bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prolymphocytic leukemia treated with natural and recombinant α‐interferon
Authors: Terashima Takeshi , Ohtake Ken‐Ichi , Ogawa Tetsuhei 
Published: February 2007

AbstractThe patient was a 65 year old woman with a massive splenomegaly and no lymphoadenopathy. An electron microscopic study of the peripheral blood cells revealed that these cells were prolymphocytes with a single large nucleolus. Treatment with native and recombinant α‐interferon has produced an impressive reduction in spleen size and in the count of prolymphocytes.
Type‐C virus‐like particles in a human B‐cell lymphoma cell line
Authors: Kubonishi Ichiro , Sawada Takashi , Yano Shoki , Eguchi Taisuke , Iwahara Yoshihito , Takeuchi Chise , Miyoshi Isao , Ohtsuki Yuji 
Published: February 2007

AbstractType‐C virus‐like particles (VLPs) were found in an Epstein‐Barr (EB) virus‐infected human B‐cell lymphoma cell line, SP‐50B, that was established from a patient with non‐Hodgkin lymphoma. The cell line continuously produces a small number of type‐C VLPs, 150–200 nm in diameter, over 1 year. SP‐50B cells were negative for HTLV‐I and HTLV‐II antigens and did not contain the HTLV‐I genome. In addition, two EB virus nuclear antigen (EBNA)‐positive B‐cell lines, SP‐54‐Cord and SP‐57‐CLL, were established from human cord blood and chronic lymphocytic leukemia (CLL), respectively, by coculture with lethally irradiated SP‐50B cells. Type‐C VLPs with the same morphology were also found in both cell lines.
Transformation of polycythemia vera to ph‐positive chronic myelogenous leukemia
Authors: Haq Amin U. 
Published: February 2007

AbstractA patient with polycythemia vera (PV) developed chronic myelogenous leukemia (CML) 8 years after his initial presentation with erythrocytosis. The patient received a total of 3.5‐7 g of chlorambucil over an approximate period of 5 years. Transformation of PV to CML is very unusual; only three substantiated cases had been reported in literature until 1975, and none has been described since then. All patients received either 32P or chemotherapy for PV treatment, suggesting that irradiation and alkylating agents may have played a role in the transformation to CML.
Lack of response of bone marrow, in vitro, to growth factors in congenital neutropenia
Authors: Chang James , Coutinho L. H. , Dexter Michael , Craft Alan W. , Reid Michael M. 
Published: February 2007

AbstractSevere congenital neutropenia has a poor outlook. In vitro clonogenic assays using recombinant growth factors may improve understanding of the underlying pathogenetic mechanisms and identify those in whom growth factors might be clinically useful. Marrow from a boy with congenital neutropenia was cultured with a variety of recombinant growth factors. The results show that the neutropenia did not result from a lack of myeloid progenitors but that these progenitors could not produce mature neutrophils. Bone marrow transplantation is being considered as the most likely approach to correct neutropenia.
Serum and red blood cell magnesium, copper, and zinc content in G6PD deficiency
Authors: Karunanithy R. , Ng S. E. , Saha N. 
Published: February 2007

AbstractSixty‐nine G6PD‐deficient and 67 normal non‐deficient Chinese adult males were investigated for blood hemoglobin concentration and for serum and red cell content of magnesium, copper, and zinc. There was no significant difference in blood hemoglobin content. Both the serum magnesium and copper content were found to be significantly lower in the G6PD‐deficient subjects compared to that in the non‐deficient control. Serum zinc content and red cell content of all the three metals were not significantly altered in G6PD deficiency.
Regulation of Tissue Plasminogen Activator in Sickle Cell Anemia
Authors: Phillips George , Hartman John , Keller V. Antoine , Santiago Manuel A. , Pizzo Salvatore 
Published: February 2007

