bluuebunny/crossref_metadata_2025_split huggingface dataset:
B‐cell acute lymphocytic leukemia in HIV‐antibody‐positive patients
Authors: Gold Jay E. , Castella Antonio , Zalusky Ralph 
Published: February 2007

AbstractB‐cell non‐Hodgkin lymphoma (NHL) has been well described in association with human immunodeficiency virus (HIV) and the acquired immunodeficiency syndrome (AIDS). Many of these lymphomas are of the diffuse, aggressive, subtype of B‐cell NHL, including Burkitt and Burkitt‐like lymphoma. Recently, there have been reports of B‐cell acute lymphocytic leukemia (ALL), Burkitt type, in patients who were either HIV antibody‐positive or at high risk for AIDS. We have seen three cases of B‐cell ALL, Burkitt type, and herein describe their clinical and laboratory characteristics. All patients were HIV antibody‐positive. Since stage IV Burkitt lymphoma in blood phase and B‐cell ALL, Burkitt type, represent a continuum of the same disease, and since it is also an aggressive B‐cell malignancy, we suggest that B‐cell ALL, Burkitt type, in HIV antibody‐positive patients should support the diagnosis of AIDS.
Alveolar cell carcinoma complicating sickle cell anemia: A chance occurrence?
Authors: Labi Marlon , Haponik Edward F. , Welsh Ronald A. , Summer Warren R. 
Published: February 2007

AbstractAlveolar cell carcinoma, a neoplasm associated with pre‐existing inflammatory lung disease, has not been reported previously in patients with sickle cell disease. We present such a patient and suggest that chronic pulmonary scarring in this setting predisposed to development of his malignancy. As survival increases in individuals with sickle cell disease, alveolar cell carcinoma must be considered in these differential diagnosis of otherwise unexplained chronic pulmonary infiltrates.
Successful treatment of refractory anemia with high‐dose methylprednisolone
Authors: Motoji Toshiko , Teramura Masanao , Takahashi Masatomo , Oshimi Kazuo , Okada Michiko , Kusakabe Kiyoko , Mizoguchi Hideaki 
Published: February 2007

AbstractFive patients with refractory anemia were treated with high‐dose methylprednisolone. An immediate and long‐lasting hematological improvement without serious side effects was achieved in two of the patients, although chromosomal abnormalities persisted in both. The clinical course and laboratory data of these two patients are described. Restoration of normal hematopoiesis was achieved regardless of the enhancement of colony formation of granulocyte progenitor cells by the simultaneous addition of hydrocortisone in vitro. The same treatment was given to six patients who had refractory anemia with an excess of myeloblasts (RAEB), but no improvement was observed in any of these patients. It appears that high‐dose methylprednisolone can be valuable in the treatment of refractory anemia, but is not useful for RAEB.
Evaluation of reticulocyte counts by flow cytometry in a routine laboratory
Authors: Ferguson David J. , Lee Siow‐Fong , Gordon Philip A. 
Published: February 2007

AbstractReticulocyte counting using flow cytometry and a membrane‐permeable fluorescent dye, thiazole orange, was evaluated as an alternative to the conventional manual method. The flow cytometric method was more precise (mean c.v. of 4.3%) than the imprecise manual technique (mean c.v. of 22.4%). Linearity was highly acceptable (r = 0.99) over the reticulocyte count range of 1.8–30.1%.Flow cytometry allows processing of large numbers of samples with reduction in technologist time, and the improved precision and tenfold increase in the number of cells counted should considerably improve the clinical utility of the reticuloctye count.
Thrombocytopenic purpura in a patient with lupus anticoagulant: Requirement of both immunosuppressive and antithrombotic therapies
Authors: Iwahara Yoshihito , Niiya Kenji , Yamato Kenji , Miyoshi Lsao 
Published: February 2007

AbstractA 33 year old woman with severe thrombocytopenic purpura complicated by typical lupus anticoagulant developed repeated spontaneous abortion, deep venous thrombosis, and cerebral thrombosis. The platelet count fluctuated from 4,000 to 400,000/mm3 during the 13 year clinical course. The physical and laboratory findings at the time of severe thrombocytopenic purpure were compatible with the criteria of idiopathic thrombocytopenic purpura except for positive lupus anticoagulant. Both immunosuppressive therapy with prednisolone and antithrombotic therapy with warfarin and aspirin were necessary for the control of bleeding and venous and arterial thrombosis.
Sickle hemoglobinopathies in sicily
Authors: Schilirò G. , Spena M. , Giambelluca E. , Maggio A. 
Published: February 2007

