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, Jinseok Kim, Sung Soo Yoon, Je Hwan Lee, Yeo Kyeoung Kim, Deog Yeon Jo, Jooseop Chung, Sang Kyun Sohn

Research output: Contribution to journalArticle

48 Citations (Scopus)

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).

Original languageEnglish
Pages (from-to)749-757
Number of pages9
JournalInternational Journal of Hematology
Volume97
Issue number6
DOIs
Publication statusPublished - 2013 Jun 1

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Paroxysmal Hemoglobinuria
Thromboembolism
Signs and Symptoms
Registries
Thrombosis
Hemolysis
L-Lactate Dehydrogenase
Odds Ratio
Hemoglobinuria
Mortality
Chest Pain
Dyspnea
Abdominal Pain
Multivariate Analysis
Morbidity

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Lee, Jong Wook ; Jang, Jun Ho ; Kim, Jinseok ; Yoon, Sung Soo ; Lee, Je Hwan ; Kim, Yeo Kyeoung ; Jo, Deog Yeon ; Chung, Jooseop ; Sohn, Sang Kyun. / Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. In: International Journal of Hematology. 2013 ; Vol. 97, No. 6. pp. 749-757.
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Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. / Lee, Jong Wook; Jang, Jun Ho; Kim, Jinseok; Yoon, Sung Soo; Lee, Je Hwan; Kim, Yeo Kyeoung; Jo, Deog Yeon; Chung, Jooseop; Sohn, Sang Kyun.

In: International Journal of Hematology, Vol. 97, No. 6, 01.06.2013, p. 749-757.

Research output: Contribution to journalArticle

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