AbstractEvidence of activation of the clotting system in individuals with sickle cell anemia (SCA) has been observed by several investigators. It has been suggested that the clotting and fibrinolytic systems may play a role in the pathophysiology of vaso‐occlusion in SCA. We reported previously evidence of abnormal fibrinolytic activity as reflected in decreased releasable tissue plasminogen activator (t‐PA) using a functional assay. We have examined the mechanism of the decreased functional releasable t‐PA in individuals with SCA. We studied 12 patients with respect to releasable t‐PA, fast acting inhibitor to t‐PA (or PAI‐1), and immunoreactive or antigenic t‐PA. These SCA individuals were at their baseline states and not taking medications known to interfere with the fibrinolytic or clotting systems. We found that the mean releasable t‐PA for the SCA individuals was 0.01 IU/ml of plasma with a standard error of mean (SEM) of 0.01. The mean releasable t‐PA of 118 healthy normal controls was 0.70 IU/ml with SEM 0.10 (P < 0.001). The mean level of fast‐acting inhibitor to t‐PA in unoccluded circulation of the SCA patients' plasma was 16.5 IU/ml with SEM of 3.54. The mean plasma levels of fast‐acting inhibitor to t‐PA in 56 healthy controls was 2.56 IU/ml with SEM of 0.29 (P < 0.0001). The SCA patients had a mean baseline t‐PA antigen level of 5.98 ng/ml with SEM of 1.72. The mean level of t‐PA antigen of 78 healthy controls using the same technique was 4.3 ng/ml with SEM of 2.7 (not significant). The mean baseline functional t‐PA for SCA individuals was 0.15 IU/ml with SEM 0.01 and the mean baseline functional t‐PA for 118 controls was 0.17 IU/ml with SEM 0.10. These data suggest that the mechanism of decreased releasable t‐PA in sickle cell anemia is related to an elevation of fast‐acting inhibitor to t‐PA and that antigenically t‐PA is present in normal quantities in the baseline plasma in this population.
Neonatal alloimmune thrombocytopenic purpura associated with sensitization against the platelet‐specific antigen Yuka
Authors: Matsuda Tadashi , Yonaha Hisashi , Azuma Eiichi , Sakurai Minoru , Imai Shigemi 
Published: February 2007

AbstractWe report a Japanese newborn who developed alloimmune thrombocytopenia by the antibodies to the newly discovered platelet antigen Yuka. The infant recovered uneventfully in 10 days without specific treatment. Antiplatelet ailoantibodies in the patient were IgG class detected by mixed passive hemagglutination (MPHA). Family study showed that Yuka antigen was inherited as an autosomal dominant trait. Cases with Yuka‐associated alloimmune thrombocytopenia are reviewed.
Congenital factor XIII deficiency associated with von willebrand disease
Authors: Grand B. , Blanco A. , Riveros D. , Ventura A. , Maugeri N. , Woods A. , Lazzari M. 
Published: February 2007

AbstractA boy with umbilical bleeding and severe hemorrhages after minor trauma, without family bleeding history, was studied. Coagulation tests showed abnormalities in FXIII subunits and FVIII/vWF complex. Both parents presented results compatible with a heterozygote state for FXIII deficiency and the father had abnormalities of FVIII/vWF. The propositus was diagnosed as congenital FXIII deficiency associated with vWD. No severe hemorrhagic complication was observed after a prophylactic regimen with cryoprecipitates.
Acute myocardial infarction, non‐bacterial thrombotic endocarditis, and disseminated intravascular coagulation in a severe hemophiliac
Authors: Green David , Snapper Howard , Abu‐Jawdeh Graziella , Reddy Janardan 
Published: February 2007

