bluuebunny/crossref_metadata_2025_split huggingface dataset:
Induction of differentiation in the human leukemia cell line SPI‐802: Morphological, immunological, and isoenzymatic changes
Authors: Drexler Hans Guenter , Gaedicke Gerhard , Komiyama Atsushi , Minowada Jun 
Published: February 2007

AbstractThe phorbolester 12‐0‐tetradecanoylphorbol 13‐acetate (TPA) was used for the induction of differentiation in cells of the human leukemia cell line SPI‐802. The cellular morphology, surface marker antigen expression, and isoenzyme profiles of four enzymes (carboxylic esterase, acid phosphatase, hexosaminidase, and lactate dehydrogenase) served as parameters for monitoring the induced phenotypical changes. TPA led to distinct alterations of the morphology and significantly affected the growth rate with cessation of cell proliferation. No major increase in the number of nitro blue tetrazolium‐positive cells or aggregation of cells, phagocytosis of latex beads, adherence to plastic surface, or development of pseudopodia were observed. As TPAtreated SPI‐802 cells remained negative for these markers of the monocyte‐macrophage complex, it can be concluded that the cells did not differentiate into monocytes and macrophages. The immunological marker profile based on testing of 55 monoclonal antibodies, terminal deoxynucleotidyl transferase and two erythrocyte rosette tests indicated a differentiation of SPI‐802 cells along the granulocytic cell lineage. This was confirmed by isoenzyme analysis, especially that of carboxylic esterase. An isoenzyme specific for monocytes and macrophages was not detected. In earlier studies it was found that SPI‐802 cells produce hemoglobin upon exposure to TPA or hemin. This latter observation and the present results suggested a comparison with the two erythroleukemia cell lines K‐562 and HEL. SPI‐802 cells appear to have the potential to differentiate along several cell lineages.
Unexplained periparturient thrombocytopenia
Authors: Freedman J. , Musclow E. , Garvey B. , Abbott D. 
Published: February 2007

AbstractSince the advent of routine automated platelet counting we have observed unexplained periparturient thrombocytopenia (PPT) in an unexpected number of periparturient women, ie, during labor or within 24 hr postpartum. Mean ± SD platelet count in 686 random blood donors was 236 ± 50 ± 109/L and 1.02% had a platelet count < 136 ± 109/L; in 2,204 random prenatal and postpartum women mean count was significantly higher (275 ± 86 ± 109/L; p < 0.001). Of 1,621 periparturient women, 74 (4.6%) had unexplained PPT (mean ± SD platelet count 122 ± 24 ± 109/L, range 21‐135 ± 109/L, N = 74). Platelet count in PPT usually rose to normal within 1 week of delivery; in 10% thrombocytopenia persisted > 6 months. PPT occurred in successive pregnancies with normal intervening platelet counts. Nine of 34 newborns of mothers with PPT were thrombocytopenic; there was no correlation between mother's and baby's platelet counts. In no case of PPT was there excessive bleeding in mother or infant. Positive indirect platelet radioactive antiglobulin tests (PRAT) were seen in 11% of normal postpartum women and in 90% of 22 women with PPT; 65% of the positive tests in PPT were due to reactions with anti‐C3 only. In contrast, pregnant women with autoimmune thrombocytopenic purpura (AITP) had positive PRAT primarily because of anti‐IgG ( ± anti‐C3); only 10% were positive only with anti‐C3. Results were concordant in all of eight women with PPT tested by both indirect and direct PRAT. Amount of C3 bound per platelet in direct or indirect PRAT was not predictive of degree of thrombocytopenia, but there was correlation of fg C3 per platelet detected by the two assays in individual patients (r = 0.8). Mean levels of serum C3, C4, and factor B in women with PPT did not differ from normal; individual patients had abnormal serum complements but no characteristic pattern was observed. Increased immune complexes were observed in 6% of normal subjects and 33% of women with PPT.Etiology and mechanism of PPT is unclear. Despite lack of clinical evidence in women with PPT of syndromes associated with increased platelet destruction, the presence of preeclampsia cannot be absolutely excluded. Similarly, although the pattern of antiglobulin sensitization in PPT differed markedly from that seen in AITP, autoimmune disorder cannot be excluded. Alloantibodies did not appear to be responsible for PPT. While PPT is usually benign, some patients had a markedly reduced platelet count. Recognition of the phenomenon may be important in obstetrics.
Activation of platelet function in Fabry's disease
Authors: Igarashi Takashi , Sakuraba Hitoshi , Suzuki Yoshiyuki 
Published: February 2007

