Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on our experience with the application of recombinant human granulocyte colony-stimulating factor (G-CSF, Neupogen, Hoffmann La Roche) in patients with various types of hematological malignancies, who had chemotherapy-induced myelosuppression, and in patients with idiopathic aplastic anemia. The administration of G-CSF was associated with marked increase in white blood cells counts (WBC) in twelve out of 14 treated patients. In one patient with aplastic anemia the WBC decreased rapidly to the initial value after the cessation of cytokine therapy. Significant increase of platelet number was observed in 6 patients. No toxicity was encountered with the hematopoietic growth factor therapy. Our study points to the fact that G-CSF have a stimulating effect on the regeneration of hematopoiesis, particularly within the granulopoietic compartment. The effect can be obtained both in a case of idiopathic- and cytostatic-dependent marrow aplasia.
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PMID:Treatment of chemotherapy-induced or idiopathic bone marrow aplasia with granulocyte colony-stimulating factor (G-CSF). 857 3

Aplastic anaemia (AA) of the chronic type with severe cytopenia is very frequently a difficult therapeutic problem. Patients with granulocyte values below 0.5 G/l are threatened by infections, incl. sepsis possibly with a fatal outcome. If the pool of stem cells for granulocytes is not completely exhausted and can respond to growth factors, these patients can be treated either chronically and/or in risk situations (e.g. injury, surgery) with preparations of the type of a recombinant, granulocyte colony stimulating factor (rhG-CSF), or granulocyte and monocyte colony stimulating factor (rhGM-CSF). The authors present a review of diagnostic and therapeutic algorithms in patients with the AA syndrome and summarize their own experience with the preparation Neupogen Roche (rhG-CSF).
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PMID:[G-CSF (Neupogen Roche) in the treatment of patients with chronic aplastic anemia with severe neutropenia]. 857 1

Point mutations in the gene for the G-CSF receptor have been reported previously in a subgroup of patients with severe congenital neutropenia. Here, we investigated the frequency of these specific G-CSF receptor mutations in patients with neutropenic disorders undergoing treatment with recombinant human (r-metHu)G-CSF (Filgrastim). Nucleotides 2306 to 2561, including the critical region (nucleotides 2384-2429) from the intracellular domain of the G-CSF receptor gene, were amplified by reverse transcriptase-polymerase chain reaction, and DNA was sequenced directly and after transformation in E. coli. Four of 30 patients with severe congenital neutropenia displayed a point mutation in the tested cytoplasmic region of the G-CSF receptor gene. Two of the four patients with a mutated G-CSF receptor developed acute myeloid leukemia secondary to congenital neutropenia. G-CSF receptor analyses were performed in myeloid cells taken at different time points in the four patients with the mutated receptor, and no correlation between occurrence of the mutation and time or dose of r-metHuG-CSF treatment was found. No point mutations in the G-CSF receptor critical domain could be detected in cells from the other 26 congenital neutropenia patients. Additionally, no G-CSF receptor point mutations could be seen in neutrophils, blood and bone marrow mononuclear cells from patients with cyclic or idiopathic neutropenia, and bone marrow mononuclear cells from patients suffering from severe aplastic anemia. Similar results were obtained by Touw et al., demonstrating that five out of 25 patients with congenital neutropenia reveal G-CSF receptor mutations. These data show that the point mutations in the critical region of the intracellular part of the G-CSF receptor occur only in a subgroup of severe congenital neutropenia patients. Furthermore, our data suggest that the described G-CSF receptor point mutations are not correlated with the start, duration or doses of r-metHuG-CSF treatment, but might result from genetic instability in the G-CSF receptor gene in severe congenital neutropenia.
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PMID:Frequency of point mutations in the gene for the G-CSF receptor in patients with chronic neutropenia undergoing G-CSF therapy. 936 31

Ticlopidine, an inhibitor of platelet aggregation, is widely used for peripheral arterial disease. The use of this drug has been associated with neutropenie and other adverse hematologic effects. Aplastic anemie is a rate complication that carries high mortality. We describe the case of a 91 years old woman with ticlopedine-induced aplastic anemia. The patient was treated with filgrastim brood spectrum antibiotics and other general supportive care measures. Despite initial improvement the old woman died 7 weeks after admission for septic shock. A review of medical literature revealed 24 similar cases. Filgrastim has been used previous by with variable success.
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PMID:[Ticlopidine-induced aplastic anemia. A case report and review of the literature]. 1213 88