AbstractThrombosis in hemophilia is very rare and is usually associated with the administration of prothrombin complex concentrates. We describe a severe hemophiliac with P. carinii pneumonia who had clinical and laboratory evidence of acute myocardial infarction and disseminated intravascular coagulation, and at autopsy, nonbacterial thrombotic endocarditis as well. We suggest that prothrombin complex concentrates should be used cautiously in the setting of acute infection, and perhaps be given with appropriate doses of anticoagulants such as heparin.
Ph chromosome in a patient with non‐leukemic non‐hodgkin B‐cell lymphoma
Authors: Fujii Hiroshi , Yashige Hiromi , Misawa Shinichi , Tanaka Shinji , Urata Yohji , Matuyama Fumio 
Published: February 2007

AbstractA standard Philadelphia translocation, t(9;22) (q34;q11), was found in lymph node cells from a patient with non‐leukemic non‐Hodgkin lymphoma at the time of diagnosis. The rearrangement of the breakpoint cluster region (bcr) was not detected with a bcr‐3′ probe. The neoplastic clone was of monoclonal B‐cell character with E‐, CD5‐, CD10‐, CD13‐, CD19+, CD20+, CD21+, CD25‐, HLA DR+, and positive surface lg(kappa). The patient showed no evidence of chronic myelogenous leukemia.
The size and number of bone marrow megakaryocytes in malignant lymphoma and their relationship to abnormalities in platelet count
Authors: Gladwin A. M. , Martin J. F. , Trowbridge E. A. , Slater D. N. , Reardon D. 
Published: February 2007

AbstractPlatelet counts were measured routinely in 718 volunteer blood donors and 124 patients with malignant lymphoma. When considered by gender, the mean platelet counts of the patients with malignant lymphoma were significantly increased when compared to controls (P < 0.01). In addition, 9% of males and 4% of females with lymphoma exhibited a thrombocytosis, in which their platelet counts exceeded the upper limit of the normal platelet count for control subjects (defined as 2 SDs in excess of the mean value). Megakaryocyte whole size, cytoplasmic and nuclear sizes, and megakaryocyte numbers were also quantified in bone marrow obtained from the iliac crest of 5 hematologically normal subjects and 26 patients with malignant lymphoma. Mean megakaryocyte sizes were significantly greater in subjects with lymphoma, when compared to control values (P < 0.05). No significant differences in megakaryocyte numbers in bone marrow were observed between the two groups. For subjects with malignant lymphoma, platelet count was found to be significantly positively correlated with megakaryocyte size. The results of this study suggest that the elevated platelet count associated with malignant lymphoma is mediated by an increase in the size of the megakaryocytes in the bone marrow.
Absence of natural killer cells in a child with pure red blood cell aplasia
Authors: Abrahamov Ayala , Schlesinger Michael 
Published: February 2007

AbstractA 16‐year old boy was known to suffer from red blood cell (RBC) aplasia from the age of 4 years. Peripheral blood lymphocytes (PBL) from the patient were found to lack natural killer (NK) cytotoxic activity, even after stimulation with α‐interferon. His PBL also lacked Leu‐7+ and Leu‐11+ cells, although his granulocytes showed normal expression of the Leu‐11 marker. The lack of NK cells did not seem to result from the various immunosuppressive treatments he received, since the NK deficiency was noted 2 years after he stopped receiving such treatment. The possibility is discussed that lack of NK cells may lead to the development of RBS aplasia if NK cells play a role in promoting the production of RBC.
G‐CSF enhanced granulocyte production in a child with severe neutropenia in the setting of fatal virus‐associated hemophagocytic syndrome
Authors: Azuma Eiichi , Tabata Nobutada , Shibata Takeo , Komada Yoshihiro , Ito Masahiro , Sakurai Minoru , Atsumi Shin‐Ichiro , Kawasaki Yoko , Ishii Masahiko 
Published: February 2007

