Med Bull Sisli Etfal Hosp. 2025; 59(1): 98-105 | DOI: 10.14744/SEMB.2025.24022

Primary Central Nervous System Lymphomas: A Single-Center Experience

Sidika Gulkan Ozkan¹, Seyedehtina Safaei², Ali Kimiaei², Zeynep Asli Durak², Mehmet Serdar Yildiz³, Yuksel Asli Ozturkmen⁴, Hasan Atilla Ozkan^1
1Division of Hematology, Department of Internal Medicine, Bahcesehir University Faculty of Medicine, Istanbul, Türkiye; Adult Hematology and Bone Marrow Transplantation Unit, Medical Park Göztepe Hospital, Istanbul, Türkiye
²Division of Hematology, Department of Internal Medicine, Bahcesehir University Faculty of Medicine, Istanbul, Türkiye
³Department of Internal Medicine, University of Health Sciences Türkiye, Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Türkiye
⁴Division of Hematology, Department of Internal Medicine, Sivas Numune Hospital, Sivas, Türkiye

Objectives: Primary central nervous system lymphoma (PCNSL) is a rare and aggressive form of non-Hodgkin lymphoma (NHL). This study aimed to investigate the characteristics, treatment approaches, and outcomes of patients with PCNSL in a single institution.
Methods: We retrospectively analyzed 11 patients with PCNSL treated at our institution between October 2022 and July 2024. Patient demographics, clinical characteristics, treatment modalities, and outcomes were evaluated.
Results: The median age of the patients was 65 years, with male predominance (63.64%). The median follow-up duration was 10 months. All patients were immunocompetent, and 90.91% had diffuse large B-cell lymphoma. At diagnosis, 81% of the patients were considered fit to receive HDMTX treatment. R-MPV was the most common first-line treatment (45.45%). The complete response rate to initial treatment was 80%. The treatment-related mortality was 9.09%. Autologous stem cell transplantation (ASCT) was performed in 72.73% of the patients, with rituximab-thiotepa-carmustine as the predominant conditioning regimen (62.50%). Treatment-related toxicities occurred in 50% of patients, and 87.50% of patients experienced transplant-related complications. The transplantation-related mortality rate was 25%. The relapse rate was 25% among the patients undergoing ASCT. The mortality rate was 36.36%, and cerebellar involvement was significantly associated with a higher mortality rate (p=0.045).
Conclusion: This study demonstrated the efficacy of methotrexate-based regimens and ASCT in the treatment of PCNSL and achieved high complete response rates. However, the significant incidence of treatment-related toxicities and mortality underscores the persistent challenges of managing this disease. In addition, the association between cerebellar involvement and increased mortality requires further investigation. Larger prospective studies are needed to validate these findings.

Keywords: Non-Hodgkin lymphoma, PCNSL, primary central nervous system lymphoma

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Merkezi Sinir Sistemi Lenfomalarý: Tek Merkez Deneyimi

Sidika Gulkan Ozkan¹, Seyedehtina Safaei², Ali Kimiaei², Zeynep Asli Durak², Mehmet Serdar Yildiz³, Yuksel Asli Ozturkmen⁴, Hasan Atilla Ozkan^1
1Bahçeþehir Üniversitesi Týp Fakültesi, Ýç Hastalýklarý Anabilim Dalý, Hematoloji Bilim Dalý, Ýstanbul; Medical Park Göztepe Hastanesi, Eriþkin Hematoloji ve Kemik Ýliði Transplantasyon Ünitesi, Ýstanbul
²Bahçeþehir Üniversitesi Týp Fakültesi, Ýç Hastalýklarý Anabilim Dalý, Hematoloji Bilim Dalý, Ýstanbul
³Türkiye Saðlýk Bilimleri Üniversitesi, Þiþli Hamidiye Etfal Eðitim ve Araþtýrma Hastanesi, Ýç Hastalýklarý Kliniði, Ýstanbul
⁴Sivas Numune Hastanesi, Ýç Hastalýklarý Kliniði, Hematoloji Bölümü, Sivas

Amaç: Primer merkezi sinir sistemi lenfomasý (PMSSL), nadir ve agresif bir non-Hodgkin lenfoma (NHL) türüdür. Bu çalýþma, tek bir merkezde PMSSL'li hastalarýn özelliklerini, tedavi yaklaþýmlarýný ve sonuçlarýný araþtýrmayý amaçladý.
Yöntemler: Ekim 2022 ile Temmuz 2024 arasýnda kurumumuzda tedavi edilen 11 PMSSL hastasýný retrospektif olarak analiz ettik. Hasta demografisi, klinik özellikler, tedavi yöntemleri ve sonuçlarý deðerlendirildi.
Bulgular: Hastalarýn medyan yaþý 65 olup, erkeklerde baskýnlýk (%63,64) gözlendi. Medyan takip süresi 10 aydý. Tüm hastalar immünokompetan olup, %90,91’i diffüz büyük B hücreli lenfoma (DBBHL) tanýsý almýþtý. Taný anýnda hastalarýn %81’i yüksek doz metotreksat tedavisi almaya uygun olarak deðerlendirildi. Rituksimab, metotreksat, prokarbazin, vinkristin en yaygýn birinci basamak tedavi yöntemiydi (%45,45). Ýlk tedaviye tam yanýt oraný %80 olarak belirlendi. Tedaviye baðlý mortalite oraný %9,09’du. Hastalarýn %72,73’üne otolog hematopoetik kök hücre nakli (OKHN) yapýldý ve en sýk kullanýlan hazýrlýk rejimi rituksimab-tiothepa-karmustin oldu. Tedaviye baðlý toksisiteler hastalarýn %50’sinde, nakil ile iliþkili komplikasyonlar ise %87,50’sinde görüldü. Nakil ile iliþkili mortalite oraný %25 olarak tespit edildi. OKHN uygulanan hastalarda relaps oraný %25’ti. Mortalite oraný %36,36 olup, serebellar tutulumun daha yüksek mortalite oranýyla anlamlý þekilde iliþkili olduðu bulundu (p=0,045).
Sonuç: Bu çalýþma, PMSSL tedavisinde metotreksat bazlý rejimlerin ve OKHN'nin etkinliðini göstermiþ ve yüksek tam yanýt oranlarýna ulaþýlmýþtýr. Ancak, tedaviye baðlý toksisite ve mortalitenin önemli bir insidansý, bu hastalýðýn yönetimindeki zorluklarýn devam ettiðini vurgulamaktadýr. Ayrýca, serebellar tutulum ile artan mortalite arasýndaki iliþki daha fazla araþtýrma gerektirmektedir. Bu bulgularýn doðrulanmasý için daha geniþ çaplý prospektif çalýþmalara ihtiyaç vardýr. (SETB-2024-11-192)

Anahtar Kelimeler: Non-Hodgkin lenfoma (NHL), otolog hematopoetik kök hücre nakli (OKHN), primer merkezi sinir sistemi lenfomasý (PMSSL)

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