Skip to main content

Main menu

  • Home
  • Content
    • Latest
    • Archive
    • home
  • Info for
    • Authors
    • Reviewers
    • Subscribers
    • Institutions
    • Advertisers
    • Join SMJ
  • About Us
    • About Us
    • Editorial Office
    • Editorial Board
  • More
    • Advertising
    • Alerts
    • Feedback
    • Folders
    • Help
  • Other Publications
    • NeuroSciences Journal

User menu

  • My alerts
  • Log in

Search

  • Advanced search
Saudi Medical Journal
  • Other Publications
    • NeuroSciences Journal
  • My alerts
  • Log in
Saudi Medical Journal

Advanced Search

  • Home
  • Content
    • Latest
    • Archive
    • home
  • Info for
    • Authors
    • Reviewers
    • Subscribers
    • Institutions
    • Advertisers
    • Join SMJ
  • About Us
    • About Us
    • Editorial Office
    • Editorial Board
  • More
    • Advertising
    • Alerts
    • Feedback
    • Folders
    • Help
  • Follow psmmc on Twitter
  • Visit psmmc on Facebook
  • RSS
Research ArticleOriginal Article
Open Access

The immunologic abnormalities in patients with paroxysmal nocturnal hemoglobinuria are associated with disease progression

Guanrou Wang, Mengting Che, Lijie Zeng, Hui Liu, Liyan Li, Zhaoyun Liu and Rong Fu
Saudi Medical Journal April 2024, 45 (4) 424-432; DOI: https://doi.org/10.15537/smj.2024.45.4.20231010
Guanrou Wang
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mengting Che
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Lijie Zeng
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hui Liu
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Liyan Li
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Zhaoyun Liu
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Rong Fu
From the Department of Hematology, Tianjin Medical University General Hospital, Tianjin, People’s Republic of China.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Rong Fu
  • Article
  • Figures & Data
  • eLetters
  • Info & Metrics
  • References
  • PDF
Loading

