International Journal of Hematology

, Volume 97, Issue 6, pp 749–757

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

  • Jong Wook Lee
  • Jun Ho Jang
  • Jin Seok Kim
  • Sung-Soo Yoon
  • Je-Hwan Lee
  • Yeo-Kyeoung Kim
  • Deog-Yeon Jo
  • Jooseop Chung
  • Sang Kyun Sohn
Original Article

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).

Keywords

Risk factor Hemolysis Thrombosis Mortality Paroxysmal nocturnal hemoglobinuria 

Copyright information

© The Japanese Society of Hematology 2013

Authors and Affiliations

  • Jong Wook Lee
    • 1
  • Jun Ho Jang
    • 2
  • Jin Seok Kim
    • 3
  • Sung-Soo Yoon
    • 4
  • Je-Hwan Lee
    • 5
  • Yeo-Kyeoung Kim
    • 6
  • Deog-Yeon Jo
    • 7
  • Jooseop Chung
    • 8
  • Sang Kyun Sohn
    • 9
  1. 1.Division of Hematology, Seoul St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaSeoulKorea
  2. 2.Division of Hematology-Oncology, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulKorea
  3. 3.Division of HematologyYonsei University College of MedicineSeoulKorea
  4. 4.Division of Hematology-OncologySeoul National University College of MedicineSeoulKorea
  5. 5.Division of HematologyUniversity of Ulsan College of MedicineSeoulKorea
  6. 6.Division of Hematology-OncologyChonnam National University Medical SchoolGwangjuKorea
  7. 7.Division of Hematology-OncologyChungnam National University School of MedicineDaejeonKorea
  8. 8.Division of Hematology-OncologyPusan National University School of MedicinePusanKorea
  9. 9.Division of Hematology-OncologyKyungpook National University School of MedicineDaeguKorea

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