Study on Clinical and Laboratory Features of Childhood Paroxysmal Nocturnal Hemoglobinuria
- VernacularTitle:阵发性夜间血红蛋白尿的临床及实验室检查特点
- Author:
Ai-dong, LU
;
Le-ping, ZHANG
;
Gui-lan, LIU
;
Bin, WANG
- Publication Type:Journal Article
- Keywords:
paroxysmal nocturnal hemoglobinuria;
children;
clinical feature;
glycosyl-phosphatidyl inositol;
CD_(55);
CD_(59)
- From:Journal of Applied Clinical Pediatrics
1993;0(03):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To learn more about the clinical and laboratory features of childhood paroxysmal nocturnal hemoglobinuria(PNH) and to improve the diagnosis.Methods The clinical and laboratory features of 12 cases of PNH were analyzed,who were diagnosed from January 2000 to November 2004,and the positive responses to treatments were observed.Results 1) The youngest age of onset was 2 years;the disease often manifested with anemia(100%),recurrent infections(50%) and hemorrhages(33%),occurring mainly in skin and mucosa.No patient developed a thrombosis.2) 66.7% of the patients showed peripheral blood cytopenia.Dysplasia of bone marrow was observed in 25% of patients.Fifty percent of them had an increased percentage of erythroid lineage.3) Positive hemolytic tests included urine OB 41.6%,Ham′s test 33.3% and Rous′ test 25%.Glycosyl-phosphatidyl inositol(GPI) deficient cells were found in 100% of the patients.4) 57.1% of patients was improved after being treated with adrenocortical hormone,androgens or cysporin.Conclusions Besides hemoglobinuria,peripheral blood cytopenia were also the common manifestation of PNH.Flow cytometry based immunophenotypic methods for the analysis of CD_(55) and CD_(59) may improve the diagnosis of PNH.J Appl Clin Pediatr,2006,21(3):153-154
