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)

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired haemolytic anaemia, clonal in nature, due to somatic mutation. PNH may evolve to aplastic anaemia; more rarely to a myelodysplastic syndrome (MDS) or to acute myeloid leukaemia (AML). We have studied a patient who suffered from PNH and later developed refractory anaemia with ringed sideroblasts (RARS) associated with trisomy 8. By testing peripheral blood cells with appropriate antibodies we have shown that all of the red cells, neutrophils and monocytes, as well as 20% of the lymphocytes, belonged to the PNH clone; in contrast, only 43% of neutrophils and 22% of monocytes belonged to the MDS clone. We infer that the MDS must have arisen from within the PNS clone.
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PMID:Myelodysplasia in a patient with pre-existing paroxysmal nocturnal haemoglobinuria: a clonal disease originating from within a clonal disease. 794 86

The study was aimed to investigate the action of Panax Notoginosides (PNS, extracted from notoginseng herb) on the expression of the apoptosis-related proteins (Daxx, Fas) and transcription factors (NFkB, c-Rel) in the hematopoietic cells and to explore the mechanisms of supporting cells to survive. The colony formation of CFU-GM and CFU-E in human bone marrow was assayed in the presence of various concentrations of PNS. The viability of cells was assayed by trypan blue and the changes of cell morphology were observed with microscope. The Annexin-V positive cells were detected by FCM. Three lineages of human myeloid HL-60, erythroid K562, megakaryoid CHRF-288 and Meg-01 cells were incubated in addition of PNS (10 mg/L) for 14 days. The nuclear or cytoplasm protein of cells was extracted and analyzed by Western blot with monoclonal antibodies against Daxx, Fas or NFkB, c-Rel. The results showed: (1) the proliferation on hematopoietic progenitor cells (CFU-GM and CFU-E) and four cell lines was promoted by PNS; (2) after the four cell lines were promoted by PNS and hungered through wiping off the sera, the viability of the four cell lines was high without significant morphological change and neither the detection of Annexin-V positive cells; (3) the expression of Daxx and Fas protein could be inhibited by PNS. Western Blot showed that Daxx in four cell lines treated by PNS were 33.3-61.5% lower than that in untreated controls. The Fas protein was also descended in three cell lines of K562, CHRF-288 and Meg-01 by 33.3-71.4% respectively, while Fas protein in HL-60 cells was no detectable difference after PNS treatment. (4) The transcription factors NFkB and c-Rel protein could be increased by PNS. The NFkB, c-Rel protein were also enhanced in three cell lines of K562, CHRF-288 and Meg-01 by (2.0-2.7) and (1.5-2.3)-fold respectively, while there were also no detectable difference in HL-60 cells after PNS treatment. It is concluded that PNS inhibites the expression of Daxx and Fas proteins, may decrease the apoptosis of the hematopoietic cells. The level of NFkB and c-Rel proteins can be enhanced by PNS, which not only stimulates the proliferation of cells, but also inhibits the activity of the waterfall of caspase and apoptosis of the hematopoietic cells. PNS may treat the disease with over-apoptosis of hematopoietic cells, as aplastic anemia.
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PMID:[Expression of apoptosis-related proteins in the human bone marrow hematopoietic cells treated by Panax Notoginosides]. 1663 11