Study on the clinical features of patients with aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome and typical paroxysmal nocturnal hemoglobinuria.
- VernacularTitle:再生障碍性贫血-阵发性睡眠性血红蛋白尿综合征与典型阵发性睡眠性血红蛋白尿症的临床对照研究
- Author:
Mingfeng ZHAO
;
Zonghong SHAO
;
Ke LI
- Publication Type:Journal Article
- Keywords:
Aplastic anemia paroxysmal nocturnal hemoglobinuria syndrome\ Paroxysmal nocturnal hemoglobinuria\ GPI anchor protein\ Diagnosis
- From:
Chinese Journal of Practical Internal Medicine
2001;0(03):-
- CountryChina
- Language:Chinese
-
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 features:①Lower 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.
