SavedAdd your journal

Journals / Haseki Tıp Bülteni / 2018 / Cilt: 56 - Sayı: 3

Frequency of Paroxysmal Nocturnal Hemoglobinuria Clone in Turkish Myelodysplastic Syndrome Group

Türk Myelodisplastik Sendrom Hasta Grubunda Paroksismal Noktürnal Hemoglobinüri Klonu Sıklığı

Esra Hayriye ATAOĞLU
Onur Hakkı KIRKIZLAR
Mesut AYER
Fuat ŞAR
Fatma Aylin AYER
Tayfun ELİBOL
Merve ÖZTÜRK ÇİLOĞLU
Pages
175–180
Publication Date
2022-10-17
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

The number of patients with a PNHclone size of >10%, >1%, >0.1% and >0.01% was one, one, one andeigth, respectively (p

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Aim: Retrospective, cross-sectional, observational study to examinethe frequency and features of paroxysmal nocturnal hemoglobinuria(PNH) clones in patients with myelodysplastic syndrome (MDS).Methods: Data were analyzed from the medical files of 41 MDSpatients diagnosed and followed up in the hematology departmentat a referral center between 2006 and 2017.
📊
Key Findings & Data:PNH clone sizes were evaluated usingthe fluorescently labeled inactive toxin aerolysin method.Results: The study population comprised 22 (53.7%) female and19 (46.3%) male patients with confirmed MDS; the mean age ofthe patients was 68.20±9.84 years (range, 45-85).
💡
Conclusion & Impact:The number of patients with a PNHclone size of >10%, >1%, >0.1% and >0.01% was one, one, one andeigth, respectively (p
🔬Design:Research Article
📍Scope:2006 Verileri
⚙️Variables:Domain Parameters

Abstract

Aim: Retrospective, cross-sectional, observational study to examinethe frequency and features of paroxysmal nocturnal hemoglobinuria(PNH) clones in patients with myelodysplastic syndrome (MDS).Methods: Data were analyzed from the medical files of 41 MDSpatients diagnosed and followed up in the hematology departmentat a referral center between 2006 and 2017. Descriptive data,cytogenetic and hematologic characteristics, prognostic features andPNH clone sizes were assessed. PNH clone sizes were evaluated usingthe fluorescently labeled inactive toxin aerolysin method.Results: The study population comprised 22 (53.7%) female and19 (46.3%) male patients with confirmed MDS; the mean age ofthe patients was 68.20±9.84 years (range, 45-85). PNH clones weredetected in eight (19.5%) patients. The number of patients with a PNHclone size of >10%, >1%, >0.1% and >0.01% was one, one, one andeigth, respectively (p

Özet

Amaç: Türk miyelodisplastik sendrom (MDS) hasta grubunda retrospektif, kesitsel, gözlemsel çalışma ile paroksismal noktürnal hemoglobinüri (PNH) klon sıklığı ve özellikleri araştırılmıştır. Yöntemler: Hematoloji bölümümüzde 2006-2017 yılları arasında takip edilen 41 MDS hastasının verileri analiz edildi. Sitogenetik, hematolojik ve prognostik ve karakteristik özellikler, PNH klon varlığı değerlendirildi. PNH klonu taraması için floresan ile işaretlenmiş inaktif toksin aerolizin metodu kullanıldı. Bulgular: Çalışma grubu teyit edilmiş MDS tanılı 22 (%53,7) kadın ve 19 (%46,3) erkek hastadan oluşmaktaydı, ortalama yaş 68,20±9,84 (aralık, 45-85) idi. Sekiz hastada (%19,5) PNH klonu saptandı. PNH klon genişliği >%10, >%1, >%0,1, >%0,01 olarak sırasıyla; bir, bir, bir ve sekiz hastada (p

🔖

Cite This Article

ATAOĞLU, E. H., KIRKIZLAR, O. H., AYER, M., ŞAR, F., AYER, F. A., ELİBOL, T., ÇİLOĞLU, M. Ö. (2022). Frequency of Paroxysmal Nocturnal Hemoglobinuria Clone in Turkish Myelodysplastic Syndrome Group. Haseki Tıp Bülteni, Cilt: 56 - Sayı: 3, 175–180. https://ajindex.com/articles/535110

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (14)

  1. Rollison DE, Howlader N, Smith MT, et al. Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programs. Blood 2008;112:45-52.
  2. Sahin F, Akay OM, Ayer M, et al. Pesg PNH diagnosis, followup and treatment guidelines. Am J Blood Res 2016;6:19-27.
  3. Rosse WF, Ware RE. The molecular basis of paroxysmal nocturnal hemoglobinuria. Blood 1995;86:3277-86. Brodsky RA. How I treat paroxysmal nocturnal hemoglobinuria. Blood 2009;113:6522-7. Young NS. Paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes: clonal expansion of PIG-A-mutant hematopoietic cells in bone marrow failure. Haematologica 2009;94:3-7.
  4. Young NS, Meyers G, Schrezenmeier H, Hillmen P, Hill A. The management of paroxysmal nocturnal hemoglobinuria: recent advances in diagnosis and treatment and new hope for patients. Semin Hematol 2009;46(Suppl 1):1-16.
  5. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med 1995;333:1253-8.
  6. Nakakuma H, Nagakura S, Iwamoto N, et al. Paroxysmal nocturnal hemoglobinuria clone in bone marrow of patients with pancytopenia. Blood 1995;85:1371-6.
  7. Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005;106:3699-709.
  8. Hillmen P, Muus P, Dührsen U, et al. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria. Blood 2007;110:4123-8.
  9. Iwanaga M, Furukawa K, Amenomori T, et al. Paroxysmal nocturnal haemoglobinuria clones in patients with myelodysplastic syndromes. Br J Haematol 1998;102:465-74.
  10. Greenberg P, Cox C, LeBeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997;89:2079-88.
  11. Oken MM, Creech RH, Tormey DC, et al. Toxicity and response criteria of the Eastern Cooperative Oncology Group. Am J Clin Oncol 1982;5:649-55.
  12. Horikawa K, Nakakuma H, Kawaguchi T, et al. Apoptosis resistance of blood cells from patients with paroxysmal nocturnal hemoglobinuria, aplastic anemia, and myelodysplastic syndrome. Blood 1997;90:2716-22.
  13. Hill A, Richards SJ, Hillmen P. Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuria. Br J Haematol 2007;137:181-92.
  14. Hall C, Richards S, Hillmen P. Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria (PNH). Blood 2003;102:3587-91. Ny