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Prevalence of paroxysmal nocturnal hemoglobinuria in C hinese patients with B udd‐ C hiari syndrome or portal vein thrombosis
Author(s) -
Qi Xingshun,
He Chuangye,
Han Guohong,
Yin Zhanxin,
Wu Feifei,
Zhang Qing,
Niu Jing,
Wu Kaichun,
Fan Daiming
Publication year - 2013
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1111/j.1440-1746.2012.07282.x
Subject(s) - paroxysmal nocturnal hemoglobinuria , medicine , cd59 , gastroenterology , hemoglobinuria , portal vein thrombosis , thrombosis , eculizumab , immunology , hemolysis , antibody , complement system
Background and Aim 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 S eptember 2009 and D ecember 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 P aroxysmal 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.