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Letter to the Editor

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Blood Res 2017; 52(4):

Published online December 31, 2017

https://doi.org/10.5045/br.2017.52.4.337

© The Korean Society of Hematology

Low-dose prednisolone in patients with paroxysmal nocturnal hemoglobinuria and inadequate response to eculizumab

Ji Young Moon, Deog-Yeon Jo*, So Yeon Lee, Deok Yeong Kim, Seung-Woo Baek, and Ik-Chan Song

Author Info. +

Department of Internal Medicine, Chungnam National University Hospital, Daejeon, Korea.

Correspondence to : Deog-Yeon Jo. Department of Internal Medicine, Chungnam National University Hospital, 282 Munhwa-ro, Jung-gu, Daejeon 35015, Korea. deogyeon@cnu.ac.kr

Received: December 14, 2016; Revised: February 27, 2017; Accepted: May 25, 2017

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

TABLES

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Table. 1.

Table 1 Characteristics of patients with inadequate response to eculizumab.

a)At the time additional prednisolone was started. b)Reference range: 23.0–46.0 U/mL. c)Reference range: 50–200 ng/mL. d)Reference range: 3.7–31.5 mIU/mL. e)CD55-negative cells/CD59-negative cells.

Abbreviations: CI, cerebral infarction; CKD, chronic kidney disease; LDH, lactate dehydrogenase; NT, not tested; Pd, low-dose prednisolone treatment (5 mg/day); RPI, red cell production index.

REFERENCES

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  1. Luzzatto, L, Gianfaldoni, G, Notaro, R. Management of paroxysmal nocturnal haemoglobinuria: a personal view. Br J Haematol, 2011;153;709-720.
    Pubmed
  2. Brodsky, RA. How I treat paroxysmal nocturnal hemoglobinuria. Blood, 2009;113;6522-6527.
    Pubmed
  3. Hillmen, P, Young, NS, Schubert, J, et al. The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med, 2006;355;1233-1243.
    Pubmed
  4. Hillmen, P, Muus, P, Dührsen, U, et al. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria. Blood, 2007;110;4123-4128.
    Pubmed
  5. Kelly, RJ, Hill, A, Arnold, LM, et al. Long-term treatment with eculizumab in paroxysmal nocturnal hemoglobinuria: sustained efficacy and improved survival. Blood, 2011;117;6786-6792.
    Pubmed
  6. Röth, A, Hock, C, Konik, A, Christoph, S, Dührsen, U. Chronic treatment of paroxysmal nocturnal hemoglobinuria patients with eculizumab: safety, efficacy, and unexpected laboratory phenomena. Int J Hematol, 2011;93;704-714.
    Pubmed
  7. Risitano, AM, Notaro, R, Marando, L, et al. Complement fraction 3 binding on erythrocytes as additional mechanism of disease in paroxysmal nocturnal hemoglobinuria patients treated by eculizumab. Blood, 2009;113;4094-4100.
    Pubmed
  8. Risitano, AM, Marando, L, Seneca, E, Rotoli, B. Hemoglobin normalization after splenectomy in a paroxysmal nocturnal hemoglobinuria patient treated by eculizumab. Blood, 2008;112;449-451.
    Pubmed
  9. Peffault de Latour, R, Fremeaux-Bacchi, V, Porcher, R, et al. Assessing complement blockade in patients with paroxysmal nocturnal hemoglobinuria receiving eculizumab. Blood, 2015;125;775-783.
    Pubmed
  10. McMullin, MF, Hillmen, P, Elder, GE, Lappin, TR, Luzzatto, L. Serum erythropoietin levels in paroxysmal nocturnal haemoglobinuria: implications for therapy. Br J Haematol, 1996;92;815-817.
    Pubmed
  11. Berzuini, A, Montanelli, F, Prati, D. Hemolytic anemia after eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med, 2010;363;993-994.
    Pubmed
  12. Risitano, AM, Notaro, R, Luzzatto, L, Hill, A, Kelly, R, Hillmen, P. Paroxysmal nocturnal hemoglobinuria--hemolysis before and after eculizumab. N Engl J Med, 2010;363;2270-2272.
    Pubmed

Article

Letter to the Editor

Blood Res 2017; 52(4): 337-339

Published online December 31, 2017 https://doi.org/10.5045/br.2017.52.4.337

Copyright © The Korean Society of Hematology.

