2020 Volume 57 Issue 5 Pages 385-389
Paroxysmal nocturnal hemoglobinuria (PNH) is rare in children. Most childhood PNH involves progression from aplastic anemia (AA). A 15-year-old girl presented with pancytopenia and severe hypoplastic bone marrow. PNH-type blood cell-positive AA was diagnosed from findings that 1.9% of red blood cells (RBCs) and 50.6% of granulocytes were PNH-type blood cells (CD55–/59–). She received immunosuppressive therapy (IST) and achieved complete remission six months later, but her disease then became chronic. At 23 years of age, she showed thrombocytopenia and hemolytic anemia. Bone marrow findings showed erythroid hyperplasia, and percentages of PNH RBCs and PNH granulocytes were 24.4% and 84.6%, respectively. AA-PNH syndrome was diagnosed. About 4–9% of cases of AA in adulthood progresses to PNH. Cases of childhood AA in remission after IST require long-term follow-up into adulthood for hemolytic anemia and PNH-type blood cells.
