bluuebunny/crossref_metadata_2025_split huggingface dataset:
Leukemic manifestation in a case of alpha‐chain disease with multiple polypoid intestinal lymphocytic lymphoma
Authors: Chang Chao‐Sung , Lin Sheng‐Fung , Chen Tyen‐Po , Liu Hong‐Wen , Liu Ta‐Chi , Li Chin‐Yang , Chao Mei‐Chyn , Wu Pei‐Lin 
Published: February 2007

AbstractWe describe a female aborigine from Taiwan with alpha‐chain disease associated with multiple polypoid intestinal lymphocytic lymphoma and leukemic manifestation. Initially, the patient experienced intermittent diarrhea, abdominal pain, and leukemic manifestation. No evidence of bone marrow involvement was found. Alpha‐chain protein was demonstrated in the serum. Gastroendoscopy and a series of radiographs of the small intestine revealed multiple polypoid tumors involving the entire length of the small intestine. Duodenal biopsy showed diffuse lymphocytic lymphoma. Immunohistochemical staining of tumor samples revealed features typical of alpha‐chain disease. Cytogenetic analysis showed the same abnormal karyotypes of neoplastic clones in intestinal tumor cells and in circulating leukemic cells. The data suggest that alpha‐chain disease can present initially with intestinal multiple polypoid lymphocytic lymphoma and leukemic manifestation without evidence of bone marrow involvement. The data also support the homing theory of lymphomas from mucosa‐associated lymphoid tissue. © 1992 Wiley‐Liss, Inc.
Autoimmune neutropenia following peripheral blood stem cell transplantation
Authors: Klumpp Thomas R. , Herman Jay H. , Macdonald John S. , Schnell Maryann K. , Mullaney Mark , Mangan Kenneth F. 
Published: February 2007

AbstractThe differential diagnosis of unexpected neutropenia following bone marrow transplantation includes several potentially life‐threatening complications including graft rejection, overwhelming infection, relapse of the underlying neoplasm, and intrinsic graft failure. However, a number of recent reports document that the differential diagnosis also includes autoimmune neutropenia, which, although potentially life‐threatening, often responds well to corticosteroids or splenectomy. Autoimmune neutropenia has been reported following both autologous and allogeneic bone marrow transplantation. Herein we report a 31‐year‐old woman who developed a rapidly falling neutrophil count 11 days following peripheral blood stem cell transplantation for non‐Hodgkin's lymphoma. A laboratory evaluation supported a diagnosis of autoimmune neutropenia, and the neutropenia resolved following treatment with steroids and granulocyte‐colony stimulating factor. © 1992 Wiley‐Liss, Inc.
Treatment with gallium nitrate: Evidence for interference with iron metabolism in vivo
Authors: Seligman Paul A. , Moran Patrick L. , Schleicher Rhoda B. , David Crawford E. 
Published: February 2007

AbstractGallium, when bound to transferrin, has been previously shown to cause tumor cell cytotoxicity by preventing cellular uptake of transferrin bound iron in vitro. Patients treated with constant infusion gallium nitrate for carcinoma show a rise in serum iron within 6 hr of the start of treatment. Serum iron returns to baseline by 24 hr post‐infusion. Atomic analysis of iron and gallium content of Sephadex G‐150 fractions of treatment sera indicate that about an equamolar amount of gallium and iron are associated with transferrin. These gallium and iron concentrations result in inhibition of transferrin mediated iron uptake in vitro, and in vivo allow for >90% saturation of transferrin with metal. All seven patients who completed two courses of gallium therapy exhibited hypochromic microcytic anemia (mean fall in hemoglobin 3.5 grams %). Evidence for red cell iron depletion was confirmed by an increase (mean 3.3‐fold) in zinc protoporphyrin levels. Since transferrin receptor increases on gallium treated iron requiring cells in vitro, we assessed cell surface transferrin receptor on peripheral blood lymphocytes by measuring fluorescent transferrin receptor antibody binding. A population of highly transferrin receptor positive cells peaks at 48 hr into the infusion. DNA analysis as well as double staining indicate the majority of transferrin receptor positive cells are unstimulated B lymphocytes. These studies provide the first documentation that constant infusion gallium treatment results in significant interference with iron metabolism and evidence for tissue iron depletion in vivo. These changes may correlate with therapeutic effects of gallium such as tumor response. © 1992 Wiley‐Liss, Inc.
Is persistent polyclonal b lymphocytosis caused by epstein‐barr virus? A study with polymerase chain reaction and in situ hybridization
Authors: Chow Kuan‐Chih , Witzig Thomas E. , Li Chin‐Yang , Nacilla Johnny Q. 
Published: February 2007