AbstractA 14‐year‐old boy with fatal varicella zoster virus‐associated hemophagocytic syndrome (VAHS) was treated with recombinant human granulocyte colony‐stimulating factor (G‐CSF) based on the finding that the patient had severe neutropenia and possible bacterial superinfection. Support for the G‐CSF therapy in VAHS is provided by the recent reports that G‐CSF is relatively specific for the granulocyte lineage; it would not activate mature monocyte/macrophage/histiocyte lineage in VAHS, where the most striking morphologic feature is histiocytic hyperplasia with hemophagocytosis. He responded well to G‐CSF with an elevation of neutrophil counts. There were no effects on other blood cells. The result indicates that G‐CSF is useful to increase granulocyte production in severe neutropenia, even in the setting of fatal VAHS.
Protein S and C alterations in acutely III patients
Authors: Sheth S. B. , Carvalho A. C. 
Published: February 2007

AbstractProtein C, a potent vitamin K‐dependent protein activated by an endothelial cell cofactor, thrombomodulin, has anticoagulant and profibrinolytic activity. Free protein S, a cofactor for protein C, potentiates protein C activity at the endothelial cell surface. Pulmonary thromboemboli are a consistent finding in adult respiratory distress syndrome (ARDS). To determine if protein S or protein C were affected by widespread endothelial cell damage in ARDS, we measured bound and free protein S levels and protein C antigenic and functional levels in 18 patients with acute lung injury, 6 critically ill patients without lung history, and 22 normal subjects. Free (PS:F) and bound (PS:Ag) protein S and protein C antigen (PC:Ag) levels were measured using an enzyme‐linked immunoassay and protein C function (PC:Fn) by measuring its anticoagulant activity. We found a significant decrease in bound and free protein S levels of both patient groups in comparison to normal and a shift toward the inactive, bound protein S form. In addition, a significant decrease in free protein S compared to bound protein S in both patient groups was observed. While both PC:Ag and PC:Fn were significantly reduced compared to normal, the PC:Fn was significantly and severely decreased out of proportion to the PC:Ag in both patient groups. There was no difference between those with and without lung injury for both protein S and protein C. Analyzed according to etiology of lung injury, there was no difference in the bound and free protein S, nor in PC:Ag and PC:Fn levels between patients with sepsis and trauma. However, there were significant decreases in both protein S and protein C levels compared with normal subjects. Levels of both PS and PC levels in patients who survived did not differ from those who died. In summary, our data show that both protein S and C are markedly deranged in acutely ill patients who suffered from either sepsis or trauma, and these changes are independent of lung injury. The marked reductions in functional activity of PS and PC may be contributing factors to the thromboembolic complications often observed in these patients.
Effective therapy of human immunodeficiency virus‐associated anemia with recombinant human erythropoietin despite high endogenous erythropoietin
Authors: Dacosta Noshir A. , Hultin Mae B. 
Published: February 2007

AbstractA 32‐year‐old woman with human immunodeficiency virus (HIV) infection and progressive anemia presented to University Hospital with a hemoglobin of 3.4 g/dl. Because of her religious beliefs, she refused transfusion, and no iron or vitamin deficiency was found. She responded to recombinant human erythropoietin 150 U/kg intramuscularly thrice weekly with a rise in hemoglobin to 9.3 g/dl by 3 months of treatment. The serum erythropoietin level before treatment was markedly elevated at 1,340 mU/ml.
Modulation of tissue plasminogen activator biosynthesis by phosphatidylinositol liposomes in human fetal lung fibroblasts
Authors: Floru S. , Gelvan A. , Maran R. , Kadouri A. , Cohen A. M. 
Published: February 2007

AbstractPhosphatidylinositol (PI) liposomes at 40 μM increased tissue plasminogen activator (t‐PA) biosynthesis by human fetal lung fibroblasts IMR‐90 (FLF), after 5 days of incubation by 7.4 ± 1.4 times of the control level. Other phospholipid liposomes, such as phosphatidylserine (PS), phosphatidylcholine (PC), and phosphatidylglycerol (PG), had no effect on t‐PA biosynthesis by FLF. The induction of t‐PA biosynthesis by PI liposomes was inhibited by specific inhibitors of phosphoinositide pathway: gentamycin and lithium chloride. Thus, gentamycin inhibited the effect of PI liposomes on t‐PA biosynthesis by 76% (P < 0.001), while it had no effect on control FLF. Likewise, lithium chloride inhibited t‐PA biosynthesis of both PI‐treated and control FLF by >84%. The induction of t‐PA biosynthesis by PI liposomes was dependent on RNA transcription and independent of DNA biosynthesis.
Danazol therapy for cyclic thrombocytopenia
Authors: Rocha Rafael , Horstman Lawrence , Ahn Yeon S. , Mylvaganam Ravindra , Harrington William J. 
Published: February 2007