References

  1. 1.↵
    1. Hosokawa K,
    2. Nakao S.
    Somatic mutations and clonal expansions in paroxysmal nocturnal hemoglobinuria. Semin Hematol 2022; 59: 143-149.
    OpenUrl
  2. 2.↵
    1. Mortazavi Y,
    2. Tooze JA,
    3. Gordon-Smith EC,
    4. Rutherford TR.
    N-RAS gene mutation in patients with aplastic anemia and aplastic anemia/paroxysmal nocturnal hemoglobinuria during evolution to clonal disease. Blood 2000; 95: 646-650.
    OpenUrlAbstract/FREE Full Text
  3. 3.↵
    1. Hu R,
    2. Mukhina GL,
    3. Piantadosi S,
    4. Barber JP,
    5. Jones RJ,
    6. Brodsky RA.
    PIG-a mutations in normal hematopoiesis. Blood 2005; 105: 3848-3854.
    OpenUrlAbstract/FREE Full Text
  4. 4.↵
    1. Araten DJ,
    2. Nafa K,
    3. Pakdeesuwan K,
    4. Luzzatto L.
    Clonal populations of hematopoietic cells with paroxysmal nocturnal hemoglobinuria genotype and phenotype are present in normal individuals. Proc Natl Acad Sci U S A 1999; 96: 5209-5214.
    OpenUrlAbstract/FREE Full Text
  5. 5.↵
    1. Kawagoe K,
    2. Kitamura D,
    3. Okabe M,
    4. Taniuchi I,
    5. Ikawa M,
    6. Watanabe T, et al.
    Glycosylphosphatidylinositol-anchor-deficient mice: implications for clonal dominance of mutant cells in paroxysmal nocturnal hemoglobinuria. Blood 1996; 87: 3600-3606.
    OpenUrlAbstract/FREE Full Text
  6. 6.
    1. Murakami Y,
    2. Kinoshita T,
    3. Maeda Y,
    4. Nakano T,
    5. Kosaka H,
    6. Takeda J.
    Different roles of glycosylphosphatidylinositol in various hematopoietic cells as revealed by a mouse model of paroxysmal nocturnal hemoglobinuria. Blood 1999; 94: 2963-2970.
    OpenUrlAbstract/FREE Full Text
  7. 7.↵
    1. Tremml G,
    2. Dominguez C,
    3. Rosti V,
    4. Zhang Z,
    5. Pandolfi PP,
    6. Keller P, et al.
    Increased sensitivity to complement and a decreased red blood cell life span in mice mosaic for a nonfunctional Piga gene. Blood 1999; 94: 2945-2954.
    OpenUrlAbstract/FREE Full Text
  8. 8.↵
    1. Luzzatto L,
    2. Notaro R.
    The “escape” model: a versatile mechanism for clonal expansion. Br J Haematol 2019; 184: 465-466.
    OpenUrl
  9. 9.
    1. Wang H,
    2. Chuhjo T,
    3. Yasue S,
    4. Omine M,
    5. Nakao S.
    Clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria-type cells in bone marrow failure syndrome. Blood 2002; 100: 3897-3902.
    OpenUrlAbstract/FREE Full Text
  10. 10.
    1. Yoshizato T,
    2. Dumitriu B,
    3. Hosokawa K,
    4. Makishima H,
    5. Yoshida K,
    6. Townsley D, et al.
    Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia. N Engl J Med 2015; 373: 35-47.
    OpenUrlCrossRefPubMed
  11. 11.↵
    1. Kulagin A,
    2. Lisukov I,
    3. Ivanova M,
    4. Golubovskaya I,
    5. Kruchkova I,
    6. Bondarenko S, et al.
    Prognostic value of paroxysmal nocturnal haemoglobinuria clone presence in aplastic anaemia patients treated with combined immunosuppression: results of 2-centre prospective study. Br J Haematol 2014; 164: 546-554.
    OpenUrlCrossRefPubMed
  12. 12.↵
    1. van Bijnen ST,
    2. Withaar M,
    3. Preijers F,
    4. van der Meer A,
    5. de Witte T, et al.
    T-cells expressing the activating NK-cell receptors KIR2DS4, NKG2C and NKG2D are elevated in paroxysmal nocturnal hemoglobinuria and cytotoxic toward hematopoietic progenitor cell lines. Exp Hematol 2011; 39: 751-762.
    OpenUrl
  13. 13.↵
    1. Kunyaboon R,
    2. Wanachiwanawin W
    , U-Pratya Y, Thedsawad A, Taka O. Mechanism of paroxysmal nocturnal hemoglobinuria clonal dominance: possible roles of different apoptosis and CD8+ lymphocytes in the selection of paroxysmal nocturnal hemoglobinuria clones. Hematol Oncol Stem Cell Ther 2012; 5: 138-145.
    OpenUrlPubMed
  14. 14.↵
    1. Gargiulo L,
    2. Papaioannou M,
    3. Sica M,
    4. Talini G,
    5. Chaidos A,
    6. Richichi B, et al.
    Glycosylphosphatidylinositol-specific, CD1d-restricted T-cells in paroxysmal nocturnal hemoglobinuria. Blood 2013; 121: 2753-2761.
    OpenUrlAbstract/FREE Full Text
  15. 15.
    1. Espinoza JL,
    2. Elbadry MI,
    3. Chonabayashi K,
    4. Yoshida Y,
    5. Katagiri T,
    6. Harada K, et al.
    Hematopoiesis by iPSC-derived hematopoietic stem cells of aplastic anemia that escape cytotoxic T-cell attack. Blood Adv 2018; 2: 390-400.
    OpenUrlAbstract/FREE Full Text
  16. 16.↵
    1. Hosokawa K,
    2. Kajigaya S,
    3. Keyvanfar K,
    4. Qiao W,
    5. Xie Y,
    6. Townsley DM, et al.
    T-cell transcriptomes from paroxysmal nocturnal hemoglobinuria patients reveal novel signaling pathways. J Immunol 2017; 199: 477-488.
    OpenUrlAbstract/FREE Full Text
  17. 17.↵
    1. Rachidi S,
    2. Musallam KM,
    3. Taher AT.
    A closer look at paroxysmal nocturnal hemoglobinuria. Eur J Intern Med 2010; 21: 260-267.
    OpenUrlCrossRefPubMed
  18. 18.↵
    1. Murakami Y,
    2. Kosaka H,
    3. Maeda Y,
    4. Nishimura J,
    5. Inoue N,
    6. Ohishi K, et al.
    Inefficient response of T-lymphocytes to glycosylphosphatidylinositol anchor-negative cells: implications for paroxysmal nocturnal hemoglobinuria. Blood 2002; 100: 4116-4122.
    OpenUrlAbstract/FREE Full Text
  19. 19.↵
    1. Young NS,