AbstractPrevious reports of patients with persistent polyclonal B lymphocytosis have found associations with female sex, cigarette smoking, HLA‐DR7 phenotype, and moderate elevation of peripheral blood polyclonal B lymphocytes. The presence of binucleated cells and atypical lymphocytes in the peripheral blood of these patients was highly suggestive of a viral infection, such as with the Epstein‐Barr virus. We report a 47‐year‐old asymptomatic woman who was incidentally found to have persistent polyclonal B lymphocytosis and serum IgG against virus capsid antigen (VCA) and Epstein‐Barr virus (EBV)‐associated nuclear antigen (EBNA) of EBV. The presence of EBV was investigated in the peripheral blood lymphocytes from this patient by in situ hybridization and polymerase chain reaction methods. EBV DNA was demonstrated in the lympohcyte fraction by polymerase chain reaction, and it was further located in lymphoid cells by in situ hybridization. These results indicate that persistent polyclonal B lymphocytosis is strongly associated with EBV. © 1992 Wiley‐Liss, Inc.
Angiodysplasia and von willebrand's disease type IIB treated with estrogen/progesterone therapy
Authors: Chey William D. , Hasler William L. , Bockenstedt Paula L. 
Published: February 2007

AbstractThe association between angiodysplasia and von Willebrand's disease was first reported in 1967. The cases reported to date have involved patients with type I and IIA von Willebrand's disease. We report a patient with type IIB von Willebrand's disease who suffered gastrointestinal bleeding attributable to gastric angiodysplasia. The patient underwent endoscopic electrocautery acutely and has been treated long‐term with estrogen/progesterone therapy. She has suffered no recurrent gastrointestinal bleeding at over 11 months of follow‐up. We suggest hormonal therapy as an alternative to repeated blood product transfusion or extensive surgical resection in patients with von Willebrand's disease and gastrointestinal bleeding from angiodysplasia. © 1992 Wiley‐Liss, Inc.
Evidence that NADPH‐dependent methemoglobin reductase and administered riboflavin protect tissues from oxidative injury
Authors: Hultquist Donald E. , Xu Feng , Quandt Kim S. , Shlafer Marshal , Mack Christopher P. , Till Gerd O. , Seekamp Andreas , Betz A. Lorris , Ennis Steven R. 
Published: February 2007

AbstractNADPH‐dependent methemoglobin reductase, first detected in erythrocytes sixty years ago, has subsequently been purified and characterized as a methylene blue reductase and a flavin reductase. The reductase plays no role in methemoglobin reduction under normal conditions, but its activity serves as the basis for the treatment of methemoglobinemia with methylene blue or flavin. On‐going studies demonstrate that this cytosolic protein is also present in liver and that its primary structure distinguishes it from other known proteins. The bovine erythrocyte reductase tightly binds hemes, porphyrins, and fatty acids with resulting loss of activity. Pyrroloquinoline quinone serves as a high‐affinity substrate of the reductase, suggesting that this naturally‐occurring compound may be a physiological substrate. The ability of the reductase to catalyze the intracellular reduction of administered riboflavin to dihydroriboflavin suggested that this system might be exploited to protect tissues from oxidative damage. This hypothesis was supported by our finding that dihydroriboflavin reacts rapidly with Fe(IV)O and Fe(V)O oxidation states of hemeproteins, states that have been implicated in tissue damage associated with ischemia and reperfusion. Preliminary studies demonstrate that, as predicted, administration of low concentrations of riboflavin protects isolated rabbit heart from reoxygenation injury, rat lung from injury resulting from systemic activation of complement, and rat brain from damage caused by four hours of ischemia. Data from these animal studies suggest that flavin therapy holds promise in protecting tissue from the oxidative injuries of myocardial infarction, acute lung injury, stroke, and a number of other clinical conditions. © 1993 Wiley‐Liss, Inc.
Hepatic iron stores and plasma ferritin concentration in patients with sickle cell anemia and thalassemia major
Authors: Brittenham Gary M. , Cohen Alan R. , McLaren Christine E. , Martin Marie B. , Griffith Patricia M. , Nienhuis Arthur W. , Young Neal S. , Allen Christopher J. , Farrell David E. , Harris John W. 
Published: February 2007