AbstractThe clinical and hematological features of 202 Sicilian subjects with sickle cell disease are reported, 41 being homozygous for betaS (βSβS), 64 with betas thal betaS (βsβS), and 97 beta+ thal betaS (β+ βS). Analysis of the findings showed that the disease observed in Sicilians is of intermediate severity and falls between the severe form observed in patients of African origin and the milder one seen in subjects of Arabian origin.
Thrombin Vs. Plasmin generation in disseminated lntravascular coagulation associated with various underlying disorders
Authors: Takahashi Hoyu , Tatewaki Wataru , Wada Ken , Hanano Masaharu , Shibata Akira 
Published: February 2007

AbstractIn order to assess the thrombin and plasmin generation in vivo in disseminated intravascular coagulation (DIC), plasma levels of thrombin‐antithrombin III (ATIII) complex (TAT) and plasmin‐alpha 2‐antiplasmin (a2AP) complex (PAP) were measured together with standard coagulation and fibrinolytic parameters in 80 patients with DIC. Both TAT and PAP were markedly elevated in patients with DIC. When plotted by the underlying disease categories, differences in the magnitude of the elevations of these complexes were recognized among groups. Patients with acute promyelocytic leukemia (APL) had the highest PAP, the lowest TAT/PAP ratio, low a2AP, and low fibrinogen, indicating that the most excessive fibrinolysis can occur in APL. Similar profiles, although less marked, were observed in patients with other leukemias and vascular diseases. Patients with sepsis showed the highest TAT/PAP ratio and the lowest PAP with no decrease in a2AP or fibrinogen, demonstrating a relatively impaired fibrinolysis. Patients with cancer had a relatively high TAT and high TAT/PAP ratio. In addition, both TAT and PAP were markedly elevated in patients with shock. From these, it was suggested that, although laboratory manifestations in DIC are extremely variable from patient to patient, underlying disorders are, at least in part, responsible for the observed variations. Recognition of this variable activation of coagulation and fibrinolysis would be helpful for the proper management of patients with DIC.
The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia
Authors: Benbassat Jochanan , Gilon Dan , Penchas Shmuel 
Published: February 2007

AbstractThe objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myelod metaplasia (AMM). We searched the literature (Medline, 1970‐1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or transfusion requirements in patients with AMM. We employed formal decision analysis to determine the relative value of medical and surgical treatment of advanced AMM.Results of data synthesis showed that splenectomy in AMM is associated with an operative mortality of 13.4% (95% confidence intervals (Cl): 9.5‐1 ‐17.2%), an early morbidity of 45.3% (Cl: 39.6‐51.1%), and a late morbidity of 16.3% (Cl: 9.9‐22.5%). Almost all patients with portal hypertension and painful splenomegaly, but only about half of those with thrombopenia and anemia were reported to have experienced relief in their symptoms or signs after splenectomy. We found no evidence that splenectomy affects survival in AMM.We concluded that splenectomy in advanced AMM is a palliative procedure that carries a substantial risk. It may be considered for symptomatic patients after they have been informed about the operative mortality, morbidity, and chances of palliation. Decisions about treatment of advanced AMM should be guided predominantly by the patient's preferences.
Decreased tetranectin in multiple myeloma
Authors: Nielsen Henrik , Clemmensen Inge , Nielsen Hans J. , Drivsholm Aage 
Published: February 2007

AbstractTetranectin, a recently described human protein widely distributed in the body and with possible importance for cell growth and differentiation, has previously been observed to be decreased in patients with solid malignant tumors. In patients with multiple myeloma, either untreated or previously treated, serum levels were found to be significantly reduced. A negligible interindividual variation was observed. Levels of serum tetranectin were not correlated with serum albumin, hemoglobin, or M‐component. Low levels of tetranectin may be related to the growth and spread of malignant cells or reflect a general catabolic state of chronic disease.
Brief report: Pure red cell aplasia following pernicious anemia
Authors: Dan Kazuo , Ito Toshiharu , Nomura Takeo 
Published: February 2007

AbstractA rare case of pure red cell aplasia (PRCA) in association with pernicious anemia is reported. A 40‐year‐old man presented with typical clinical and laboratory features of pernicious anemia and received intramuscular injections of vitamin B12, with satisfactory response. Anemia recurred 6 months later despite continued therapy, and the patient was noted to have PRCA, which was treated successfully with two courses of high‐dose bolus methylprednisolone therapy. His peripheral mononuclear cells before the therapy suppressed colony formation of early erythroid precursors (BFU‐E) from normal bone marrow; such a suppressive effect was not found after recovery from anemia.
Simple and rapid enzyme‐linked immunosorbent assay for the detection of hemoglobin C[α2β26(A3)Glu → Lys] in cord blood using a monoclonal antibody
Authors: Shyamala M. , Kiefer Charles R. , Moscoso Hugo , Garver Fred A. 
Published: February 2007

