Apoptotic cell death induced by cross-linking Fas receptor (FasR/CD95) has been investigated in human acute myelogenous leukemia (AML) cells. FasR-mediated growth inhibition and DNA fragmentation could be induced in certain cases of AML. Interestingly, when DNA synthesis and G1 -> S transition in the cell cycle were enhanced by interleukin-3 or granulocyte-macrophage colony-stimulating factor, Fas-insensitive blast cells acquired cellular susceptibility toward FasR-mediated growth inhibition. To further evaluate an association between the Fas-R- mediated action and a specific phase of the cell cycle, a FasR+ leukemic cell line, MML-1, was established from a patient with AML. The morphologic feature of dying cells and DNA fragmentation indicated that FasR cross-linking induced apoptotic cell death in MML-1 cells. Cell cycle arrest in G1A phase with the treatment of phorbol 12-myristate 13- acetate or thymidine rendered MML-1 cells resistant to FasR-mediated apoptosis without downregulation of surface FasR expression. However, S- phase arrest with 5-fluorouracil could neither enhance nor inhibit FasR- mediated apoptosis. Simultaneous DNA/RNA quantification analysis revealed the selective loss of cells in G1B compartment, accompanied by the increase of apoptotic nuclei in sub-G1 fraction. These findings suggested that FasR-mediated apoptotic signals could be transduced into cells in G1B compartment and G1A -> G1B transition might augment the induction of FasR-mediated apoptosis.
Summary:Case reportA 4-year-old boy was first noted to have severe anemia at A 4-year-old boy with Diamond-Blackfan anemia and a history of multiple transfusions underwent umbilical 1 month of age. An evaluation showed hemoglobin 50 g/l, white blood cell count 5.5 × 10 9 /l with normal distribution, cord blood transplantation from his HLA-identical female sibling born by vaginal delivery at 38 weeks. The platelet count 385 × 10 9 /l, and 0% reticulocyte. The bone marrow showed marked erythroid hypoplasia with no patient was prepared with busulfan, cyclophosphamide and antilymphocyte globulin. Methotrexate and cycloabnormal cells and a diagnosis of Diamond-Blackfan anemia was confirmed. He was 2680 g at birth, the product sporin A were given for the prophylaxis of GVHD. Regimen-related toxicity was not observed and successful of a full-term, uncomplicated gestation. The parents were healthy and there was no consanguinity or family history engraftment occurred, including the erythroid series. No evidence of acute or chronic GVHD has been of hematological disorders. Several therapeutic approaches including intravenous high-dose methylprednisolone and observed for 14 months after transplantation. This is the first case of successful umbilical cord blood transoral corticosteroids had been tried. However, they had only a transient effect on the progressive anemia, and the patient plantation to a patient with Diamond-Blackfan anemia. Keywords: cord blood transplantation; Diamondbecame steroid-resistant and transfusion-dependent. He received 60 units of filtered and irradiated packed red cells Blackfan anemia; childhood; hemosiderosis before transplantation without any iron-chelating therapy. His HLA-type was A24, 31; B51, 52; DR15(2), 4; DQ1, 4, and not identical to the parents and a sibling (father: Diamond-Blackfan anemia is a disorder of childhood A31, 33; B44, 52; DR15(2), 8; DQ1, mother: A24, 33; B44, characterized by normochromic-macrocytic anemia, reti-51; DR4, 13; DQ1, 4, brother: A31, 33; B44, 52). culocytopenia and a normocellular marrow with a selective At 3 years of age, he displayed signs of iron overload deficiency of erythroid progenitors. Most patients respond including elevations of serum transaminase, iron and ferrito corticosteroids initially. However, about 60% require tin. CT scan showed a marked increase in the density of chronic red cell transfusion and die in the second and third the liver parenchyma, which was consistent with liver decade of life as a result of hemosiderosis. 1 Allogeneic hemosiderosis. Echocardiography revealed mild left ven-BMT is already accepted as effective therapy for steroidtricular dilatation and grade II mitral regurgitation with norresistant, transfusion-dependent patients with Diamondmal wall mobility. His mother became pregnant and a girl Blackfan anemia. 2-5 However, the limited availability of was delivered vaginally at 38 weeks without any complisuitable donors remains a problem. Recently, unbilical cord cations. At the time of delivery, 70 ml of cord blood was b...
2',5'-oligoadenylate synthetase activity, which is assumed to be induced by interferon, is reported to be one of the useful markers reflecting interferon activity. The enzyme activity of patients with aseptic meningitis and febrile convulsion were compared in order to evaluate interferon activity as one of the local immuno-defense mechanisms of aseptic meningitis. The surface antigen of mononuclear cells in cerebrospinal fluid and peripheral blood of some patients with aseptic meningitis was also measured. The enzyme activity of patients with aseptic meningitis was 191.4 pmoVdL in the cerebrospinal fluid and 395.8 pmol/dL in the serum during the acute phase, while that of patients with febrile convulsion was 45.2pmoYdL in the cerebrospinal fluid and 326.0pmoVdL in the serum. The enzyme activity of the former patients significantly decreased during the recovery phase in both the cerebrospinal fluid and serum. CD3 positive cells in the peripheral blood were 56.3% of the total mononuclear cells during the acute phase and 65.2% during the recovery phase, whereas in the cerebrospinal fluid mononuclear cells, they were 87.1 and 85.5%, respectively. During the acute phase, CD4 positive cells were the predominant T lymphocyte subset in the cerebrospinal fluid cells, while CD8 positive cells were predominant during the recovery phase. The relative proportions of CD4 positive and CD8 positive cells during the acute a d recovery phase in the cerebrospinal fluid mononuclear cells were quite high compared to the recovery phase, although that ratio of peripheral blood mononuclear cells was not changed throughout the course. It was concluded that T lymphocytes and increased 2',5'-oligoadenylate synthetase activity in the cerebrospinal fluid may be one of the important components in the local inflammatory process independent of the systemic host defense mechanism in aseptic meningitis.
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