[Paroxysmal nocturnal hemoglobinuria].

Paroxysmal nocturnal haemoglobinuria is a rare clonal disease of the hematopoietic stem cell. Since the 80's considerable advances in the biology of this rare disease have been done. The pathophysiology, recent advances in the diagnosis and treatment of this disease are reviewed, pointing out, however, the yet unanswered question with regard to the biology and treatment.