AbstractIncreased platelet aggregation and high plasma concentration of β‐thromboglobulin were observed in hemizygotes and heterozygotes of Fabry's disease. Carbamazepine and phenytoin administered for the treatment of pains in these patients showed no significant effect on platelet aggregation. No activation of platelets was observed after the addition of ceramide trihexoside, the storage lipid of this disease. Mitral valve prolapse was found in eight of 12 patients. Although the pathogenesis of platelet activation and mitral valve prolapse are not known, the platelet activation could be an early indicator and an accelerating factor of thromboembolic vascular change in this disease.
Multimeric pattern discrepancy between platelet and plasma von Willebrand factor in type IIC von Willebrand disease
Authors: Batlle J. , Fernandez M. F. Lopez , Villamor A. Fernandez , Berges C. Lopez , Zimmerman T. S. 
Published: February 2007

Abstractvon Willebrand factor (vWF) from platelet lysate and plasma, collected in the presence of protease inhibitors, was studied in two patients with type IIC von Willebrand disease (vWD). Platelet and plasma vWF showed the smallest multimer increased, but the latter had a repeating single band whereas the former had a repeating “doublet.” This platelet‐plasma discrepancy observed for the first time in these patients suggests that the repeating “doublet” or single band described in other type IIC patients represent minor subgroups of type IIC vWD.
Impaired immunoglobulin synthesis in multiple myeloma: A B‐cell dysfunction
Authors: Carter A. , Silvian I. , Tatarsky I. , Spira G. 
Published: February 2007

AbstractPeripheral blood samples collected from normal individuals and patients with benign monoclonal gammopathy and multiple myeloma were separated and assayed for their T‐ and B‐cell subpopulations as well as immunoglobulin (IgG, IgM) synthesis in vitro. To rule out any functional or quantitative alterations related to therapy, only newly diagnosed multiple myeloma patients and subjects with benign monoclonal gammopathy were included in our study. Multiple myeloma patients were further subdivided into two groups. Group A consisted of patients with low and intermediate tumor burdens, while group B included patients with high tumor mass. B‐ and T‐cell subset analysis revealed an abnormal ratio between B/T and OKT4+/OKT8+ lymphocytes compared to healthy controls. These alterations were especially prominent in group B multiple myeloma. Immunoglobulin synthesis was studied in pokeweed‐mitogen‐stimulated lymphocyte cultures. The results indicate that the failure to synthesize and secrete immunoglobulin resides within the B‐cell lineage and is probably due to a functional B‐cell defect. T‐cell immunoregulatory functions seem to be unaffected in both multiple myleoma and benign monoclonal gammopathy patients.
Two new glucose‐6 phosphate dehydrogenase (G6PD) variants associated with hemolytic anemia: G6PD amman‐1 and G6PD amman‐2
Authors: Karadsheh Naif S. , Awidi A. S. , Tarawneh M. S. 
Published: February 2007

AbstractTwo glucose‐6‐phosphate dehydrogenase (G6PD) variants were investigated. G6PD Amman‐1 was partially purified from the red cells of a patient suffering from recurrent jaundice and spontaneous episodic attacks of severe hemolysis in the absence of oxidant drugs, infection, or fava beans. The enzymatic characteristics of G6PD Amman‐I were markedly reduced activity, fast eletrophoretic mobility, slightly increased km for NADP, normal km for G‐6‐P, normal heat stability, normal utilization of substrate analogues 2‐deoxy G‐6‐P and deamino‐NADP, and a monophasic pH curve with a peak at 8.5 to 9.3. The second variant, G6PD Amman‐2, was partially purified from the red cells of a patient suffering from recurrent jaundice with episodic mild hemolysis caused by infection or unknown factors. G6PD Amman‐2 characteristics were severely reduced activity, slow electrophoretic mobility, normal km for NADP, decreased km for G‐6‐P, decreased heat stability, increased utilization of substrate analogues, and a monophasic pH curve with a narrow peak at pH 9.5. The red cell level of reduced glutathione was markedly decreased with twofold increase in the activity of glutathione reductase in the patient with G6PD Amman‐2.
Interaction between leukocytes and serum plasminogen: An essential mechanism in peripheral blood fibrinolytic activity
Authors: Ghezzo F. , Savoca P. , Vallero P. , Bellone G. 
Published: February 2007

