Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is acquired chronic hemolytic anemia characterized by hemoglobinuria and/or hemosiderinuria but presents variable clinical signs.
In recent years significant number of PNH associated with aplastic anemia have been published and the relationship between these two diseases has been noticed. More recently PNH associated with myeloproliferative disorders-myelofibrosis, acute and chronic myeloid leukemia and so forth have been reported.
I have an interest in this problem and discussed association PNH with these blood diseases in the literature.
1) Association with aplastic anemia.
Most cases reported to develop to PNH from aplastic anemia were found to be PNH manifested with pancytopenia at the onset. There were a few cases developed to PNH from atypical or secondary aplastic anemia, a few cases developed to aplastic anemia-PNH syndrome from aplastic anemia (mainly atypical) and a case developed to PNH through aplastic anemia-PNH syndrome from atypical aplastic anemia.
2) Association with myeloproliferative disorders.
There were nine cases complicated with PNH and myelofibrosis. In six cases there was clear evidence for the development of PNH on preexisting myelofibrosis, in other three cases the time relationship between the onset of myelofibrosis and PNH was not clear. Only one case complicated chronic myeloid leukemia and PNH was found. There were eight PNH cases terminated in acute leukemia or erythroleukemia. It is interesting to note that in five cases followed-up hemoglobinuria and/or hemosiderinuria and abnormalities of PNH red cells disappeared before or after the onset or leukemia of erythroleukemia.
