ArticleEuropean journal of haematology2011
Natural history of paroxysmal nocturnal hemoglobinuria clones in patients presenting as aplastic anemia.
Article in European journal of haematology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 78 citations in OpenAlex.
- Ferroptosis in kidney disease.Nature reviews. Nephrology · 2026Review
- Pancytopenia in the Emergency Department.The Journal of pediatrics · 2024Article
- Swiss Survey on current practices and opinions on clinical constellations triggering the search for PNH clones.Frontiers in medicine · 2023Article
- The spectrum of paroxysmal nocturnal hemoglobinuria clinical presentation in a Brazilian single referral center.Annals of hematology · 2022Article
- Aplastic anemia and paroxysmal nocturnal hemoglobinuria in children and adults in two centers of Northern Greece.Frontiers in oncology · 2022Article
- Approach to the diagnosis of aplastic anemia.Blood advances · 2021Review
- When Should We Think of Myelodysplasia or Bone Marrow Failure in a Thrombocytopenic Patient? A Practical Approach to Diagnosis.Journal of clinical medicine · 2021Review
- Baseline clinical characteristics and disease burden in patients with paroxysmal nocturnal hemoglobinuria (PNH): updated analysis from the International PNH Registry.Annals of hematology · 2020Article
- Laboratory studies for paroxysmal nocturnal hemoglobinuria, with emphasis on flow cytometry.Practical laboratory medicine · 2020Review
- Flow Cytometric Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Pearls and Pitfalls - A Critical Review Article.EJIFCC · 2019Review
- Paroxysmal nocturnal haemoglobinuria.Nature reviews. Disease primers · 2017Review
- Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia.Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation · 2017Article
- Aplastic anemia and clonal evolution: germ line and somatic genetics.Hematology. American Society of Hematology. Education Program · 2016Review
- Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria.Hematology. American Society of Hematology. Education Program · 2016Review
- Review
- [Clinical and laboratory characteristics in patients of myelodysplastic syndrome with PNH clones].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2016Article
- [Clinical characteristics and evolution of paroxysmal nocturnal hemoglobinuria clones in patients with acquired aplastic anemia].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2016Article
- Characteristics of Taiwanese patients of PNH in the international PNH registry.Thrombosis journal · 2016Article
- Morphological Spectrum of Paroxysmal Nocturnal Hemoglobinuria (PNH).Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2015Article
- Paroxysmal nocturnal hemoglobinuria.Blood · 2014Review
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
objectiveTo investigate the natural history of paroxysmal nocturnal hemoglobinuria (PNH) clones in patients with acquired aplastic anemia (AA). PATIENTS AND
methodsTwenty-seven patients with AA and a detectable PNH clone were monitored for a median of 5.7 years (range 1.5-11.5 years). Twenty-two patients received high-dose cyclophosphamide (HiCy) therapy. The erythrocyte and granulocyte PNH clone sizes were measured using flow cytometry and analyzed via CellQuest software. PE-conjugated anti-glycophorin A, anti-CD15, FITC-conjugated anti-CD59, and FLAER staining were used to define glycosylphosphatidylinositol-AP-deficient cells.
resultsWe found a linear relationship between PNH clone size and the development of intravascular hemolysis, assessed by lactate dehydrogenase (LDH) values (Pearson correlation coefficient = 0.80, P < 0.001 for erythrocyte PNH clones; and Pearson correlation coefficient = 0.73, P < 0.0001 for granulocyte PNH clones). An erythrocyte PNH size of 3-5% and granulocyte PNH size of 23% were the thresholds to predict hemolysis as measured by an elevated LDH (receiver operating characteristic analyses with AUC = 0.96 for erythrocyte PNH clone sizes and AUC = 0.88 for granulocyte PNH clone sizes). Patients with small (≤15%) initial PNH clone sizes were less likely to develop an elevated LDH (mean ± SD: 236.9 ± 109.9 vs. 423.1 ± 248.8; P = 0.02). Over time, the PNH clone sizes remained stable in 25.9% of patients; 48.1% experienced a rise in the PNH clone size; and 25.9% experienced a decrease.
conclusionThe risk of developing clinically significant PNH after HiCy therapy appears to be low in AA patients with PNH clones, especially for those with small initial PNH clones and for those who respond to HiCy therapy.
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