AbstractCyclic thrombocytopenia is a rare disease characterized by cyclic oscillations of platelet counts from very low to normal or higher. Severe hemorrhage may occur during the thrombocytopenic phase. To date, treatments for this disorder have been disappointing. Its pathophysiology is unknown. We report a successful outcome using danazol therapy.It can be argued that the long‐term outcome was due to spontaneous remission. However, significant improvement was noted from the outset of danazol therapy, and further improvement with long‐term therapy, as seen in the response of chronic ITP to danazol therapy. Danazol may offer lasting benefit in cyclic thrombocytopenia.
Atypical lymphocytes with a multilobated nucleus from a patient with tsutsugamushi disease (scrub typhus) in Japan
Authors: Iwasaki Hiromichi , Ueda Takanori , Uchida Michihiko , Nakamura Toru , Takada Nobuhiro , Mahara Fumihiko 
Published: February 2007

AbstractA case of tsutsugamushi disease (scrub typhus) with atypical lymphocytes with a multilobated nucleus is reported. Although this type of atypical lymphocyte has been reported in patients with viral infections such as adult T‐cell leukemia, infectious mononucleosis, human immunodeficiency virus (HIV) infection, this is the first reported case of atypical lymphocyte with a multilobated nucleus in a patient with rickettsial infection. This type of atypical lymphocyte seems to exist in a broad spectrum of infectious diseases.
Eosinophilia resulting from administration of recombinant granulocyte‐macrophage colony‐stimulating factor (rhGM‐CSF) in a patient with T‐γ lymphoproliferative disease
Authors: Gonzales‐Chambers R. , Rosenfeld C. , Winkelstein A. , Dameshek L. 
Published: February 2007

AbstractA therapeutic trial of recombinant human granulocyte‐macrophage colony‐stimulating factor (rhGM‐CSF) was attempted in a patient with neutropenia and frequent infections secondary to T‐γ lymphoproliferative disease (T‐γ LPD). During the 14 days of subcutaneous rhGM‐CSF (500 μg/m2/day), the absolute eosinophil count increased from 0 to 9,455/μl. By contrast, the absolute neutrophil count decreased. Toxicity related to rhGM‐CSF included arthralgia and nonspecific chest pain. The possible mechanism for the rhGM‐CSF induced selective eosinophilia is discussed.
Effect of reversible androgen deprivation on hemoglobin and serum immunoreactive erythropoietin in men
Authors: Weber Jed P. , Walsh Patrick C. , Peters Craig A. , Spivak Jerry L. 
Published: February 2007

AbstractTo examine the role of testosterone in the maintenance of hemoglobin levels, we studied the effect of reversible androgen deprivation on hemoglobin, serum immunoreactive erythropoietin, and serum testosterone in seven men treated with a luteinizing hormone‐releasing factor (LHRH) agonist for 6 months and then followed for an additional 6 months. The mean serum testosterone level was 4.35 ± 1.05 ng/ml initially and it decreased to castrate levels in all patients by 6 months. After stopping therapy, there was a rapid increase in serum testosterone such that by 12 months the mean concentration was normal. The pretreatment hemoglobin was 15.2 ± 0.9 g/dl (mean ± SD); after 6 months of androgen deprivation it had fallen to 14.1 ± 0.4 g/dl (P < 0.05). Six months after stopping therapy, the hemoglobin rose to pre‐treatment levels. Before treatment, serum immunoreactive erythropoietin was 9.5 ± 4.6 mu/ml (mean ± SD) and did not change significantly during or after the 6 month period of androgen deprivation. No significant inhibition of burst‐forming unit‐erythroid (BFU‐E) or colony‐forming unit‐granulocyte macrophage (CFU‐GM) was observed at the serum levels of nafarelin acetate obtainable in vivo. These data suggest that, within the normal range of hemoglobin in men, androgens are a determinant of the red cell mass.
Platelet lipoxygenase defect (wien‐penzing defect) in two patients with myocardial infarction
Authors: Sinzinger Helmut , Kaliman Josef , O'Grady John 
Published: February 2007

