Study on the clinical features of patients with aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome and typical paroxysmal nocturnal hemoglobinuria / 中国实用内科杂志

ABSTRACT

Objective To explore the differences of clinical features and relationship between aplastic anemia paroxysmal nocturnal hemoglobinuria syndrome(AA PNH syndrome)and typical paroxysmal nocturnal hemoglobinuria(t PNH).Methods A case control study on the discrepancies of clinical and laboratory features between patients with AA PNH syndrome and t PNH was carried out.Results Compared with t PNH,AA PNH syndrome showed following features①Lower frequencies of venous thrombosis,jaundice and enlarged liver or spleen.②Higher percentages of pancytopenia and bone marrow hypoplasia.③Lower percentages of positive hemolysis tests.The percentages of CD55 and CD59 of peripheral blood cells were not significantly different in most cases of both groups.④Immunoglobulins and subgroups of T lymphocytes were normal in cases of both groups.⑤Adrenocortical hormone was effective in cases of both groups.Conclusion AA PNH syndrome shares a same pathophysiology with t PNH;CD55 and CD59 tests can improve the diagnosis of AA PNH syndrome.