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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The urinary extracts from patients with aplastic anaemia and from healthy donors were investigated in vivo and in vitro for their ability to stimulate megakaryopoiesis and platelet production. There was a significantly higher concentration of thrombopoiesis stimulating factor (TSF) and megakaryocyte colony stimulating factor (MEG-CSF) in the urine from patients with aplastic anaemia than in that from healthy donors. Neuraminidase treatment did not affect the thrombopoietic activity of TSF, whereas coexisting erythropoietin (EPO) in the extract lost in its activity in vivo. These findings suggest that TSF and/or MEG-CSF seems to be different from EPO and that the urine from aplastic anaemia patients would be a good source of TSF and MEG-CSF for purification and characterization.
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PMID:Thrombopoiesis and megakaryocyte colony stimulating factor in the urine of patients with aplastic anaemia. 696 85

The urinary extract from patients with severe, chronic idiopathic thrombocytopenic purpura (ITP) was capable of inducing significant thrombocytosis in rats in vivo and enhancing megakaryocyte colony formation in culture of mouse bone marrow cells. The apparent specific activity of megakaryocyte colony stimulating factor (MEG--CSF) in the ITP extract was approximately one-half of that of the urinary extract from patients with aplastic anaemia (AA). Daily injections of ITP extract did not cause an increase in Hb concentration, while rats receiving AA urinary extract revealed profound erythropoiesis 3 weeks later. In vitro assay of erythropoietin (EPO) failed to show significant EPO activity in the extract from patients with ITP. These findings excluded the possibility that the observed activities of thrombopoiesis-stimulating factor (TSF) and MEG--CSF in the ITP urinary extract are due to contaminating EPO. Urine of patients with severe ITP appears to be a good source of TSF and MEG--CSF.
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PMID:Thrombopoiesis- and megakaryocyte colony-stimulating factors in the urine of patients with idiopathic thrombocytopenic purpura. 697 2

Megakaryocyte colon-stimulating factor (MEG-CSF) in the urinary extracts from patients wit aplastic anaemia (AA) revealed two distinct peaks of activity on Sephadex G-200 gel filtration with apparent molecular weights of 155,000 and 76,000. Both fractions induced significant thrombocytosis in peripheral blood and megakaryocytosis in the spleen of rats. Heterogeneity of MEG-CSF was also found in the extracts from the urine of patients with idiopathic thrombocytopenic purpura. The higher molecular weight MEG-CSF was significantly reduced when the gel filtration was performed under the dissociating conditions. Ion-exchange chromatography indicated that the higher molecular weight MEG-CSF had a different charge from the lower molecular weight MEG-CSF. These results suggest that the apparent heterogeneity of MEG-CSF is due to interaction of MEG-CSF with other proteins in the urinary extracts.
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PMID:Apparent heterogeneity of human megakaryocyte colony- and thrombopoiesis-stimulating factors: studies on urinary extracts from patients with aplastic anaemia and idiopathic thrombocytopenic purpura. 698 64

Thrombopoietic (Tpo) and megakaryocyte-colony stimulating (Meg-CSF) activities were found in urinary extracts from patients with aplastic anemia. Preparative biochemical extractions were accomplished using Sephadex G-50 and DEAE-cellulose column chromatography. The biological activities of these extracts were assessed using not only an in vivo assay but were also examined in vitro employing the clonal development of megakaryocyte colonies. Both in vivo, as well as in vitro, biological activities were detected in the batch fraction which was stepwise eluted from DEAE-cellulose between 0.022 M NaCl in 0.016 M NaH2PO4 and 0.15 M NaCl in 0.05 M Na2HPO4 as a single fraction. When 0.4 mg of this fraction was injected daily into rats, a marked thrombopoiesis ensued producing an increase of 40% over initial platelet counts by 3 days after administration. This was followed by a decrease in platelets to a subnormal range by 21 days after the injection. Hemoglobin concentration gradually increased from 5% above initial value by day 7 to 20% above initial value by day 21. The effect of neuraminidase (NAse) on the properties of this extract was also examined. NAse-treated extracts, similar to the native extracts described above retained Tpo activity. Changes in megakaryocyte numbers in the spleen and bone marrow of rats were assayed with both the NAse-treated extract as well as with the native extract. A remarkable increase in megakaryocyte numbers, threefold above the normal count, was found in the spleens of rats given the native extract preparation; by contrast, however, no change was observed in splenic megakaryocyte numbers in rats given the NAse-treated extract. On the other hand, NAse-treated extract retained its ability to stimulate bone marrow megakaryocyte proliferation in the same rat. The urinary extract also revealed in vitro Meg-CSF activity with a specific activity of 31, 750 CFU-Meg colonies/mg of protein.
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PMID:Partial purification and biological properties of thrombopoietin extracted from the urine of aplastic anemia patients. 716 15

Fifteen patients with various myeloid and lymphoid neoplasias after receiving highly myelotoxic chemotherapy were treated with a single daily dose of 5 micrograms/kg of GM-CSF. G-CSF at a daily dose of 5-8 micrograms/kg was used in four patients with severe pancytopenia in the course of acute or chronic lymphoid leukemia treated with cytotoxic agents, and in two patients with idiopathic aplastic anemia. The administration of cytokines was provided for 4-10 days GM-CSF increased the WBC in twelve out of fifteen patients, mainly because of an increase in the number of neutrophils. Six patients receiving GM-CSF demonstrated rapid platelet recoveries. The rapid increase of the WBC was observed in all G-CSF treated patients. In one patient with aplastic anemia the WBC/ANC decreased rapidly to the initial values after the cessation of the G-CSF therapy. Fast platelet recovery was seen in three patients treated with G-CSF.
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PMID:Application of hematopoietic growth factors (G-CSF and GM-CSF) in the treatment of chemotherapy-induced or idiopathic bone marrow failure. 750 85