AbstractIntense fibrinolytic activity (FA) by granulocytes, mononuclear adherent cells, and natural killer (NK) cells was demonstrated by using 125I‐fibrin‐coated wells. This FA was greatly dependent on serum plasminogen, provided that serum inhibitors were destroyed by heating at 56°C. Using monoclonal antibodies, it was also observed that these leukocytes produce urokinase and that this enzyme is secreted during adhesion by mononuclear adherent cells and during the cytotoxicity reaction against neoplastic cells by NK cells. Since cell intactness is necessary for leukocyte‐serum interaction, stress is placed on the membrane function. We conclude that leukocytes are essential in peripheral blood FA and that transport of urokinase by circulating cells, inside their cytoplasm, could offset its lack of affinity for fibrin and thus avoid undesirable systemic effects.
Myeloid cell surface phenotype in myelodysplasia: Evidence for abnormal persistence of an early myeloid differentiation antigen
Authors: Baumann Michael A. , Keller Robert H. , McFadden Patrick W. , Libnoch Joseph A. , Patrick Catherine W. 
Published: February 2007

AbstractA panel of monoclonal antibodies recognizing myeloid cell surface differentiation‐associated antigens was used to study the peripheral blood myeloid population of 23 patients with myelodysplastic syndromes (MDS) and 23 controls. A marker for immaturity, defined by the presence of the antigen recognized by the My9 antibody, was found to persist on the surface of mature neutrophils in a subgroup of MDS patients. Abnormal My9 positivity was concentrated primarily, but not exclusively, in previously described morphologically defined subgroups of MDS patients considered to be at highest risk for leukemic conversion. Longitudinal study of a larger number of patients will be required to test the hypothesis that abnormal persistence of My9 may have prognostic significance.
Sickle cell anemia associated with α‐thalassemia in Malaysian Indians
Authors: Lie‐Injo Luan Eng , Hassan Khalid , Joishy Suresh K. , Lim Mu Lan 
Published: February 2007

AbstractThe Indian rubber estate workers in Negri Sembilan, Malaysia, who originated from Orissa in India were found to have a high frequency of Hb S (Joishy SK, Hassan K: Clin Res 28:280, 1980). Unlike the usually severe clinical picture of sickle cell anemia seen in African and American blacks, the clinical picture of the disease in this population was mild and many have reached old age. We studied the leukocyte DNA of 12 patients with sickle cell anemia, ranging in age from 4 to 61 years and 30 sickle cell trait carriers, ranging in age from 7 to 63 years, for the presence of α‐globin gene deletions by gene mapping according to Southern (Southern EM: J Mol Biol 98:503, 1975), using α‐ and ζ‐globin gene probes obtained by nick translation of the α‐ and ζ‐globin genes cloned into plasmid. All 12 sickle cell anemia patients were found to have α‐thalassemia2 (α‐thal2), either in the homozygous or heterozygous condition. Of the Hb S trait carriers, six did not have α‐that2 or or‐thal1 and 24 had α‐thal2 (15 heterozygous, 9 homozygous). Seven of these Hb S trait carriers with α‐thal2 had an additional gene abnormality. Five of them had a fast‐moving Eco RI fragment 5.6 kb long that hybridized with ζ‐specific probe but not with α‐specific probe. An unusual DNA pattern of a different type was further found in the other two. Bgl II restriction analysis showed that the α‐thal2 was mostly of the rightward deletion α‐thal2 genotype. None of the sickle cell anemia patients and Hb S trait carriers had deletion type α‐thal1. The sickle cell anemia patients had very high levels of Hb F and low levels of Hb A2. The Hb S trait carriers with α‐thal2 had relatively low levels of Hb S.
Paravertebral extramedullary hematopoiesis associated with improvement of anemia in congenital dyserythropoietic anemia type II
Authors: Lugassy Gilles , Michaeli Joseph , Harats Noam , Libson Eugene , Rachmilewitz Eliezer A. 
Published: February 2007

AbstractThoracic masses resulting from extramedullary hematopoiesis developed in two sisters of Moroccan origin with congenital dyserythropoietic anemia type II (HEMPAS). In one patient, the diagnosis of extramedullary hematopoiesis was confirmed histologically. The appearance of extramedullary foci of hematopoiesis mimicking mediastinal tumors has not been previously described in HEMPAS. These masses result from persistent erythropoietic stimulation associated with chronic hemolytic anemia. In both patients, detection of the asymptomatic masses was preceded by normalization of hemoglobin levels. Thus unexpected correction of a chronic refractory anemia associated with the appearance of mediastinal masses might be the heralding manifestation of an effective extramedullary hemopoiesis.
Acute leukemia with mediastinal mass, lymphadenopathy, and monocytic precursor cells
Authors: Schwarzmeier Josef D. , Bettelheim Peter , Radaszkiewicz Thaddäus , Prischl Friedrich , Schwabe Michael , Fuhrmann Manfred 
Published: February 2007

