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Dergiler / 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

Mesut AYER
Merve ÖZTÜRK ÇİLOĞLU
Fuat ŞAR
HAYRİYE ESRA ATAOĞLU
Fatma Aylin AYER
Tayfun ELİBOL
HAKKI ONUR KIRKIZLAR
Sayfa
175–180
DOI
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Yapay Zeka Hızlı Bakış & Çıkarımlar

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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

Further studies on a more extensive cohort are required to better understand the pathophysiological and clinical relationships between MDS and PNH.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Aim: Retrospective, cross-sectional, observational study to examine the frequency and features of paroxysmal nocturnal hemoglobinuria (PNH) clones in patients with myelodysplastic syndrome (MDS).
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Temel Bulgular & Veri:Methods: Data were analyzed from the medical files of 41 MDS patients diagnosed and followed up in the hematology department at a referral center between 2006 and 2017.
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Sonuç & Katkı:Further studies on a more extensive cohort are required to better understand the pathophysiological and clinical relationships between MDS and PNH.
🔬Tür:Araştırma Makalesi
📍Kapsam:2006 Verileri
⚙️Değişkenler:Alan Parametreleri

Özet

Aim: Retrospective, cross-sectional, observational study to examine the 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 MDS patients diagnosed and followed up in the hematology department at a referral center between 2006 and 2017. Descriptive data, cytogenetic and hematologic characteristics, prognostic features and PNH clone sizes were assessed. PNH clone sizes were evaluated using the fluorescently labeled inactive toxin aerolysin method. Results: The study population comprised 22 (53.7%) female and 19 (46.3%) male patients with confirmed MDS; the mean age of the patients was 68.20±9.84 years (range, 45-85). PNH clones were detected in eight (19.5%) patients. The number of patients with a PNH clone size of >10%, >1%, >0.1% and >0.01% was one, one, one and eigth, respectively (p<0.001 for all subgroups). Conclusion: These data indicate that PNH clones exist in approximately one-fifth of MDS patients. Further studies on a more extensive cohort are required to better understand the pathophysiological and clinical relationships between MDS and PNH.

Abstract

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<0,001 tüm alt gruplarda) saptandı. Sonuç: MDS hasta grubumuzun yaklaşık 1/5’inde PNH klonu saptanmıştır. MDS ve PNH arasındaki klinik ve patofizyolojik ilişkinin daha iyi anlaşılması için geniş olgu serilerinde yapılacak çalışmalara ihtiyaç vardır. Tayfun Elibol**, Onur Hakkı Kırkızlar***

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Bu Makaleye Atıf Yap

AYER, M., ÇİLOĞLU, M. Ö., ŞAR, F., ATAOĞLU, H. E., AYER, F. A., ELİBOL, T., KIRKIZLAR, H. O. (2018). Frequency of Paroxysmal Nocturnal Hemoglobinuria Clone in Turkish Myelodysplastic Syndrome Group. Haseki Tıp Bülteni, Cilt: 56 - Sayı: 3, 175–180. https://ajindex.com/articles/199655

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Kaynakça (14)

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