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Distinct subgroups of paroxysmal nocturnal hemoglobinuria (PNH) with cytopenia: results from South Korean National PNH Registry

Authors
 Jin Seok Kim  ;  Jun Ho Jang  ;  Sung-Soo Yoon  ;  Je-Hwan Lee  ;  Yeo-Kyeoung Kim  ;  Deog-Yeon Jo  ;  Joo Seop Chung  ;  Sang Kyun Sohn  ;  Jong Wook Lee 
Citation
 ANNALS OF HEMATOLOGY, Vol.95(1) : 125-133, 2016 
Journal Title
 ANNALS OF HEMATOLOGY 
ISSN
 0939-5555 
Issue Date
2016
MeSH
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Hemoglobinuria, Paroxysmal/classification* ; Hemoglobinuria, Paroxysmal/diagnosis* ; Hemoglobinuria, Paroxysmal/mortality ; Humans ; Male ; Middle Aged ; Pancytopenia/classification* ; Pancytopenia/diagnosis* ; Pancytopenia/mortality ; Registries* ; Republic of Korea/epidemiology ; Retrospective Studies ; Survival Rate/trends ; Young Adult
Keywords
Aplastic anemia ; Cytopenia ; Paroxysmal nocturnal hemoglobinuria ; Thromboembolism
Abstract
We retrospectively assessed the clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria (PNH) according to severity of cytopenia. A total of 282 patients with hematological parameters assessed at the time of diagnosis of PNH were included. There were 24 patients with PNH/severe aplastic anemia (SAA) (at least two of the three criteria; hemoglobin ≤8 g/dL; absolute neutrophil count (ANC) <0.5 × 10(9)/L; platelet count <20 × 10(9)/L), 96 patients with PNH/aplastic anemia (AA) (at least two of the three criteria; hemoglobin ≤10 g/dL; ANC 0.5-1.5 × 10(9)/L; platelet count 20-100 × 10(9)/L), and 162 classic PNH patients. Compared with the classic PNH subgroup, the PNH/SAA subgroup had a significantly lower median granulocyte PNH clone size (26.7 vs. 51.0 %, P = 0.021) and lower incidence of lactate dehydrogenase ≥1.5 times the upper limit of normal (52.9 vs. 80.0 %, P = 0.049). The incidence of thromboembolism was similar in both subgroups. Overall survival was significantly lower in the PNH/SAA subgroup than in the classic PNH subgroup (P = 0.033). Our findings suggest that identification of patients with PNH/SAA at the time of diagnosis is important because of different clinical manifestations and poorer outcome compared with patients with classic PNH (clinicaltrials.gov identifier: #NCT01224483).
Full Text
http://link.springer.com/article/10.1007/s00277-015-2511-z
DOI
10.1007/s00277-015-2511-z
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Jin Seok(김진석) ORCID logo https://orcid.org/0000-0001-8986-8436
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/146286
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