title: Prevalence of paroxysmal nocturnal hemoglobinuria in Chinese patients with Budd-Chiari syndrome or portal vein thrombosis.
abstract: OBJECTIVE
  Routine screening for paroxysmal nocturnal hemoglobinuria (PNH) in patients with Budd-Chiari syndrome (BCS) or portal vein thrombosis (PVT) has been recommended in Western countries. However, little is known about whether the routine screening test should be necessary in Chinese patients with BCS or PVT. We conducted a prospective observational study to examine the prevalence of PNH in these patients.
  
  
  METHODS
  Patients with primary BCS or non-malignant PVT who were consecutively admitted to our department or regularly followed up between September 2009 and December 2011 were eligible for the study and detected the expression of CD55 and CD59 on erythrocytes and granulocytes. The CD55 or CD59 deficiency was considered as the proportion of erythrocytes or granulocytes with normal expression of CD55 or CD59 was less than 90%. PNH was diagnosed by both CD55 and CD59 deficient clone at flow cytometry of peripheral blood cells.
  
  
  RESULTS
  CD55 and/or CD59 deficiencies were found in 1.6% (2/127) of patients with primary BCS, 1.0% (1/100) of non-malignant and non-cirrhotic patients with PVT, and 4.7% (4/85) of cirrhotic patients with PVT. Only one patient had both CD55 and CD59 deficiencies on granulocytes. But he had been diagnosed with PNH before BCS.
  
  
  CONCLUSIONS
  Paroxysmal nocturnal hemoglobinuria was very rare in Chinese patients with BCS or PVT, suggesting that routine screening for PNH should not be indiscriminately performed in such patients.
authors.affiliation: Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China.
authors.forename: Xingshun
authors.initials: X
authors.lastname: Qi
authors.forename: Chuangye
authors.initials: C
authors.lastname: He
authors.forename: Guohong
authors.initials: G
authors.lastname: Han
authors.forename: Zhanxin
authors.initials: Z
authors.lastname: Yin
authors.forename: Feifei
authors.initials: F
authors.lastname: Wu
authors.forename: Qing
authors.initials: Q
authors.lastname: Zhang
authors.forename: Jing
authors.initials: J
authors.lastname: Niu
authors.forename: Kaichun
authors.initials: K
authors.lastname: Wu
authors.forename: Daiming
authors.initials: D
authors.lastname: Fan
mesh_terms: D000328:Adult; D006502:Budd-Chiari Syndrome / Q000453:epidemiology*; D018958:CD55 Antigens / Q000378:metabolism; D018957:CD59 Antigens / Q000378:metabolism; D016009:Chi-Square Distribution; D002681:China / Q000453:epidemiology; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D006098:Granulocytes / Q000378:metabolism; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis* / Q000453:epidemiology*; D006801:Humans; D008103:Liver Cirrhosis / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011169:Portal Vein; D015995:Prevalence; D011446:Prospective Studies; D020246:Venous Thrombosis / Q000453:epidemiology*
chemical_list: D018958:CD55 Antigens; D018957:CD59 Antigens