title: Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry.
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.
authors: Division of Hematology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 505 Banpo-dong, Seocho-gu, Seoul, Korea. jwlee@catholic.ac.kr
authors: Jong Wook
authors: JW
authors: Lee
authors: Jun Ho
authors: JH
authors: Jang
authors: Jin Seok
authors: JS
authors: Kim
authors: Sung-Soo
authors: SS
authors: Yoon
authors: Je-Hwan
authors: JH
authors: Lee
authors: Yeo-Kyeoung
authors: YK
authors: Kim
authors: Deog-Yeon
authors: DY
authors: Jo
authors: Jooseop
authors: J
authors: Chung
authors: Sang Kyun
authors: SK
authors: Sohn
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D045022:Lactate Dehydrogenases / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012372:ROC Curve; D012042:Registries; D056910:Republic of Korea; D012189:Retrospective Studies; D012307:Risk Factors; D013923:Thromboembolism / Q000175:diagnosis / Q000209:etiology; D013927:Thrombosis / Q000175:diagnosis* / Q000209:etiology*; D055815:Young Adult
chemical_list: D045022:Lactate Dehydrogenases
references: Blood. 2005 Oct 1;106(7):2559-65
references: 15985537
references: Thromb Res. 2010 Apr;125 Suppl 2:S106-7
references: 20433987
references: Br J Haematol. 2012 Aug;158(3):409-14
references: 22639982
references: JAMA. 2005 Apr 6;293(13):1653-62
references: 15811985
references: Med Clin North Am. 1992 May;76(3):649-68
references: 1578962
references: Blood. 1993 Aug 15;82(4):1192-6
references: 7688991
references: Lancet. 1996 Aug 31;348(9027):573-7
references: 8774569
references: Blood. 2008 Feb 15;111(4):1840-7
references: 18055865
references: Haematologica. 2005 Dec;90(12 Suppl):ECR40
references: 16464755
references: Semin Thromb Hemost. 2011 Apr;37(3):315-21
references: 21455865
references: Annu Rev Physiol. 1995;57:771-90
references: 7539996
references: Blood. 2005 Dec 1;106(12):3699-709
references: 16051736
references: Blood. 2011 Jun 23;117(25):6786-92
references: 21460245
references: Blood. 2007 Dec 1;110(12):4123-8
references: 17702897
references: Eur J Haematol. 2011 Jul;87(1):37-45
references: 21447004
references: Am J Hematol. 2010 Aug;85(8):553-9
references: 20658586
references: Medicine (Baltimore). 2004 May;83(3):193-207
references: 15118546
references: N Engl J Med. 2006 Sep 21;355(12):1233-43
references: 16990386
references: Cell. 1993 May 21;73(4):703-11
references: 8500164
references: Blood. 2003 Nov 15;102(10):3587-91
references: 12893760
references: Br J Haematol. 2010 May;149(3):414-25
references: 20230403
references: Br J Haematol. 2004 Jul;126(1):133-8
references: 15198744
references: Blood. 2008 Oct 15;112(8):3099-106
references: 18535202
references: N Engl J Med. 2004 Feb 5;350(6):552-9
references: 14762182
references: N Engl J Med. 1995 Nov 9;333(19):1253-8
references: 7566002
references: Int J Hematol. 2011 Jan;93(1):36-46
references: 21222185
references: Biologics. 2008 Jun;2(2):205-22
references: 19707355
references: J Thromb Haemost. 2007 Mar;5(3):642-5
references: 17319910
references: Am J Hematol. 1984 Jan;16(1):75-81
references: 6695911
references: Ann Clin Lab Sci. 2010 Summer;40(3):226-32
references: 20689133
references: Dig Dis Sci. 2002 Jan;47(1):58-64
references: 11837733