Kronik lenfositik lösemi hastalarının 17p genetik durumu, evrelerine göre sağkalım etkisi ve aldıkları tedavilerinin retrospektif incelenmesi
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Tarih
2022
Yazarlar
Dergi Başlığı
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Cilt Başlığı
Yayıncı
Dicle Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Amaç: KLL periferik kan, kemik iliği, lenf nodları ve dalakta neoplastik B lenfositlerin monoklonal proliferasyonu ve birikimi ile karakterize bir hastalıktır. Bu çalışmada kliniğimizde KLL tanısı almış hastaların demografik özelliklerini, hastalık evrelerini, 17p delesyonu durumlarını ve çeşitli faktörlere göre sağkalımlarını incelemeyi ve literatür ile karşılaştırmayı amaçladık. Yöntem: 1 Ocak 2015-1 Ocak 2021 tarihleri arasında Dicle Üniversitesi iç hastalıkları hematoloji kliniğinde KLL tanısı almış 308 hastanın verileri retrospektif olarak incelendi. Çalışmaya alınan hastaların tanı anındaki genel özellikleri, laboratuvar değerleri, hastalık evreleri, 17p delesyonu genetik sonuçları, tedavi seçenekleri ve sağkalım süreleri araştırıldı. Bulgular: Çalışmaya dahil edilen toplam 308 hastanın 190'ı (%61,7) erkek 118'i (%38,3) kadındı. Erkek/kadın oranı 1.6/1, ortalama yaş 62,7 (ortanca yaş 63) ve standart sapma 10,7 olarak hesaplandı. Yaş aralığı 28 ile 86 arasında bulundu. 170 hastada 17p delesyonu çalışılmış olup 26 hastanın sonucu pozitif (%15,3) olarak belirlendi. Hastaların tanı anında Rai evrelemesine göre dağılımı incelendiğinde hastaların %8,4'ü evre 0, %42,2'si evre I, %30,5'i evre II, %12,5'i evre III, %6,5'i evre IV olarak belirlendi. Takip süresi ortanca değeri 68,1 ay (min-max=3,1-186,6 ay) olarak hesaplandı. Takipleri esnasında 9 (%2,9) hastada Richter transformasyonu, 31 (%10,1) hastada OİHA, 6 (%1,9) hastada İTP, 58(%18,8) hastada ise sekonder immün yetmezlik geliştiği gözlendi. Hastaların aldıkları tedaviler incelendiğinde 109(%35,4) hastanın tedavi almadığı, 199(%64,6) hastanın ise tedavi aldığı görüldü. Tedavisiz sağkalım ortanca değeri 21,2 ay (%95CI=16,9-25,4) olarak hesaplanmıştır. Rai evresi arttıkça tedavisiz sağkalım süresinin kısaldığı gözlendi(p<0,001). LDH seviyesi normal olanların tedavisiz sağkalım süresi 29,4 ay (%95CI=20,6-38,3) iken LDH seviyesi yüksek olanların tedavisiz sağkalım süresi 13,9 ay (%95CI=10,5-17,4) olarak gözlendi(p=0,03). 17p delesyonu pozitif olan hastaların tedavisiz sağkalım süresi 10,5 (%95CI=0-21,3) iken negatif olanların tedavisiz sağkalım süresi 21 (%95CI=15,7-26,3) olarak görüldü(p=0,03). Çalışmaya dahil olan 308 hastanın takipleri esnasında 118'i (%38,3) eksitus olmuştur. Hastaların total sağkalım ortanca süresi 117,2 ay (%95CI=104,-129,9) olarak hesaplandı. Hastaların Rai evresi arttıkça total sağkalımın kısaldığı gözlendi(p=0,001). LDH değeri yüksek olan hastaların total sağkalım ortanca değeri 84,5 ay (%95CI=70,2-98,7), normal olanların ise 128,7 (%95CI=114,5-142,8) ay saptandı(p<0,001). 17p delesyonu pozitif olan hastaların total sağkalım ortanca değeri 83,4 ay (%95CI=52,9-114) 17p delesyonu negatif olanların ise 116 ay (%95CI=92,6-139,4) olduğu görüldü(p=0,015). Richter transformasyonu olan hastaların total sağkalım ortanca değeri 90,1 ay (%95CI=20,5-159,4) iken Richter transformasyonu olmayan hastaların total sağkalım ortanca değeri 118,3 ay (%95CI=105,1-131,5) olduğu görüldü(p=0,037). Sonuç: KLL ileri yaş hastalığı olup erkeklerde daha sık görülür. Bizim çalışmamızda literatür ile uyumlu olarak hastalarımızın çoğu ileri yaş hasta olup erkek baskınlığı ön plandaydı. Hastaların 17p delesyonu pozitifliği %15,3 olup, 17p delesyonunun kötü prognostik faktör olduğu gözlendi. Hastaların evreleri arttıkça tedavisiz ve total sağkalımlarının kısaldığı gözlendi.
