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Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry

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Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by chronic, complement-mediated hemolysis, frequently leading to debilitating clinical symptoms and life-threatening complications such as thromboembolism (TE). A retrospective analysis was performed on 301 patients from the South Korean National PNH Registry to describe disease burden and identify TE-associated risk factors. TE was identified in 18 % of patients and was associated with increased risk for mortality [odds ratio (OR), 6.85; P < 0.001]. A multivariate analysis showed that PNH patients with elevated hemolysis [lactate dehydrogenase (LDH) levels ≥1.5 times the upper limit of normal (ULN)] at diagnosis were at significantly higher risk for TE than patients with LDH <1.5 × ULN (OR 7.0; P = 0.013). The combination of LDH ≥1.5 × ULN with the clinical symptoms of abdominal pain, chest pain, dyspnea, or hemoglobinuria was associated with a greater increased risk for TE than elevated hemolysis or clinical symptoms alone. Continuous monitoring of these risk factors is critical for identifying PNH patients at risk for morbidities and mortality and allowing early intervention. (clinicaltrials.gov identifier: NCT01224483).

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Acknowledgments

The authors would like to thank G. Khursigara, PhD, of Alexion Pharmaceuticals; H. H. Cho of Handok Pharmaceuticals; K. Bonin, PhD, M. Hughes, PhD, and J. Safran of Infusion Communications for assistance in writing and editing; and P. McCloud, PhD, of McCloud Consulting for statistical analysis and interpretation. This work was supported by the Korean Society of Hematology.

Conflict of interest

JWL has received honoraria for lectures and advisory boards and has received consulting fees from Alexion Pharmaceuticals. JHJ, JSK, S-SY, J-HL, and D-YJ have received consulting fees from Alexion Pharmaceuticals. All other authors declare that they have no conflicts of interest.

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Authors and Affiliations

  1. Division of Hematology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 505 Banpo-dong, Seocho-gu, Seoul, 137-701, Korea

    Jong Wook Lee

  2. Division of Hematology-Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

    Jun Ho Jang

  3. Division of Hematology, Yonsei University College of Medicine, Seoul, Korea

    Jin Seok Kim

  4. Division of Hematology-Oncology, Seoul National University College of Medicine, Seoul, Korea

    Sung-Soo Yoon

  5. Division of Hematology, University of Ulsan College of Medicine, Seoul, Korea

    Je-Hwan Lee

  6. Division of Hematology-Oncology, Chonnam National University Medical School, Gwangju, Korea

    Yeo-Kyeoung Kim

  7. Division of Hematology-Oncology, Chungnam National University School of Medicine, Daejeon, Korea

    Deog-Yeon Jo

  8. Division of Hematology-Oncology, Pusan National University School of Medicine, Pusan, Korea

    Jooseop Chung

  9. Division of Hematology-Oncology, Kyungpook National University School of Medicine, Daegu, Korea

    Sang Kyun Sohn

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  1. Jong Wook Lee
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  2. Jun Ho Jang
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Correspondence to Jong Wook Lee.

Additional information

Jong Wook Lee and Jun Ho Jang contributed equally to this work.

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Lee, J.W., Jang, J.H., Kim, J.S. et al. Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. Int J Hematol 97, 749–757 (2013). https://doi.org/10.1007/s12185-013-1346-4

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  • DOI: https://doi.org/10.1007/s12185-013-1346-4

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