![]() Mutation or aberrant expression of recombinant signal binding protein of immunoglobulin kJ region ( RBPJ) ( Exp Ther Med 2019 17:4536).Other factors leading to proliferation and expansion of PNH clones include:.Mechanism of mutation is unknown: proposed theory of immune attack on normal HSCs and sparing of PNH stem cell clones due to close association between PNH and acquired aplastic anemic (AA) ( Hematol Transfus Cell Ther 2020 Jul 6 ).PNH is caused by an acquired mutation in the phosphatidylinositol glycan class A ( PIGA) gene this gene produces the glycosylphosphatidylinositol (GPI) anchor proteins (GPI-APs), a posttranslational modification that links cell surface proteins to cell membranes ( Hematol Transfus Cell Ther 2020 Jul 6 ).Penis: priapism or erectile dysfunction, also due to effect of nitric oxide scavenging by free hemoglobin ( Hematol Transfus Cell Ther 2020 Jul 6, Anemia 2011 2011:297364).Esophagus: esophageal spasm, free hemoglobin in plasma depletes nitric oxide (NO), causing smooth muscle dystonia ( Hematol Transfus Cell Ther 2020 Jul 6, Blood 2014 124:2804).Kidneys: renal insufficiency or renal failure ( Hematol Transfus Cell Ther 2020 Jul 6 ).PNH patients with aplastic anemia: 95% have hypocellularity in bone marrow myelopoiesis decreased in 85% and megakaryopoiesis decreased in 100%.Classic PNH: 65% of patients have normal or hypercellular bone marrow erythroid hyperplasia is common myelopoiesis is decreased in 86% and megakaryopoiesis is decreased in 46%.Bone marrow ( J Hematopathol 2013 6:71):.Vascular system: intravascular hemolysis, thrombosis (especially venous thrombosis) in atypical locations such as hepatic vein ( Hematol Transfus Cell Ther 2020 Jul 6 ).
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |