Clinical and Laboratory Features of Patients with Marginal Zone Lymphoma: A Single Center Experience

Öz

Marginal zone lymphoma (MZL) is a slowly progressing subtype of Non-Hodgkin Lymphoma (NHL). There are four subtypes: extranodal MZL of mucosa-associated lymphoid tissue (MALT lymphoma), nodal MZL, splenic MZL, and primary cutaneous MZL. Due to its heterogeneity and rarity, treatment management in MZL has not been clearly defined. In this study, clinical features and survival data of 36 patients with MZL were examined. The mean total follow-up period of the patients was 64.5 months. Follow-up periods ranged from 6 to 240 months. Thirty-two (88.8%) of the patients were alive. The median OS was 54 months. There were 4 patients (11.1%) who died and 22 (61%) patients who had relapsed. The median PFS was 45 months. Sixteen (44.5%) patients received CHOP or R-CHOP, 13 (36.1%) patients received weekly rituximab, 3 (8.3%) patients received rituximab-bendamustine. When treatment responses were evaluated, complete response was found in 22 (61.1%) patients, stable disease in 9 (25%) and partial response in 5 (13.9%). No progressive disease was observed in the response evaluation performed at the end of the first-line treatment. In our study, the effects of variables such as age, gender, stage, MALT-IPI score, treatment response, lactate dehydrogenase (LDH), β2-microglobulin, albumin, bone marrow infiltration at the time of diagnosis, liver involvement and non-hematopoietic area involvement on survival were examined. No significant effects were found (p>0.05). MALT-IPI score (p=0.029) and β2-microglobulin elevation (p=0.041) were found to be risk factors with adverse effects on PFS according to univariate survival analysis. According to the results of multivariate analysis, no statistically significant effects on survival were found (p>0.05). MALT-IPI score can be used to predict disease prognosis, but according to the results obtained from our study, adding β2-microglobulin to this score may be considered. Evaluating MZL subtypes separately will reduce the difficulty in interpreting results due to patient heterogeneity.

Anahtar Kelimeler

• marginal zone lymphoma
• non-Hodgkin lymphoma
• progression free survival

Marjinal Zon Lenfomalı Hastaların Klinik ve Laboratuar Özellikleri: Tek Merkez Deneyimi

Öz

Marjinal zon lenfoma (MZL), Non-Hodgkin Lenfoma (NHL)’nın yavaş seyirli bir alt tipidir. Mukoza ilişkili lenfoid dokunun ekstranodal MZL’sı (MALT lenfoma), nodal MZL, splenik MZL, primer kutanöz MZL olmak üzere dört alt tipi vardır. Heterojenitesi ve nadirliği sebebiyle MZL’da tedavi yönetimi net olarak tanımlanamamıştır. Bu çalışmada MZL tanılı 36 hastanın klinik özellikleri ve sağkalım verileri incelenmiştir. Hastaların toplam takip süresi ortalama 64.5 aydı. Takip süreleri 6-240 ay arasındaydı. Hastaların 32’si (%88.8) hayattaydı. Ortanca OS 54 aydı. Ölen 4 hasta (%11.1), relaps olan 22 (%61) hasta vardı. Ortanca PFS 45 aydı. On altı (%44.5) hasta CHOP veya R-CHOP, 13 (%36.1) hasta haftalık rituksimab, 3 (%8.3) hasta rituksimab-bendamustin tedavileri almıştı. Tedavi yanıtları değerlendirildiğinde 22 (%61.1) hastada tam yanıt, 9’unda (%25) stabil hastalık, 5’inde (%13.9) kısmi yanıt saptandı. İlk sıra tedavi bitiminde yapılan yanıt değerlendirilmesinde progresif hastalık görülmedi. Çalışmamızda yaş, cinsiyet, evre, MALT-IPI skoru, tedavi yanıtı, laktat dehidrogenaz (LDH), β2-mikroglobulin, albümin, tanı anında kemik iliği infiltrasyonu, karaciğer tutulumu ve hematopoetik olmayan alan tutulumu gibi değişkenlerin sağkalım üzerine etkileri incelendi ancak OS üzerine istatistiksel olarak anlamlı etkileri saptanmadı (p>0.05). MALT-IPI skoru (p=0.029) ve β2-mikroglobulin yüksekliğinin (p=0.041) tek değişkenli sağkalım analizine göre PFS üzerine olumsuz etkileri olan birer risk faktörü oldukları görüldü. Çoklu değişkenli analiz sonuçlarına göre ise sağkalım üzerine istatistiksel olarak anlamlı etkileri saptanmadı (p>0.05). MALT-IPI skoru hastalık prognozunu öngörmede kullanılabilir ancak çalışmamızdan elde edilen sonuçlara göre bu skora β2-mikroglobulin eklenmesi düşünülebilir. MZL alt tiplerinin ayrı ayrı değerlendirilmesi hasta heterojenitesine bağlı sonuçların yorumlanmasındaki güçlüğü azaltacaktır.

Anahtar Kelimeler

• marjinal zon lenfoma
• non hodgkin lenfoma
• progresyonsuz sağkalım

Kaynakça

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