    2. Calado RT,
    3. Scheinberg P.
    Current concepts in the pathophysiology and treatment of aplastic anemia. Blood 2006; 108: 2509-2519.
    OpenUrlAbstract/FREE Full Text
  20. 20.↵
    1. Zaimoku Y,
    2. Patel BA,
    3. Kajigaya S,
    4. Feng X,
    5. Alemu L,
    6. Quinones Raffo D, et al.
    Deficit of circulating CD19+ CD24hi CD38hi regulatory B-cells in severe aplastic anaemia. Br J Haematol 2020; 190: 610-617.
    OpenUrl
  21. 21.↵
    1. Risitano AM,
    2. Kook H,
    3. Zeng W,
    4. Chen G,
    5. Young NS,
    6. Maciejewski JP.
    Oligoclonal and polyclonal CD4 and CD8 lymphocytes in aplastic anemia and paroxysmal nocturnal hemoglobinuria measured by V beta CDR3 spectratyping and flow cytometry. Blood 2002; 100: 178-183.
    OpenUrlAbstract/FREE Full Text
  22. 22.↵
    1. Sloand E,
    2. Kim S,
    3. Maciejewski JP,
    4. Tisdale J,
    5. Follmann D,
    6. Young NS.
    Intracellular interferon-gamma in circulating and marrow T-cells detected by flow cytometry and the response to immunosuppressive therapy in patients with aplastic anemia. Blood 2002; 100: 1185-1191.
    OpenUrlAbstract/FREE Full Text
  23. 23.↵
    1. Colden MA,
    2. Kumar S,
    3. Munkhbileg B,
    4. Babushok DV.
    Insights into the emergence of paroxysmal nocturnal hemoglobinuria. Front Immunol 2022; 12: 830172.
    OpenUrl
  24. 24.↵
    1. Keller B,
    2. Strohmeier V,
    3. Harder I,
    4. Unger S,
    5. Payne KJ,
    6. Andrieux G, et al.
    The expansion of human T-bethighCD21low B-cells is T-cell dependent. Sci Immunol 2021; 6: eabh0891.
    OpenUrlCrossRef
  25. 25.↵
    1. Karnell JL,
    2. Kumar V,
    3. Wang J,
    4. Wang S,
    5. Voynova E,
    6. Ettinger R.
    Role of CD11c(+) T-bet(+) B cells in human health and disease. Cell Immunol 2017; 321:40-45.
    OpenUrl
  26. 26.↵
    1. Wilbrink R,
    2. Spoorenberg A,
    3. Arends S,
    4. van der Geest K,
    5. Brouwer E,
    6. Bootsma H, et al.
    CD27 (-) CD38 (low) CD21 (low) B-cells are increased in axial spondyloarthritis. Front Immunol 2021; 12: 686273.
    OpenUrl
  27. 27.
    1. Thorarinsdottir K,
    2. Camponeschi A,
    3. Jonsson C,
    4. Granhagen Önnheim K,
    5. Nilsson J,
    6. Forslind K, et al.
    CD21-/low B-cells associate with joint damage in rheumatoid arthritis patients. Scand J Immunol 2019; 90: e12792.
    OpenUrlCrossRef
  28. 28.↵
    1. Marrapodi R,
    2. Pellicano C,
    3. Radicchio G,
    4. Leodori G,
    5. Colantuono S,
    6. Iacolare A, et al.
    CD21low B-cells in systemic sclerosis: a possible marker of vascular complications. Clin Immunol 2020; 213: 108364.
    OpenUrl
  29. 29.↵
    1. Palm AE,
    2. Henry C.
    Remembrance of things past: long-term B-cell memory after infection and vaccination. Front Immunol 2019; 10: 1787.
    OpenUrlPubMed
  30. 30.↵
    1. Akkaya M,
    2. Kwak K,
    3. Pierce SK.
    B-cell memory: building 2 walls of protection against pathogens. Nat Rev Immunol 2020; 20: 229-238.
    OpenUrlCrossRefPubMed
  31. 31.↵
    1. Cerutti A,
    2. Cols M,
    3. Puga I.
    Marginal zone B-cells: virtues of innate-like antibody-producing lymphocytes. Nat Rev Immunol 2013; 13: 118-132.
    OpenUrlCrossRefPubMed
  32. 32.↵
    1. Mauri C,
    2. Menon M.
    Human regulatory B-cells in health and disease: therapeutic potential. J Clin Invest 2017; 127: 772-779.
    OpenUrlCrossRefPubMed
  33. 33.↵
    1. Wang L,
    2. Liu H.
    Pathogenesis of aplastic anemia. Hematology 2019; 24: 559-566.
    OpenUrl
  34. 34.↵
    1. Steinbrink K,
    2. Mahnke K,
    3. Grabbe S,
    4. Enk AH,
    5. Jonuleit H.
    Myeloid dendritic cell: from sentinel of immunity to key player of peripheral tolerance? Hum Immunol 2009; 70: 289-293.
    OpenUrlCrossRefPubMed
  35. 35.↵
    1. Kwan WH,
    2. van der Touw W,
    3. Heeger PS.
    Complement regulation of T-cell immunity. Immunol Res 2012; 54: 247-253.
    OpenUrlCrossRefPubMed
  36. 36.↵
    1. Brembilla NC,
    2. Senra L,
    3. Boehncke WH.
    The IL-17 family of cytokines in psoriasis: IL-17A and beyond. Front Immunol 2018; 9: 1682.
    OpenUrlCrossRefPubMed
  37. 37.↵
    1. von Stebut E,
    2. Boehncke WH,
    3. Ghoreschi K,
    4. Gori T,
    5. Kaya Z,
    6. Thaci D, et al.
    IL-17A in psoriasis and beyond: cardiovascular and metabolic implications. Front Immunol 2020; 10: 3096.
    OpenUrlCrossRefPubMed
  38. 38.↵
    1. Giudice V,
    2. Cardamone C,
    3. Triggiani M,
    4. Selleri C.
    Bone Marrow Failure Syndromes, Overlapping Diseases with a Common Cytokine Signature. Int J Mol Sci 2021; 22(2):705.
    OpenUrl
  39. 39.↵
    1. Vaht K,
    2. Brenner J,
    3. Ednersson SB,
    4. Ljungman P,
    5. Brune M,
    6. Andersson PO.
    Bone marrow expression of CD68/CD163 macrophages, IL-17 and FOXP3 cells in aplastic anemia and their relation to prognosis. Eur J Haematol 2023; 110: 313-321.
    OpenUrl
  40. 40.↵
    1. de Latour RP,
    2. Visconte V,
    3. Takaku T,
    4. Wu C,
    5. Erie AJ,
    6. Sarcon AK, et al.
    Th17 immune responses contribute to the pathophysiology of aplastic anemia. Blood 2010; 116: 4175-4184.
    OpenUrlAbstract/FREE Full Text
PreviousNext
Back to top