An 83-year-old male was admitted with pulmonary tuberculosis. He was started on rifampicin, isoniazid, and streptomycin (SM). The hematological data at 12 weeks after the treatment showed pancytopenia (RBC: 2.14 x 10(6)/microliters, Hb: 7.2g/dl, Plt: 1.8 x 10(4)/microliters, WBC: 700/microliters). All the above medicines were discontinued and he received bolus methylprednisolone (bmPSL) and recombinant human granulocyte-colony stimulating factor (rhG-CSF). After 3 cycles of bmPSL, red blood cells and platelets gradually increased. White blood cells also increased in response to rhG-CSF. Bone marrow aspirate and biopsy specimens showed normocellularity, indicating recovery from aplastic anemia. Drug lymphocyte stimulation test was positive for SM.
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PMID:[Streptomycin-induced severe aplastic anemia successfully treated with high-dose methylprednisolone pulse therapy and rhG-CSF]. 750 52

We investigated the effects of long-term therapy with recombinant human granulocyte colony-stimulating factor (rhG-CSF) in a 69-year-old man with severe aplastic anemia (SAA). The patient was too old to receive a bone marrow transplantation, and high-dose methylprednisolone and antilymphocyte globulin were ineffective. We administered rhG-CSF in combination with metenolone acetate. Trilineage blood cell components were recovered 6 months later. These findings suggest that long-term G-CSF therapy may be beneficial in patients with SAA who are not candidates for bone marrow transplantation.
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PMID:Trilineage response in severe aplastic anemia following long-term therapy with recombinant human granulocyte colony-stimulating factor. 750 89

We report on a patient with very severe aplastic anemia (SAA) unresponsive to immunosuppressive therapy (cyclosporine A, ATG). Because no HLA-identical family or unrelated donor could be found, a trial with recombinant human granulocyte stimulating factor (G-CSF) was started. This was followed by a rapid 3-lineage response with near-normal blood counts and transfusion independence. A similar response was obtained by 2 further G-CSF cycles which were given for relapsing SAA after G-CSF withdrawal. Following the third cycle, an acquired platelet function disorder was observed which preceded a myelodysplastic syndrome.
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PMID:[3-line response following long-term therapy of severe aplastic anemia using glycosylated rHuG-CSF: dysfunctional thrombocytes as early indication of a myelodysplastic syndrome]. 750

The present multicenter study was undertaken to confirm whether filgrastim/recombinant human granulocyte colony stimulating factor (rhG-CSF) could mobilize residual multipotential stem cells by its G0-shortening effect in patients with aplastic anemia (AA) and induce a multilineage response. Twenty-seven patients with acquired severe or moderate AA received long-term administration (2 to 12+ months) of rhG-CSF in doses from 100 to 400 micrograms/body/day by s.c. injection or 250 to 1,500 micrograms/body/day by i.v. infusion. Twenty-six out of the 27 evaluable patients showed a substantial increase in neutrophils associated with a recovery of myeloid precursors in bone marrow within one month of therapy. Interestingly, 10 out of the 27 patients showed a dramatic improvement in severe anemia after two to ten months of therapy. Moreover, severe thrombocytopenia improved after two to four months of therapy in three out of these ten patients accompanied by a significant increase in megakaryocytes in bone marrow. Clonal cultures of bone marrow cells revealed a recovery in myeloid as well as erythroid precursors in most of these ten patients. In two patients who showed a trilineage response, mixed and megakaryocyte colony formations also recovered. These results suggest that long-term administration of rhG-CSF mobilizes myeloid, erythroid, megakaryocyte and multipotential progenitor cells and induces a multilineage response in some patients with AA.
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PMID:Multilineage response in aplastic anemia patients following long-term administration of filgrastim (recombinant human granulocyte colony stimulating factor). 750 23

We investigated the efficacy and safety of high-dose recombinant human granulocyte colony-stimulating factor (rhG-CSF) in treating 10 children with severe aplastic anemia and fewer than 0.05 x 10(9)/L neutrophils. Doses of rhG-CSF ranging from 400 to 2,000 micrograms/m2/d were administered as a 30-minute intravenous infusion daily for 4 weeks. In 6 of the 10 children, treatment increased the neutrophil count by 10-fold to greater than 60-fold (range, 0.21 to 1.8 x 10(9)/L). Bacterial or fungal infections that were present at study entry resolved in all 6 responders, who are still alive with a median survival of more than 27 months (range, 15 to 54 months) since the initiation of treatment. Three of 4 nonresponders died of infection, whereas 1 nonresponder received a bone marrow transplant and is alive. No serious toxicity was attributable to rhG-CSF. It was well tolerated at doses up to 2,000 micrograms/m2/d and effectively stimulated granulopoiesis. This agent thus offers promise as adjuvant treatment for severe infections in children with aplastic anemia and very severe neutropenia.
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PMID:Stimulation of granulopoiesis by high-dose recombinant human granulocyte colony-stimulating factor in children with aplastic anemia and very severe neutropenia. 751 Jan 41


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