AbstractWe have generated a murine hybridoma that secretes a monoclonal antibody (mAb) that is highly specific for hemoglobin C (HbC) [α2ß26(A3)Glu→Lys] and shows no cross reactivity with HbA2, HbF2, HbS, HbE, or Hb O‐Arab. Using this antibody, we developed a simple and rapid enzyme linked immunosorbent assay (ELISA) technique for the detection of HbC in both adult and cord blood. The assay can be carried out using either whole blood samples or hemolysates. With as little as 10 μl/well of whole blood or 5 μg Hb/well of hemolysates, and, with dilutions of the antibody up to 10−5 we were able to detect HbC unequivocally in cord blood samples. The ELISA procedure could detect HbC in proportions as low as 0.01%. This simple diagnostic test represents a technological advance in Hb identification and can easily be used for mass screening (96 samples in less than 45 min) to detect HbC. Furthermore, this assay, when employed in conjunction with an mAb specific for β6GLU of HbA, allows the discrimination between HbC homozygotes, heterozygotes, and normals.
Apparent duplication of inv(3)(q21q26) in one of five cases with inv (3) in myelodysplastic syndromes and acute leukemia
Authors: Walter Thomas A. , Morgan Rodman , Ondreyco Sharon , Sandberg Avery A. 
Published: February 2007

AbstractRearrangements affecting the long arm of chromosome 3 have been linked to abnormalities of platelet production and morphology in hematologic disorders. We present five patients with myelodysplastic syndromes or acute leukemia in whom inv(3)(q21q26) was seen. One of the patients showed an inversion of both homologs involving precisely the same breakpoints, suggesting apparent duplication of the abnormal chromosome 3 and loss of the normal homolog. To our knowledge this is the first report detailing such a condition.
Enhanced botrocetin‐induced type iib von willebrand factor binding to platelet glycoprotein ib initiates hyperagglutination of normal platelets
Authors: Nishio K. , Fujimura Y. , Niinomi K. , Takahashi Y. , Yoshioka A. , Fukui H. , Usami Y. , Titani K. , Ruggeri Z. M. , Zimmerman T. S. 
Published: February 2007

AbstractBotrocetin, a protein isolated from the venom of the snake Bothrops jararaca, induces platelet aggregation/agglutination by von Willebrand factor (vWF) binding to the membrane glycoprotein (GP) Ib, an action resembling that of ristocetin. However, some differences in the interaction between vWF and platelet GPIb induced by these two substances have been reported.We have recently shown that the GPIb binding domain on the vWF molecule, in both instances, resides in the tryptic 52/48 kDa fragment extending from amino acid residue 449 to 728 of the constituent subunit. In the present report, we demonstrate that botrocetin does not induce agglutination of formalin‐fixed platelets from a patient with Bernard‐Soulier syndrome congenitally lacking GPIb and GPIX as well as GPV, a finding similar to that shown with ristocetin. A monoclonal antibody against GPIb (AP‐1) inhibits either ristocetin‐ or botrocetin‐dependent vWF binding to formalin‐fixed platelets from normal individuals. Therefore, botrocetin‐induced vWF binding to formalin‐fixed platelets may reflect the interaction between vWF and platelet GPIb. To strengthen this concept, we have now found that heightened botrocetin‐induced type IIB vWF binding to platelet GPIb causes hyperagglutination of normal platelets.
Enhancement of sickle erythrocyte adherence to endothelium by autologous platelets
Authors: Antonucci Richard , Walker Richard , Herion John , Orringer Eugene 
Published: February 2007

AbstractThe increased adhesiveness of sickle erythrocytes (SS RBC) to endothelial cells has been confirmed in a static system utilizing fresh umbilical vein endothelium. Adherence of SS RBC to the endothelium was as great in the presence of calcium‐containing buffer as when incubated in plasma. SS RBC suspended in autologous platelet‐rich plasma adhered to a greater extent than when suspended in autologous platelet poor plasma. Prostacyclin, thromboxane B2, and an inhibitor of collagen‐ and epinephrine‐induced platelet aggregation (B13.177) did not affect SS RBC adherence to endothelium. Aspirin in concentration of 5 μg/ml slightly decreased SS RBC adherence to endothelium in the presence of platelats may play a significant role in the increased adhesiveness of SS RBC to endothelium. To the extent that increasede SS RBC adhesiveness contributes to the genesis of painful crises and to the extent platelets augment this adhesiveness, agents affecting platelet function may prove useful in preventing painful crises.
Cyclosporin for treatment of life‐threatening alloimmunization
Authors: Tilly Hervé , Azagury Michael , Bastit Dominique , Lallemand Anne , Piguet Hubert 
Published: February 2007

