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

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

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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 × 109/L; platelet count <20 × 109/L), 96 patients with PNH/aplastic anemia (AA) (at least two of the three criteria; hemoglobin ≤10 g/dL; ANC 0.5–1.5 × 109/L; platelet count 20–100 × 109/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).

Original languageEnglish
Pages (from-to)125-133
Number of pages9
JournalAnnals of Hematology
Volume95
Issue number1
DOIs
Publication statusPublished - 2016 Jan 1

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Paroxysmal Hemoglobinuria
Registries
Aplastic Anemia
Platelet Count
Hemoglobins
Neutrophils
Thromboembolism
Incidence
L-Lactate Dehydrogenase
Granulocytes

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Kim, Jin Seok ; Jang, Jun Ho ; Yoon, Sung Soo ; Lee, Je Hwan ; Kim, Yeo Kyeoung ; Jo, Deog Yeon ; Chung, Joo Seop ; Sohn, Sang Kyun ; Lee, Jong Wook. / Distinct subgroups of paroxysmal nocturnal hemoglobinuria (PNH) with cytopenia : results from South Korean National PNH Registry. In: Annals of Hematology. 2016 ; Vol. 95, No. 1. pp. 125-133.
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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 × 109/L; platelet count <20 × 109/L), 96 patients with PNH/aplastic anemia (AA) (at least two of the three criteria; hemoglobin ≤10 g/dL; ANC 0.5–1.5 × 109/L; platelet count 20–100 × 109/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).",
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Distinct subgroups of paroxysmal nocturnal hemoglobinuria (PNH) with cytopenia : results from South Korean National PNH Registry. / Kim, Jin Seok; Jang, Jun Ho; Yoon, Sung Soo; Lee, Je Hwan; Kim, Yeo Kyeoung; Jo, Deog Yeon; Chung, Joo Seop; Sohn, Sang Kyun; Lee, Jong Wook.

In: Annals of Hematology, Vol. 95, No. 1, 01.01.2016, p. 125-133.

Research output: Contribution to journalArticle

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AU - Kim, Jin Seok

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AU - Lee, Jong Wook

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N2 - 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 × 109/L; platelet count <20 × 109/L), 96 patients with PNH/aplastic anemia (AA) (at least two of the three criteria; hemoglobin ≤10 g/dL; ANC 0.5–1.5 × 109/L; platelet count 20–100 × 109/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).

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