17p GENETIC STATUS OF CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS, SURVIVAL EFFECTS ACCORDING TO THEIR STAGES AND RETROSPECTIVE INVESTIGATION OF THE TREATMENTS THAT THEY RECEIVED Purpose: CLL is a disease characterized by monoclonal proliferation and accumulation of neoplastic B lymphocytes in peripheral blood, bone marrow, lymph nodes and spleen. In this study, we aimed to investigate the demographic characteristics, disease stages, 17p deletion status and survival according to various factors of patients diagnosed with CLL in our clinic and compare them with the literature. Methods: The data of 308 patients diagnosed with CLL in the internal medicine hematology clinic of Dicle University between January 1, 2015, and January 1, 2021, were analyzed retrospectively. The general characteristics, laboratory values, disease stages, genetic results of 17p deletion, treatment options and survival times of the patients included in the study were investigated. Results: Of the 308 patients included in the study, 190 (61.7%) were male and 118 (38.3%) were female. The male/female ratio was 1.6/1, the mean age was 62.7 (median age 63), and the standard deviation was 10.7. The age range was found to be between 28 and 86 years. 17p deletion was studied in 170 patients, and the result of 26 patients was determined as positive (15.3%). When the distribution of the patients according to Rai staging at the time of diagnosis was examined, 8.4% of the patients were stage 0, 42.2% stage I, 30.5% stage II, 12.5% stage III, 6.5%. I was determined as stage IV. The median value of the follow-up period was calculated as 68.1 months (min-max=3.1-186.6 months). During the follow-up, Richter transformation was observed in 9 (2.9%) patients, AIHA in 31 (10.1%) patients, ITP in 6 (1.9%) patients, and secondary immunodeficiency in 58 (18.8%) patients. It was observed that 109 (35.4%) patients did not receive treatment during their follow-up, and 199 (64.6%) patients received treatment. The median value of treatment-free survival was calculated as 21.2 months (95%CI=16.9-25.4). It was observed that the treatment-free survival time was declined as the Rai stage increased (p<0.001). The treatment-free survival time of those with normal LDH levels was 29.4 months (95%CI=20.6-38.3), while the treatment-free survival time of those with high LDH levels was 13.9 months (95%CI=10.5-17.4) (p=0.03). The treatment-free survival time of patients with positive 17p deletion was 10.5 (95%CI=0-21.3), while those with negative 17p deletion had a 21 (95%CI=15.7-26.3) survival time (p=0.03). During the follow-up of 308 patients included in the study, 118 (38.3%) died. The median value of overall survival time of the patients was calculated as 117.2 months (95%CI=104.-129.9). It was observed that as the Rai stage of the patients increased, the overall survival decreased (p=0.001). The median value of overall survival value of patients with high LDH value was 84.5 months (95%CI=70.2-98.7), and it was 128.7 (95%CI=114.5-142.8) months in patients with normal (p<0.001). The median value of overall survival time of patients with positive 17p deletion was 83.4 months (95%CI=52.9-114) and patients with negative 17p deletion were 116 months (95%CI=92.6-139.4) (p=0.015). The median value of overall survival of patients with Richter transformation was 90.1 months (95.CI=20.5-159.4), while the median total survival of patients without Richter transformation was 118.3 months (95.CI=105.1-131.5). observed (p=0.037). Conclusion: CLL is a disease of advanced age and is more common in men. In our study, in accordance with the literature, most of our patients were elderly patients and male predominance was in the foreground. The 17p deletion positivity of the patients was 15.3%, and 17p deletion was observed to be a poor prognostic factor. It was observed that as the stages of the patients increased, their untreated and overall survivals were decreased.