AbstractCell marker analysis with monoclonal antibodies (MoAb) as well as differentiation studies with the chemical inducer TPA were used to identify a population of apparently lymphoid cells as monocytic precursor cells in a patient with acute leukemia. The initial manifestation of the disease with mediastinal mass and lymphadenopathy was followed by the appearance of small lymphocyte‐like blast cells in bone marrow (BM) and peripheral blood (PB). Although a lymph node biopsy revealed an infiltration with monoblasts, the leukemic cells in BM and PB were not classifiable. However, when the patient relapsed after chemotherapy with large monoblasts and again with morphologically lymphoid blast cells, the latter could be classified by treatment with TPA. After incubation with the inducer (12–48 hr) the cells became positive with the MoAB VIM‐D5, showed a strong reaction with α‐napthyl‐acetate‐esterase and developed macrophage like morphology, as well as phagocytic properties. During the terminal phase of the disease, small, lymphocyte‐like blast cells predominated. These cells could be classified by a panel of MoABs. They expressed myeloid determinants (VIM‐D5, VIM‐2, MY 9, VIM‐12, VIM‐13) but showed no reactivity with MoABs specific for lymphocytic cells.
Sialic acid of platelets and granulocytes in polycythemia vera
Authors: Streichman S. , Menasheoff D. , Tatarsky I. , Marmur A. 
Published: February 2007

AbstractPrevious studies have shown that erythrocytes of polycythemia vera (PV) patients have an increased content of sialic acid on their membrane compared with normal erythrocytes. This has been attributed to the presence of an abnormal clone known to proliferate in this disease. Since granulocytes and platelets originate from the same stem cell that is involved in this disease, it was of interest to see whether these cells also bear increased amounts of sialic acid compared with their normal counterparts. While normal values were found for granulocyte populations, elevated values were found for the platelet populations of PV patients.
Massive hemolysis following inhalation of volatile nitrites
Authors: Bogart Lee , Bonsignore Justine , Carvalho Angelina 
Published: February 2007

AbstractVolatile nitrites are drugs which have become widely used for their mood‐altering and aphrodisiac effects. Their use has been associated with methemoglobinemia and mild hemolysis; more significant hemolysis has been noted in a patient with G6PD deficiency. We report here a case of massive hemolysis following inhalation of volatile nitrites in the absence of an apparent intrinsic red cell defect. It is important that health care workers are aware of side effects from these commonly abused substances.
Bone marrow transplantation for good‐risk patients with leukemia in a university affiliated hospital
Authors: Verdirame Joseph D. , Bruckman James E. , Feagler John R. , Commers James R. 
Published: February 2007

AbstractOf the first 14 patients with acute or chronic leukemia to undergo bone marrow transplantation at our hospital, 9 (64%), all good‐risk, are still alive in remission at 18 to 42 months of follow‐up (mean, 29 months) with their Karnofsky performance status between 80% and 100%. The conditioning regimen of fractionated‐dose irradiation and high‐dose chemotherapy eradicated their disease; only two patients relapsed after transplantation. Toxicity was acceptable. Acute graft‐versus‐host disease developed in six patients (43%) (grade I or II in four, grade IV in two) and progressed to chronic graft‐versus‐host disease in four. Viral pneumonitis developed in three patients (21%), but none had idiopathic interstitial pneumonitis. The mean hospital charge was $54,355. These preliminary results suggest that good‐risk patients with acute or chronic leukemia can be treated with bone marrow transplantation in a university affiliated hospital with appropriate staff and support facilities and achieve results comparable to those in research institutions at a competitive cost.
Acquired von Willebrand disease and storage pool disease in chronic myelocytic leukemia
Authors: Mohri Hiroshi 
Published: February 2007