AbstractWe describe a 16‐year‐old girl with aplastic anemia who, 1 year after initial diagnosis, developed a refractory state to platelet transfusions due to alloimmunication and resulting in severe bleeding. Treatment with cyclosporin, initially prescribed as treatment of the bone marrow failure, resulted in prompt decrease in lymphocytotoxic antibodies, which paralleled a marked improvement in platelet recovery. To our knowledge, such a dramatic effect of cyclosporin on alloimmunization has not been previously reported and merits further attention.
Anti‐leukemic potential of methyl‐cobalamin inactivation by nitrous oxide
Authors: Abels J. , Kroes A. C. M. , Ermens A. A. M. , Van Kapel J. , Schoester M. , Spijkers L. J. M. , Lindemans J. 
Published: February 2007

AbstractMyelo‐cytotoxicity of extended nitrous oxide (N2O) inhalation was described almost forty years ago and then incidentally applied already with temporary success for suppressing leukemia. In 1948 the accompanying megaloblastic maturation arrest was explained by inactivation of the methylcobalamin coenzyme and subsequent folate deficiency. We studied the anti‐leukemic effect of N2O on a transplantable acute leukemia in B(rown) N(orway) rats. Progression of this B,N,M(yelocytic)L(eukemia) was measured as spleen and liver weights, and leukemic blood cell counts. The deoxyuridine (dU)‐suppression test provided in vitro indication of the functional folate activity of leukemic cells. Breathing of N2O‐oxygen considerably reduced but did not eradicate, BNML‐proliferation. Addition of anti‐metabolites, interfering with some enzyme in the folate metabolism beyond the methylcobalamin co‐enzyme dependent methionine synthase step, acted at least synergistically. The anti‐leukemic effect of cycloleucine, which reduces S‐adenosyl‐methionine synthesis by inactivation of methionine adenosyltransferase, was moderate but became much stronger with N2O inhalation. Methotrexate, a potent anti‐leukemic agent by inhibiting tetrahydrofolate (THF) generation through inactivation of di‐HF reductase, became highly anti‐BNML, even in low dosage when combined with or preceded by N2O. 5‐Fluorouracil, which inhibits methylene‐THF dependent thymidilate synthase, itself was surprisingly anti‐BNML, but also became much more potent with previous or concomittant N2O exposure. Preliminary dU‐suppression test results with human acute leukemia cells, exposed to N2O and/or folate antagonists in vitro, correlated well with the in vivo BNML‐experiments. Combining the anticobalamin activity of N2O with an anti‐folate therefore seems to be a promising chemotherapeutic approach.
Familial adult T‐cell leukemia/lymphoma
Authors: Ratner Lee , Heyden Nancy Vander , Paine Elizabeth , Frei‐Lahr Debra , Brown Randy , Petruska Paul , Reddy Satyanarayan , Lairmore Michael D. 
Published: February 2007

AbstractClinical and laboratory data are described for two siblings who both developed adult T‐cell leukemia/lymphoma resulting from infection by human T lymphotropic virus type I (HTLV‐I). These findings suggest that genetic factors or virus‐specific factors may determine which HTLV‐I‐infected individuals will develop leukemia.
Infant leukemia: An analysis of nine chinese patients
Authors: Chuu Wen‐Min , Tien Huei‐Fang , Lin Dong‐Tsamn , Lin Kai‐Hsin , Su Ih‐Jen , Chen Bow‐Wen , Lin Kuo‐Sin , Liang Der‐Cherng 
Published: February 2007