17p GENETIC STATUS OF CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS, SURVIVAL EFFECTS ACCORDING TO THEIR STAGES AND RETROSPECTIVE INVESTIGATION OF THE TREATMENTS THAT THEY RECEIVED Purpose: CLL is a disease characterized by monoclonal proliferation and accumulation of neoplastic B lymphocytes in peripheral blood, bone marrow, lymph nodes and spleen. In this study, we aimed to investigate the demographic characteristics, disease stages, 17p deletion status and survival according to various factors of patients diagnosed with CLL in our clinic and compare them with the literature. Methods: The data of 308 patients diagnosed with CLL in the internal medicine hematology clinic of Dicle University between January 1, 2015, and January 1, 2021, were analyzed retrospectively. The general characteristics, laboratory values, disease stages, genetic results of 17p deletion, treatment options and survival times of the patients included in the study were investigated. Results: Of the 308 patients included in the study, 190 (61.7%) were male and 118 (38.3%) were female. The male/female ratio was 1.6/1, the mean age was 62.7 (median age 63), and the standard deviation was 10.7. The age range was found to be between 28 and 86 years. 17p deletion was studied in 170 patients, and the result of 26 patients was determined as positive (15.3%). When the distribution of the patients according to Rai staging at the time of diagnosis was examined, 8.4% of the patients were stage 0, 42.2% stage I, 30.5% stage II, 12.5% stage III, 6.5%. I was determined as stage IV. The median value of the follow-up period was calculated as 68.1 months (min-max=3.1-186.6 months). During the follow-up, Richter transformation was observed in 9 (2.9%) patients, AIHA in 31 (10.1%) patients, ITP in 6 (1.9%) patients, and secondary immunodeficiency in 58 (18.8%) patients. It was observed that 109 (35.4%) patients did not receive treatment during their follow-up, and 199 (64.6%) patients received treatment. The median value of treatment-free survival was calculated as 21.2 months (95%CI=16.9-25.4). It was observed that the treatment-free survival time was declined as the Rai stage increased (p<0.001). The treatment-free survival time of those with normal LDH levels was 29.4 months (95%CI=20.6-38.3), while the treatment-free survival time of those with high LDH levels was 13.9 months (95%CI=10.5-17.4) (p=0.03). The treatment-free survival time of patients with positive 17p deletion was 10.5 (95%CI=0-21.3), while those with negative 17p deletion had a 21 (95%CI=15.7-26.3) survival time (p=0.03). During the follow-up of 308 patients included in the study, 118 (38.3%) died. The median value of overall survival time of the patients was calculated as 117.2 months (95%CI=104.-129.9). It was observed that as the Rai stage of the patients increased, the overall survival decreased (p=0.001). The median value of overall survival value of patients with high LDH value was 84.5 months (95%CI=70.2-98.7), and it was 128.7 (95%CI=114.5-142.8) months in patients with normal (p<0.001). The median value of overall survival time of patients with positive 17p deletion was 83.4 months (95%CI=52.9-114) and patients with negative 17p deletion were 116 months (95%CI=92.6-139.4) (p=0.015). The median value of overall survival of patients with Richter transformation was 90.1 months (95.CI=20.5-159.4), while the median total survival of patients without Richter transformation was 118.3 months (95.CI=105.1-131.5). observed (p=0.037). Conclusion: CLL is a disease of advanced age and is more common in men. In our study, in accordance with the literature, most of our patients were elderly patients and male predominance was in the foreground. The 17p deletion positivity of the patients was 15.3%, and 17p deletion was observed to be a poor prognostic factor. It was observed that as the stages of the patients increased, their untreated and overall survivals were decreased.
Açıklama
Anahtar Kelimeler
Kronik lenfositer lösemi, KLL, Küçük lenfositik lösemi, SLL, Chronic lymphocytic leukemia, CLL, Small iymphocytic iymphoma
Kaynak
WoS Q Değeri
Scopus Q Değeri
Cilt
Sayı
Künye
Dilek, M. S. (2022). Kronik lenfositik lösemi hastalarının 17p genetik durumu, evrelerine göre sağkalım etkisi ve aldıkları tedavilerinin retrospektif incelenmesi. Yayımlanmamış uzmanlık tezi, Dicle Üniversitesi, Diyarbakır.
