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Prevalence of paroxysmal nocturnal hemoglobinuria in Chinese patients with Budd-Chiari syndrome or portal vein thrombosis

Authors

  • Xingshun Qi,

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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    • Authors' contributions: study concept and design: QX, HG; acquisition of data: QX, HC, WF, ZY, NJ, HG, WK, FD; analysis and interpretation of data: QX, HC, HG, WF, ZY, NJ, ZQ, WK, FD; drafting of the manuscript: QX; critical revision of the manuscript for important intellectual content: WF, ZQ, HG, HC, ZY, NJ, WK, FD; statistical analysis: QX; study supervision: HG.
  • Chuangye He,

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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    • Authors' contributions: study concept and design: QX, HG; acquisition of data: QX, HC, WF, ZY, NJ, HG, WK, FD; analysis and interpretation of data: QX, HC, HG, WF, ZY, NJ, ZQ, WK, FD; drafting of the manuscript: QX; critical revision of the manuscript for important intellectual content: WF, ZQ, HG, HC, ZY, NJ, WK, FD; statistical analysis: QX; study supervision: HG.
  • Guohong Han,

    Corresponding author
    • Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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  • Zhanxin Yin,

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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  • Feifei Wu,

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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  • Qing Zhang,

    1. Hightrust Diagnostics Laboratory, Beijing, China
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  • Jing Niu,

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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  • Kaichun Wu,

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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  • Daiming Fan

    1. Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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  • These authors contributed equally to this work.

Correspondence

Professor Guohong Han, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, no. 15 West Changle Road, Xi'an, 710032, China. E-mail: guohhan@126.com

Abstract

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 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.

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