AbstractIn 2 male patients (35 and 38 years) presenting with myocardial infarction an abnormal conversion of exogenous 14C‐arachidonic acid by the patients' platelets, incubated in vitro, was observed. Neither patient's platelets showed evidence of a lipoxygenase pathway.Platelet thromboxane formation from exogenous and endogenous substrate was high, while the platelet aggregation responses were normal. A myeloproliferative syndrome was excluded by bone marrow puncture. Similar defects have only been described so far in patients with myeloproliferative syndrome. This defect may be causative for the onset of clinical thrombotic events. It is speculative whether in vivo therapy with r‐IFNα1c might be able to eradicate the pathological platelet clone.
Role of leukocytes in the activation of intravascular coagulation in patients with septicemia
Authors: Okajima Kenji , Yang Wang‐Peng , Okabe Hiroaki , Inoue Masayasu , Takatsuki Kiyoshi 
Published: February 2007

AbstractTo elucidate the role of leukocytes in intravascular coagulation in patients with septicemia, plasma levels of thrombin‐antithrombin III complex (TAT), soluble fibrin monomer complex (SFMC) and fibrinogen (Fbg) were determined in 33 patients with septicemia. Twenty of 33 patients revealed marked leukopenia caused by suppression of hematopoie‐sis by the administration of chemotherapeutic agents for the treatment of hematological malignancies; the total leukocyte count of these patients was < 1,000/μl. Thirteen of 33 patients showed normal or increased leukocyte counts. Plasma levels of TAT and SFMC in septicemic patients without leukopenia were significantly higher than in patients with leukopenia. Although plasma TAT and SFMC levels correlated well with the number of leukocytes, a more significant positive correlation was found between the number of monocytes and the levels of TAT and SFMC. Plasma levels of Fbg were significantly lower in patients without leukopenia than in patients with leukopenia. No significant correlation was found between the number of leukocytes and the levels of Fbg. However, a significant negative correlation was found between the number of monocytes and the levels of Fbg. TAT levels did not correlate with the number of platelets. The fibrinolytic system was activated only in septicemic patients without leukopenia, which may be explained by secondary fibrinolysis following leukocyte‐activated coagulation. These findings suggest that leukocytes, in particular monocytes, may play a critical role in the pathogenesis of intravascular coagulation in septicemia.
Therapy of the Kasabach‐Merritt syndrome with cryoprecipitate plus intra‐arterial thrombin and aminocaproic acid
Authors: Stahl Robert L. , Henderson J. Michael , Hooks Michael A. , Martin Louis G. , Duncan Alexander 
Published: February 2007

AbstractA patient with the Kasabach‐Merritt syndrome with disseminated intravascular coagulopathy and congestive heart failure due to a giant hepatic cavernous hemangioma achieved thrombosis of his tumor with a combination of cryoprecipitate plus intra‐arterial thrombin and epsilon aminocaproic acid. This was documented by an absence of Indium 111‐labelled platelet sequestration, marked decrease in thrombin:antithrombin complex generation, and normalization of platelet count and fibrinogen. Interventional angiography with aminocaproic acid and thrombin is advocated in patients whose tumors are amenable to such an approach so as to avoid a systemic antifibrinolytic state.