Abstract | BACKGROUND: METHODS: We enrolled 6 patients with PNH who had clinically significant hemolysis. Eculizumab was administered intravenously at 600 mg/week for the first 4 weeks followed by 900 mg at week 5 and 2nd weekly thereafter. RESULTS: Three men and 3 women with a median age of 39.5 years (24-61 years) were enrolled. The median duration of PNH was 11 years (6-25 years). Hemolysis occurred in all patients [median lactate dehydrogenase (LDH) level, 7.95 times the upper limit of the reference range of LDH]. All patients treated with eculizumab had a rapid and sustained reduction in the degree of hemolysis. RBC transfusion requirements for 3 months were decreased from 0-12 units (median requirement, 1.5 units) to 0-6 units (median requirement, 0 units). Improvement in fatigue was noted in 4 patients. Further, 5 patients who had been receiving corticosteroids either reduced the dose or discontinued therapy. No significant adverse events related to eculizumab therapy were observed. CONCLUSION:
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Authors | Jin Seok Kim, Jong Wook Lee, Byoung Kook Kim, Je-Hwan Lee, Jooseop Chung |
Journal | The Korean journal of hematology
(Korean J Hematol)
Vol. 45
Issue 4
Pg. 269-74
(Dec 2010)
ISSN: 2092-9129 [Electronic] Korea (South) |
PMID | 21253430
(Publication Type: Journal Article)
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