulating levels of protein C. A rise in protein C was achieved, accompanied by an increase in factor II, factor X, antithrombin III, and protein S; but at the 8th week the patient suffered a transient ischemia attack.
Probable genetic linkage between genes coding for platelet‐specific antigens of the PIA and bak systems
Authors: Letellier Steven J. , Hunter Jay B. , Aster Richard H. 
Published: February 2007

AbstractStudies of the inheritance of the platelet‐specific alloantigens PIA1/PlA2 and Baka/Bakb in informative families provided evidence that the genes determining PlA and Bak antigens are in close genetic linkage (P = .004). In 154 normal, unrelated Caucasian subjects it was found that the antigen PIA1 is associated with Baka and the antigen PIA2 with Bakb more often than would be expected by chance (P < .05). Bak and PIA antigens are carried on platelet membrane glycoproteins IIb and IIIa, respectively, which are decreased or absent in the inherited platelet disorder Glazmann's thrombasthenia. Thus, the findings suggest that the defect in this disease involves two closely linked genes.
Testicular tumor as the first manifestation of B‐lymphoid blastic crisis in a case of Ph‐positive chronic myelogenous leukemia
Authors: Ohyashiki Junko H. , Ohyashiki Kazuma , Shimizu Hirofumi , Miki Makoto , Kimura Nobuhiro , Mori Shigeo , Fujisawa Kouji , Akatsuka Jun‐Ichi , Toyama Keisuke 
Published: February 2007

AbstractWe report an 8‐year‐old boy with Ph‐positive chronic myelogenous leukemia (CML) who showed a testicular tumor as the first manifestation of blastic crisis. Pathological examination of the excised testis revealed marked infiltration of immature cells, whereas bone marrow remained in the chronic phase. Immunohistochemical and molecular examinations disclosed the immature cells to have B‐lymphocyte surface determinants and immunoglobulin JH rearrangement. DNA obtained from the testis and peripheral blood cells showed similarly rearranged bcr configurations, indicating that infiltrating cells in the testis originated from the CML clone. Bone marrow showed B‐lymphoid blastic crisis 4 months after testicular involvement.
Daily exposure to forty percent oxygen causes a decrease in platelet count
Authors: Meintjes André F. , Baynes Roy D. , Rogers Geoff G. , Mendelow Barry V. 
Published: February 2007

AbstractA study was undertaken to evaluate a previous observation made in this laboratory that hyperoxic treadmill training is associated with a decrease in the circulating platelet count (PLT). The subjects studied breathed air containing 40% oxygen for 30 min/day, 5 days/week, for 6 weeks, while at rest in the seated position. Such exposure resulted in a consistent fall in PLT between day 1 and day 5 of each week (P < .01). In addition, there was a progressive decline in PLT over the 6 weeks of exposure to the hyperoxic air, PLT being inversely correlated with the duration of hyperoxic exposure (rs = ‐0.886, P < .02). The total decrease in PLT over the 6 weeks was 56 ± 46 ± 109 I−1 (P < .025). Not surprisingly, these changes were mirrored in the plateletcrit (Pct). The decrease in PLT did not appear to be secondary to either hemodilution or reduced erythropoietic stimulation. The mechanism of production and the biological significance of these changes remain to be elucidated.
Immunogenicity of a recombinant hepatitis B vaccine in hemophiliacs
Authors: Mannucci P. M. , Gringeri A. , Morfini M. , De Biasi R. , Tirindelli M. C. , Tamponi G. , Baudo F. , Carnelli V. , Ciavarella N. , Colombo M. , Zanetti A. 
Published: February 2007

AbstractYeast‐recombinant vaccines against hepatitis B virus (HBV) are now available, but there is no information about whether or not they are immunogenic in patients with hemophilia and other congenital bleeding disorders. Twenty micrograms of a recombinant vaccine expressing the adw serotype of the hepatitis B surface antigen (HBsAg) were given to 41 patients negative for HBV markers and again after 1 and 6 months. Ten percent of the vaccinees had anti‐hepatitis B surface antibody (anti‐HBs) responses, with titers of 10 mlU/ml or more, 1 month after the first dose of vaccine. The percentage of anti‐HBs‐positive patients increased to 54% after the second dose and to 98% after the third dose, with only one non‐responder. Hence, the recombinant vaccine was immunogenic, with percentages of seroconversion and anti‐HBs titers similar to those achieved with plasma‐derived vaccines.
Rapid excretion of gallium‐67 isotope in an iron‐overloaded patient receiving high‐dose intravenous deferoxamine
Authors: Baker David L. , Manno Catherine S. 
Published: February 2007

AbstractA 23 year‐old black male with homozygous sickle cell disease (Hb SS disease) and transfusional iron overload was admitted for evaluation of response to intravenous deferoxamine (DFO) therapy. Soon after admission, the patient suffered an intraventricular hemorrhage and during his subsequent hospitalization developed a persistent fever of undetermined origin (f.u.o.). Included in the diagnostic evaluation of fever was a gallium 67 scan (Ga‐67), which was initially nondiagnostic because of Ga‐67 citrate's preferential chelation by DFO. After DFO was discontinued, a repeat scan demonstrated a lesion above the left kidney.To our knowledge the unusual interaction in vivo of DFO with Ga‐67 citrate has not been reported in the clinical literature. With the anticipated increased use of chelation therapy for patients with transfusional iron overload, this interaction may be encountered more frequently. DFO should be discontinued before the use of Ga‐67 scanning in this clinical situation, or an alternative isotopic scan, such as indium‐labelled white cells, should be considered.
Coagulation studies in thrombotic thrombocytopenic purpura, with special reference to von willebrand factor and protein S
Authors: Takahashi Hoyu , Tatewaki Wataru , Nakamura Tadao , Hanano Masaharu , Wada Ken , Shibata Akira 
Published: February 2007

AbstractThe profile of blood coagulation and fibrinolysis was studied in detail in eight patients with acute thrombotic thrombocytopenic purpura (TTP). In the majority of the patients, fibrinogen, factor XIII, antithrombin III, α2‐plasmin inhibitor, plasminogen, and α2‐macroglobulin were normal, whereas FDP, plasmin‐α2‐plasmin inhibitor complex, and tissuetype plasminogen activator antigen were marginally or moderately elevated. Low fibronectin values were observed in four patients. Protein C and C4b‐binding protein were nearly normal, whereas total protein S and free protein S were reduced in five and six patients, respectively. A positive correlation was found between total protein S and C4 and between free protein S and C3. von Willebrand factor antigen (vWf:Ag) and ristocetin cofactor (RCof) were either normal or elevated, but RCof/vWf:Ag ratio was decreased in seven patients. Crossed immunoelectrophoresis and sodium dodecyl sulfate (SDS)‐agarose gel electrophoresis revealed that the large vWf multimers were either absent from or relatively decreased in all patients except one. In addition, one patient had unusually large vWf multimers, and a low‐molecular‐weight vWf fragment was apparently observed in three patients. These findings indicate that the intravascular generation of thrombin and plasmin was minimal in TTP and suggest that the alterations of the vWf molecule were caused not only by consumption through its participation in platelet thrombus formation but also by accelerated proteolysis. Low protein S values would be related to the immunological abnormalities underlying TTP.
Severe thrombotic tendency associated with a type I plasminogen deficiency
Authors: Leebeek Frank W. G. , Knot Eduard A. R. , Cate Jan Wouter Ten , Traas Daan W. 
Published: February 2007

AbstractWe report a 45‐year‐old female patient with recurrent spontaneous deep vein thrombosis associated with an isolated hypoplasminogenemia (plasminogen activity and antigen level of 42% and 37%, respectively). The plasminogen molecule was normal as demonstrated by a normal activation by tissue plasminogen activator, electrophoretic mobility on crossed immunoelectrophoresis, molecular weight, and binding to lysine sepharose. All other hemostatic parameters predisposing to recurrent thrombosis were normal. A stimulation test with desmopressin acetate (DDAVP) showed a normal plasma rise of both tissue plasminogen activator and factor VIIIR:WF. This isolated plasminogen deficiency apparently is due to a decreased synthesis of a normal plasminogen molecule and is associated with a severe thrombotic tendency.
Ovarian relapse in a young woman with acute lymphoblastic leukaemia
Authors: Heaton David C. , Duff Gerald B. 
Published: February 2007

AbstractBilateral ovarian relapse in a young woman with acute lymphoblastic leukaemia (ALL) was treated by oophorectomy and intensive chemotherapy. She remains well off therapy 97 months following diagnosis and 45 months from ovarian relapse.