AbstractA study was made of the cellular and molecular characteristics of nine Chinese infants, consecutively presenting with acute leukemia. Five cases were acute lymphoblastic leukemia (ALL); four were acute nonlymphoblastic leukemia (ANLL). Hyperleukocytosis, hepatosplenomegaly, and poor response to conventional therapy were common features, and CNS involvement was detected at diagnosis in three cases. The blast cells from all five cases with ALL expressed early B‐cell markers, i.e., HLA‐DR+, CD19 +, but CD10‐. Terminal deoxynucleotidyl transferase (TdT) was present in blasts from four of the five cases and periodic acid‐Schiff staining in blasts from two patients only. The leukemic cells of one patient also showed positive nonspecific esterase activity and expressed myeloid‐associated antigens CD33 (My9), CD11 (OkM1), and CD14 (My4 and Mo2). Molecular analysis of leukemic cell DNA from this and two other patients showed rearrangement of the immunoglobulin (Ig) heavy‐chain genes, but without any evidence of kappa light‐chain gene rearrangement. T‐cell receptor (TCR) genes remained in the germline configuration in these cases. Cytogenetic analysis showed translocation t(4;11) (q21;q23) in all four cases studied. In the group of ANLL, three cases belonged to the M4 and one to the M2 subtype. Chromosomal abnormality involving 11q23 was also detected in two patients: t(11;17)(q23;q11) and del(11)(q14q23) in each case respectively. Neither lg nor TCR gene rearrangement was present in blast cells from patients with ANLL. The data indicate that chromosomal rearrangement of band 11q23 was quite common in Chinese infants with either form of leukemia, a finding that may have pathogenetic implications.
Iron overload in chinese patients with hemoglobin H disease
Authors: Hsu Hui‐Chi , Lin Chung‐King , Tsay Shyh‐Haw , Tse Elizabeth , Ho Chao‐Hung , Chow Mo‐Ping , Yung Chien‐Hui , Peng Hong‐Wen 
Published: February 2007

AbstractWe examined the iron status of 23 adult patients with hemoglobin H (Hb H) disease. None of them had received multiple blood transfusions or prolonged iron therapy. Studies included serum iron and ferritin concentrations, transferrin saturation, a desferrioxamine test, computed tomography (CT) scan of the liver, and liver biopsy. Iron overload was found in 17 patients (73.9%), especially in males and in patients with splenomegaly (92.9% and 100%, respectively). Four patients with excessive alcohol consumption had clinical manifestations of severe iron overload. Idiopathic hemochromatosis associated HLA antigens, i.e., HLA‐A3, ‐B7, or ‐B14, were not found in any of the 15 patients tested. These findings indicate that iron overload is common in adult patients with Hb H disease; such patients should abstain from alcohol and be considered for treatment with an iron chelating agent before irreversible organ damage occurs.
Recent progress in the molecular genetic analysis of erythroenzymopathy
Authors: Fujii Hisaichi , Miwa Shiro 
Published: February 2007

AbstractDuring the relatively recent period in which normal genes for most red cell enzymes have been isolated, the techniques of molecular biology have been applied to the studies of erythroenzymopathy. Single nucleotide substitutions have been identified in aldolase, triosephosphate isomerase, glucose 6‐phosphate dehydrogenase, and adenylate kinase variants by the cloning and nucleotide sequence of the patients' genes. Up to now, all of the enzyme‐deficient variants which have been investigated have been caused by point mutations. An exception is a hemolytic anemia secondary to increased adenosine deaminase (ADA) activity. Red cell ADA activity increases on the order of a hundred‐fold in affected individuals. The basic abnormality appears to result from overproduction of structurally normal enzyme due to abnormal transcriptional or translational efficiency.
Role of heme metabolism in AZT‐induced bone marrow toxicity
Authors: Lutton J. D. , Mathew A. , Levere R. D. , Abraham N. G. 
Published: February 2007

AbstractWe studied the effects of azidothymidine (AZT) on rat bone marrow heme metabolism and colony growth as determined by assays of granulocyte‐macrophage (CFU‐GM), erythroid (CFU‐E), burst‐forming erythroid (BFU‐E), and α‐aminolevulinic acid synthase (ALAS), the first enzyme in the heme pathway. In all cases, AZT (1–0.01 μM) was found to be toxic to bone marrow colony growth. When AZT was included in colony assays, 1 μM resulted in 98–100% inhibition, whereas lower concentrations (0.01 μM) inhibited growth by 58–76%. In addition, cultures from AZT‐treated animals had a marked reduction in colony growth as compared with sham controls. In most cases, hemin (10−5 M) was found to overcome some of the colony inhibitory effects of AZT. Analysis of heme metabolism indicated that ALAS activity was reduced by 71% in bone marrow cells from treated animals. ALAS activity for control was 204 ± 33 pM ALA formed/4 ± 106 cells/hr, whereas ALAS activity from AZT‐treated animals was only 60 ± 3 pM ALA formed/4 ± 106 cells/hr. It is considered that AZT toxicity may be due in part to a depression in the pool of available heme, which is required for adequate hematopoiesis.