Blood Res.  2017 Jun;52(2):145-148. 10.5045/br.2017.52.2.145.

The first case of paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome treated with complement inhibitor eculizumab in Korea

• Kim H
• Kim IS
• Cho SH
• Lee HJ
• Chang CL
• Yoon KT

• KMID: 2413337
• DOI: http://doi.org/10.5045/br.2017.52.2.145

Abstract

No abstract available.

MeSH Terms

Budd-Chiari Syndrome*
Complement System Proteins*
Hemoglobinuria, Paroxysmal*
Korea*
Complement System Proteins

Figure

• 

  Fig. 1 Abdominal computed tomography evidence of Budd-Chiari Syndrome. (A) The liver shows heterogeneous enhancement and multiple ill-defined hypodense lesions with venous congestion. (B) Non-obstructive thrombus in inferior vena cava stent and multiple enhancing nodules at the liver are seen.

• 

  Fig. 2 Flow cytometric analysis of granulocytes using FLAER. (A) As a control setting, there was no evidence of decreased (type II PNH cells) or absent (type III PNH cells) expression of FLAER. (B) In this patient, decreased (42.4%) and absent (57.0%) FLAER fractions were detected, suggesting that 99.4% of PNH granulocyte clones had a FLAER deficiency, which is consistent with classic PNH. Abbreviations: FITC, fluorescein isothiocyanate; FLAER, fluoresceinlabeled proaerolysin; PNH, paroxysmal nocturnal hemoglobinuria.

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