Aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) are rare clonal bone marrow disorders. AA is characterized by autoimmune destruction of bone marrow stem cells and pancytopenia. In PNH, acquired genetic mutation and impaired glycoprotein synthesis leads to hemolysis and thrombus formation. The combination of both diseases represents a diagnostic and therapeutic dilemma, since increasing evidence suggests the connection between autoimmunity in AA clonal expansion of PNH. The purpose of this publication is to illustrate the pathogenetic relationship between AA and PNH by review of available literature regarding the mechanisms of immune-mediated destruction of bone marrow and clonal expansion in combination of these hematological pathologies. A clinical case is presented as an example of this phenomenon.
Baidurin et al. (Mon,) studied this question.