AbstractTo examine the relationship between hepatic iron stores and plasma ferritin concentration in individuals treated with red cell transfusion and iron chelation therapy, 37 patients with sickle cell anemia and 74 patients with thalassemia major were studied. In each patient, hepatic iron stores were measured by an independently validated noninvasive magnetic method, and plasma ferritin was determined by immunoassay. The correlation between hepatic iron and plasma ferritin was significant both in patients with sickle cell anemia (R = 0.75, P < 0.0001) and in those with thalassemia major (R = 0.76, P < 0.0001). Regression analysis showed no significant difference between the two groups in the linear relationships between hepatic iron stores and plasma ferritin. Considering all 111 transfused patients as a group, the coefficient of correlation between hepatic iron stores and plasma ferritin was highly significant (R = 0.76, P < 0.0001). Regression analysis found that variation in body iron stores, as assessed by magnetic determinations of hepatic iron, accounted for only ∼57% of the variation in plasma ferritin, suggesting that the remainder was the result of other factors, such as hemolysis, ineffective erythropoiesis, ascorbate deficiency, inflammation, and liver disease. The 95% prediction intervals for hepatic iron concentration, given the plasma ferritin, were so broad as to make a single determination of plasma ferritin an unreliable predictor of body iron stores. Variability resulting from factors other than iron status limits the clinical usefulness of the plasma ferritin concentration as a predictor of body iron stores. © 1993 Wiley‐Liss, Inc.
Postnatal changes in the quantities of globin chains and hemoglobin types in two babies with Hb H disease
Authors: McKie K. M. , Gu L.‐H. , Gu Y.‐C. , Huisman T. H. J. 
Published: February 2007

AbstractWe have studied two babies with Hb H disease from birth to about six months of age and analyzed the changes in the relative quantities of the five globin chains (α, β, Gγ, Aγ) and the four hemoglobins (Hb F, Hb A, Hb Bart's, Hb H) using different high performance liquid chromatography procedures. The types of Hb H disease were —(SEA)/‐α (3.7 kb) and —(Fil)/‐α (3.7 kb); the larger—(Fil) deletion includes the functional 2‐globin gene, explaining the higher chain level in the baby with the —(SEA)/‐α (3.7 kb) type. The functional hemoglobin level at birth (Hb A + Hb F) was 11 to 12 g/dl with 3 to 4 g/dl Hb Bart's (γ4). Only 5% of the “fast‐moving” hemoglobin was Hb H (β4). The level of Hb F at birth was low (less than 50% of the total Hb A + Hb F). After birth, the α and γ chain production decreases rapidly resulting in a severe anemia (total functional hemoglobin ∼7 g/dl) at 30 to 60 days postnatally, improving gradually to 8.5–9.5 g/dl at age of three months. The preferential formation of Hb A over Hb F at birth, and presumably prenatally, has the advantage that the level of the highly unstable Hb H is kept low; it also results in low levels of Hb F impairing the oxygen transfer capability of the fetal blood. © 1993 Wiley‐Liss, Inc.
Adult and childhood acute lymphocytic leukemia: Are they different diseases?
Authors: Mauer Alvin M. 
Published: February 2007

AbstractAge has long been recognized as an important factor in predicting response to treatment for acute lymphocytic leukemia (ALL). Specifically, the results of treatment of childhood ALL have been far superior to the treatment of what appears to be the same disease in adults. However, from an analysis of the clinical and biological prognostic factors known to be predictive in childhood ALL, there is a striking difference in their distribution in adults with ALL. It appears that there is a special form of ALL seen in children of some populations with a peak incidence of three to seven years. This treatment responsive leukemia appears to be different clinically, biologically, and epidemiologically from adult ALL. © 1993 Wiley‐Liss, Inc.
Plasminogen activators and their inhibitors in leukemic cell homogenates
Authors: Wada Hideo , Kumeda Yasurou , Ogasawara Zin , Ohiwa Michiaki , Kaneko Toshihiro , Tamaki Shigehisa , Ohno Toshiyuki , Kageyama Sinichi , Kobayashi Thoru , Deguchi Katsumi , Shirakawa Shigeru 
Published: February 2007