In this issue

Saudi Medical Journal: 45 (4)
Saudi Medical Journal
Vol. 45, Issue 4
1 Apr 2024
  • Table of Contents
  • Cover (PDF)
  • Index by author
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on Saudi Medical Journal.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
The immunologic abnormalities in patients with paroxysmal nocturnal hemoglobinuria are associated with disease progression
(Your Name) has sent you a message from Saudi Medical Journal
(Your Name) thought you would like to see the Saudi Medical Journal web site.
Citation Tools
The immunologic abnormalities in patients with paroxysmal nocturnal hemoglobinuria are associated with disease progression
Guanrou Wang, Mengting Che, Lijie Zeng, Hui Liu, Liyan Li, Zhaoyun Liu, Rong Fu
Saudi Medical Journal Apr 2024, 45 (4) 424-432; DOI: 10.15537/smj.2024.45.4.20231010

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
The immunologic abnormalities in patients with paroxysmal nocturnal hemoglobinuria are associated with disease progression
Guanrou Wang, Mengting Che, Lijie Zeng, Hui Liu, Liyan Li, Zhaoyun Liu, Rong Fu
Saudi Medical Journal Apr 2024, 45 (4) 424-432; DOI: 10.15537/smj.2024.45.4.20231010
Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Bookmark this article

Jump to section

  • Article
    • ABSTRACT
    • Methods
    • Results
    • Discussion
    • Acknowledgment
    • Footnotes
    • References
  • Figures & Data
  • eLetters
  • References
  • Info & Metrics
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • Exploring communication challenges with children and parents among pharmacists in Saudi Arabia
  • Exploring hypothyroidism’s effects on lipid profiles
  • Assessment of asthma control levels in a tertiary hospital
Show more Original Article

Similar Articles

Keywords

  • paroxysmal nocturnal hemoglobinuria
  • T-cells
  • B-cells
  • dendritic cells
  • cytokines

CONTENT

  • home

JOURNAL

  • home

AUTHORS

  • home
Saudi Medical Journal

© 2025 Saudi Medical Journal Saudi Medical Journal is copyright under the Berne Convention and the International Copyright Convention.  Saudi Medical Journal is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work. Electronic ISSN 1658-3175. Print ISSN 0379-5284.

Powered by HighWire