AbstractPlatelet function was evaluated in 20 patients with chronic myelocytic leukemia (CML), all Ph positive. Seven showed abnormal epinephrine‐induced aggregation, while four had impaired both ADP‐ and collagen‐induced aggregation. The platelets of all patients aggregated with arachidonic acid, thus ruling out cyclooxygenase or lipoxygenase deficiency. The intracellular concentrations of ATP and ADP were significantly below normal, and the ratio of ATP/ADP was greater than normal in all 12 patients. ATP released from platelets by Lumi‐aggregometer was reduced. In four patients with abnormal ristocetin‐induced aggregation, vWF:Ag, RCoF, and FVIII:C were all reduced. No significant inactivation of factor VIII was induced in normal plasma by incubation with patient's plasma. The crossed immunoelectrophoretic analysis revealed that vWF:Ag in these patients was mainly composed of more anodic component as compared with that of normal plasma. The ratio of vWF:Ag/RCoF was significantly greater than normal. A marked increase of factor VIII and a rapid return of vWF:Ag and RCoF to the baseline after the 1‐deamino‐8‐arginine vasopressin (DDAVP) infusion were observed. Transient increase in vWF:Ag after the infusion of DDAVP appeared with less anodic forms and in the same relative proportion as that in normal plasma. The present study shows that in some patients with CML storage pool disease occurs with acquired von Willebrand disease.
A new case of high‐molecular‐weight kininogen inherited deficiency
Authors: Lefrère Jean‐Jacques , Horellou Marie‐Hélène , Gozin Danièle , Conard Jacqueline , Muller Jean‐Yves , Clark Michael , Soulier Jean‐Pierre , Samama Meyer 
Published: February 2007

AbstractA preoperative hemostasis study discovered a prolonged activated partial thrombo‐plastin time in a 23‐year‐old Portuguese Caucasian woman without personal or past family history of hemorrhage or thrombosis. This was corrected by pooled plasma that excluded circulating anticoagulant. Activated partial thromboplastin time was prolonged whatever the activator, particularly ellagic acid, and was not corrected by prolonged kaolin incubation. Levels of factors VIII and XII were normal; factor XI and prekallikrein levels were either moderately low or normal according to activators and defective reagents used. High‐molecular‐weight kininogen (HMWK) level assessed by coagulation and immunological method was virtually nil. Fibrinolysis activity was normal before and after veinous occlusion. The programmed operation was performed without any particular preparation and no complication arose. Family investigation found heterozygous HMWK deficiency in the proposita's father and three of her siblings.
Studies of the effects of trimethoprim and sulfamethoxazole on human granulopoiesis
Authors: Bjornson Barbara H. , McLntyre Angus P. , Harvey John M. , Tauber Alfred I. 
Published: February 2007

AbstractTrimethoprim and sulfamethoxazole (Bactrim r) is a widely used antibiotic combination effective against a broad spectrum of microbial organisms. There are reports of neutropenia developing during even brief periods of oral therapy, particularly in individuals with either folate deficiency or increased folate requirements. We have investigated the effects of these drugs on circulating granulocyte precursors (CFU‐C) from normal donors and the mechanism of inhibition on granulopoiesis using an in vitro CFU‐C assay. In 12 healthy adults, the number of circulating granulocytes and granulocyte progenitors was not significantly altered by a 5‐day course of therapy. However, in experiments that simulated the in vivo condition of folate deficiency (folate‐free cultures were prepared with cells harvested from normal donors), trimethoprim (8 μg/ml) resulted in a 47% decrease in the total number of colonies; this inhibitory effect was prevented when 100 ng of folinic acid was also added to the culture. Sulfamethoxazole (40 μg/ml) had no discernible effect on granulopoiesis. The combination of 8 μg/ml of trimethoprim and 40 μg/ml of sulfamethoxazole resulted in a 52% decrease in the number of colonies generated and this inhibition was again prevented by folinic acid. Our results suggest that the neutropenia occasionally observed in patients treated with trimethoprim‐sulfamethoxazole is due to the inhibitory effects on granulopoiesis by trimethoprim, namely its antifolate action, which is reversed by folinic acid. Based on these studies, in patients with either folate deficiency or increased folate requirements, trimethoprim‐sulfamethoxazole should be used with caution.
Erythrocyte Rh antigens increase with red cell age
Authors: Ballas Samir K. , Flynn John C. , Pauline Lucy A. , Murphy Darci L. 
Published: February 2007

AbstractCertain blood group antigens may play a role in the removal of senescent red cells from the circulation. In order to elucidate this hypothesis, we have investigated the effect of red cell aging on Rh blood group antigens. Red cells from volunteer donors were separated into subpopulations of uniformly defined densities on discontinuous Stractan density gradients. The number of the five major Rh antigen sites of each fraction was determined both by enzyme‐linked immunosorbent assay (ELISA) and by hemagglutination‐scoring technique. All Rh antigens tested increased with cell age (p < 0.01). The data indicate that there is a positive correlation between red cell age and the Rh blood group antigen site number.