AbstractPlasminogen activator (PA) and PA inhibitor (PAI) were measured in homogenates of leukemia cells. Both PA and PAI levels were higher in non‐lymphoblastic leukemia than in lymphoblastic leukemia. The levels were below the sensitivity of determination in chronic myelocytic leukemia (CML) but showed significant increases in blast crisis (CML, bc). The level of the tissue type PA (t‐PA) antigen was highest in acute myelobtestic leukemia (AML) and that of the urokinase type PA (u‐PA) was highest in acute promyeiocytic leukemia (APL). The PAH antigen showed no marked cell specificity, but the PAI‐II antigen was markedly increased in myelomonocytic leukemia and acute monocytic leukemia (AMoL). From these findings, various PAs and PAIs are considered to be present in leukemia cells and to be involved in hemostatic disorders, thus they are of diagnostic value in leukemia. © 1993 Wiley‐Liss, Inc.
Comparison of cytogenetic and restriction fragment length polymorphism analyses for the detection of loss of chromosome material in clonal hemopoietic disorders
Authors: Abrahamson Gail M. , Rack Katrina , Oscier David G. , Fitchett Margaret , Buckle Veronica J. , Wainscoat James S. 
Published: February 2007

AbstractLoss of chromosome material, as manifested by monosomy or partial deletion, is commonly found in neoplastic cells. We have undertaken a systematic comparison of standard cytogenetic analysis and molecular analysis for the detection of such loss, using as a model loss of chromosome 7 in 72 patients with a clonal myeloid malignancy. A large number of probes was used to screen three regions of chromosome 7 for loss by restriction fragment length polymorphism (RFLP) analysis. There were nine cases in which loss of chromosome 7 was detected by both techniques, but seven in which loss was detected by only one of the methods, demonstrating the complementary nature of these two techniques. © 1993 Wiley‐Liss, Inc.
Tumor necrosis factor‐α overproduction in Fanconi's anemia
Authors: Schultz John C. , Shahidi Nasrollah T. 
Published: February 2007

AbstractVarious in vitro studies and clinical observations suggest that Fanconi's anemia (FA) patients are unable to detoxify adequately superoxide anions (O) released by activated phagocytes. Recent studies have shown that certain lymphokines such as tumor necrosis factor‐α (TNF‐α) and interferon‐γ (IFN‐γ) can significantly enhance O production by phagocytic cells. To ascertain lymphokine production in FA patients, we measured TNF‐α and IFN‐γ production in vivo and in vitro. TNF‐α was detected in the plasma of 16 of 18 FA patients with concentrations ranging from 6 to 131 pg/ml (mean 31 pg/ml). TNF‐α was detected in only one of 25 control (healthy donor) plasma, and the level was very low (7 pg/ml). IFN‐γ levels in normal and patient plasma were negligible. Spontaneous and phytohemagglutinin (PHA)‐induced production of IFN‐γ and TNF‐α by cultured peripheral blood mononuclear cells did not differ significantly between FA patients and normal controls. The significance of overproduction of TNF‐α in vivo in the pathophysiology of FA is discussed. © 1993 Wiley‐Liss, Inc.
Hemorrhagic pleural effusion and pleural thickening as a complication of chronic lymphocytic leukemia
Authors: Dhodapkar Madhav , Yale Steven H. , Hoagland H. C. 
Published: February 2007

AbstractWe report a case of pleural thickening and hemothorax in a 56‐year‐old male with B‐cell chronic lymphocytic leukemia (CLL). The association of this entity with CLL has not been previously reported. Awareness of this occurrence is essential to appropriate therapy for similar patients with CLL. © 1993 Wiley‐Liss, Inc.
Zinc abuse and sideroblastic anemia
Authors: Ramadurai Jayanthi , Shapiro Charles , Kozloff Mark , Telfer Margaret 
Published: February 2007

AbstractWe report the case of a young woman who presented with anemia and leukopenia. A bone marrow aspirate, revealed a marked excess of ringed sideroblasts. A detailed dietary history disclosed excessive zinc intake. High serum zinc and low serum copper concentrations were confirmed. After discontinuation of the zinc supplements, she made a complete hematologic recovery. This report emphasises the importance of trace metals and their rote in the development of hematologic abnormalities. © 1993 Wiley‐Liss, Inc.
Effect of warfarin on arterial thrombogenesis: Problems of monitoring
Authors: Kovacs Iren B. , Murphy M. , Barin E. , Rees G. M. 
Published: February 2007