Low-dose prednisolone in patients with paroxysmal nocturnal hemoglobinuria and inadequate response to eculizumab

Ji Young Moon, Deog-Yeon Jo*, So Yeon Lee, Deok Yeong Kim, Seung-Woo Baek, and Ik-Chan Song

Department of Internal Medicine, Chungnam National University Hospital, Daejeon, Korea.

Correspondence to:Deog-Yeon Jo. Department of Internal Medicine, Chungnam National University Hospital, 282 Munhwa-ro, Jung-gu, Daejeon 35015, Korea. deogyeon@cnu.ac.kr

Received: December 14, 2016; Revised: February 27, 2017; Accepted: May 25, 2017

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

    Table 1 . Characteristics of patients with inadequate response to eculizumab..

    a)At the time additional prednisolone was started. b)Reference range: 23.0–46.0 U/mL. c)Reference range: 50–200 ng/mL. d)Reference range: 3.7–31.5 mIU/mL. e)CD55-negative cells/CD59-negative cells..

    Abbreviations: CI, cerebral infarction; CKD, chronic kidney disease; LDH, lactate dehydrogenase; NT, not tested; Pd, low-dose prednisolone treatment (5 mg/day); RPI, red cell production index..

    References

    1. Luzzatto, L, Gianfaldoni, G, Notaro, R. Management of paroxysmal nocturnal haemoglobinuria: a personal view. Br J Haematol, 2011;153;709-720.
      Pubmed
    2. Brodsky, RA. How I treat paroxysmal nocturnal hemoglobinuria. Blood, 2009;113;6522-6527.
      Pubmed
    3. Hillmen, P, Young, NS, Schubert, J, et al. The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med, 2006;355;1233-1243.
      Pubmed
    4. Hillmen, P, Muus, P, Dührsen, U, et al. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria. Blood, 2007;110;4123-4128.
      Pubmed
    5. Kelly, RJ, Hill, A, Arnold, LM, et al. Long-term treatment with eculizumab in paroxysmal nocturnal hemoglobinuria: sustained efficacy and improved survival. Blood, 2011;117;6786-6792.
      Pubmed
    6. Röth, A, Hock, C, Konik, A, Christoph, S, Dührsen, U. Chronic treatment of paroxysmal nocturnal hemoglobinuria patients with eculizumab: safety, efficacy, and unexpected laboratory phenomena. Int J Hematol, 2011;93;704-714.
      Pubmed
    7. Risitano, AM, Notaro, R, Marando, L, et al. Complement fraction 3 binding on erythrocytes as additional mechanism of disease in paroxysmal nocturnal hemoglobinuria patients treated by eculizumab. Blood, 2009;113;4094-4100.
      Pubmed
    8. Risitano, AM, Marando, L, Seneca, E, Rotoli, B. Hemoglobin normalization after splenectomy in a paroxysmal nocturnal hemoglobinuria patient treated by eculizumab. Blood, 2008;112;449-451.
      Pubmed
    9. Peffault de Latour, R, Fremeaux-Bacchi, V, Porcher, R, et al. Assessing complement blockade in patients with paroxysmal nocturnal hemoglobinuria receiving eculizumab. Blood, 2015;125;775-783.
      Pubmed
    10. McMullin, MF, Hillmen, P, Elder, GE, Lappin, TR, Luzzatto, L. Serum erythropoietin levels in paroxysmal nocturnal haemoglobinuria: implications for therapy. Br J Haematol, 1996;92;815-817.
      Pubmed
    11. Berzuini, A, Montanelli, F, Prati, D. Hemolytic anemia after eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med, 2010;363;993-994.
      Pubmed
    12. Risitano, AM, Notaro, R, Luzzatto, L, Hill, A, Kelly, R, Hillmen, P. Paroxysmal nocturnal hemoglobinuria--hemolysis before and after eculizumab. N Engl J Med, 2010;363;2270-2272.
      Pubmed
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