AbstractPlatelet reactivity to shear stress and collagen and dynamic overall coagulation were measured in vitro from nonanticoagulated blood of 137 patients on warfarin. One hundred five matched, healthy subjects served as controls. Platelet reactivity to both stimuli and contribution of platelets to plasmatic coagulation were significantly inhibited in patients on warfarin. No correlation was found between platelet reactivity and the coagulation status assessed by the international normalized prothrombin time ratio (INR). Despite similar INR, platelet reactivity showed great individual variation. In 98 patients who were followed up for 3 months, measurement of platelet reactivity to shear stress could discriminate between those who had either bleeding or thromboembolic episodes. These findings suggest that monitored platelet function would help in individualizing oral anticoagulant regimens and hence would increase the benefit of therapy without the risk of bleeding complications. © 1993 Wiley‐Liss, Inc.
Selection of a new generation of orally active α‐ketohydroxypyridine iron chelators intended for use in the treatment of iron overload
Authors: Kontoghiorghes G. J. , Barr J. , Nortey P. , Sheppard L. 
Published: February 2007

AbstractThe prospect of selecting oral α‐ketohydroxypyridine chelators intended for clinical use in iron overload has been examined using several animal models of efficacy and toxicity. Studies using iron dextran‐loaded mice labelled with 59Fe have shown that only the 1‐substituted methyl, ethyl, (n)propyl, allyl, cyclopropyl, 2′‐methoxyethyl, 3′‐ethoxypropyl, or 2‐methyl‐ or 2‐ethyl‐ 3‐hydroxypyrid‐4‐one chelators were orally effective in increasing iron (59Fe) excretion by comparison to intraperitoneally administered desferrioxamine at the same dose (250 mg/kg). In contrast, chelators containing ‐H, mono‐ or dihydroxyalkyl and diethoxyethyl 1‐substituents caused very little or no increase in iron (59Fe) excretion by the oral or intraperitoneal routes. In vitro studies using ferritin and haemosiderin have shown that equivalent iron release took place with both groups of chelators irrespective of their in vivo effects. In most cases there was no correlation between the n‐octanol/water partition coefficient (Kpar) and iron removal efficacy but positive correlation between the lipophilicity and acute or subacute toxicity of these chelators in rats. The most toxic chelator in the chronic toxicity studies in rats was the lipophilic 1, 2‐diethyl‐3‐hydroxypyrid‐4‐one (EL1NEt). The most effective chelator in increasing iron excretion in mice and rabbits was 1‐allyl‐2‐methyl‐3‐hydroxypyrid‐4‐one (L1NAll), and the chelator with the highest safety margin in mice and rats was 1, 2‐dimethyl‐3‐hydroxypyrid‐4‐one (L1). Overall the oral effectiveness in increasing iron excretion by these chelators in animals does not appear to be related to their lipophilicity or their ability to mobilise polynuclear iron in vitro but rather to other properties possibly related to their rate of biotransformation and excretion. © 1993 Wiley‐Liss, Inc.
In vitro activity of chloroquine and quinine in combination with desferrioxamine against Plasmodium falciparum
Authors: Basco Leonardo K. , Bras Jacques Le 
Published: February 2007

AbstractThe activity of chloroquine and quinine, alone and in combination with desferrioxamine (7 μmol/liter), was evaluated in vitro against susceptible and resistant clones of Plasmodium falciparum by a semimicroassay system. The addition of desferrioxamine had no effect on the activity of chloroquine against both clones. Desferrioxamine had no effect on the activity of quinine against the susceptible clone but had slightly enhanced quinine action against the resistant clone. Further development of desferrioxamine as an antimalarial drug may be of limited interest. © 1993 Wiley‐Liss, Inc.
High incidence of meningeal leukemia in lymphoid blast crisis of chronic myelogenous leukemia
Authors: Saikia Tapan K. , Dhabhar Boman , Iyer R. S. , Nanjangud Gauri , Gopal R. , Nair Chandrika N. , Nadkarni Kanchan S. , Ashokkumar M. S. , Dhond Subodh R. , Advani Suresh H. 
Published: February 2007

AbstractFifteen patients with lymphoid blast crisis of chronic myelogenous leukemia (LyBC‐CML) and five patients with acute lymphoblastic leukemia converting to Philadelphia‐positive (Ph+) chronic myeloid leukemia (ALL Ph + CML) were followed. Seven of 15 (46.7%) LyBC‐CML patients developed meningeal leukemia within a median period of 6 months (range 2–11 months), while there was no medullary relapse. Five of these responded well to triple intrathecal therapy. In the ALL Ph + CML patients, in spite of central nervous system (CNS) prophylaxis with IT MTX and 18 Gy cranial radiation, two of five patients (40%) experienced meningeal leukemia, one isolated and the other with medullary relapse. The data confirm that LyBC‐CML patients experience a high incidence of meningeal leukemia. The role of CNS prophylaxis is not very clear, but its use may delay development and reduce morbidity due to CNS disease.