Single Center Experience in Patients with a Diagnosis of Nodular Lymphocyte Predominant Hodgkin Lymphoma

Abstract

Nodular lymphocyte predominant Hodgkin lymphoma is an infrequent disease with a favorable prognosis. NLPHL accounts for 5% of all HL cases. The major problems encountered in NLPHL are treatment side effects, recurrent disease or transformation into NHL. Patients with early-stage NLPHL are prefered active monitoring, surgery, radiotherapy to the part of the body affected by lymphoma, single-agent rituximab whereas individuals with advanced NLPHL are treated with chemotherapy/radiotherapy may be added. Even relapsed NLPHL responds quite well to treatment. It is important to repeat the biopsy at recurrence because of the high risk of transformation of NLPHL. Although transformed disease is mostly related to primary disease clonaly, survival of patients in which autologous stem cell transplant is done following salvage therapy, is similar to those with diffuse large B-cell lymphoma (DLBCL). All data on treatment come from retrospective studies. Ten patients diagnosed with NLPHL in our clinic between December 2011 and December 2020 were evaluated in our study. Median age was found 36 years. Patients with a diagnosis of NLPHL comprised 2.08% of patients with a diagnosis of HL. Eight of patients (80%) were male. At the time of diagnosis, all patients had ECOG (Eastern Cooperative Oncology Group) score of 0 and seven of the patients (70%) were at early stage. All patients who applied to the hospital, complained about palpable lymphadenopathy at first. Hemograms and biochemical parameters were in reference intervals at the time of diagnosis. Immunohistochemical staining showed that all patient’s specimen were positive for CD20, but just one of them (10%) positive for CD30. Nine patiens (90%) were administered ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) chemotheraphy as first-line theraphy. On the other hand one patient (10%) received coadministration of RT with ABVD (CMT). One of our patients due to disease progression and the other patient due to late recurrent disease, recieved salvage chemotherapy regimen. After chemotherapy, patients had autologous stem cell transplant. NLPHL patients diagnosed in our clinic are not competible with incidence rates and first-line treatment option given in the published foreign literature. It would be appropriate to compare and discuss the difference with other data reported in Turkey.

Keywords

• Nodular lymphocyte predominant Hodgkin lymphoma
• Classical Hodgkin lymphoma
• Prognosis

Nodüler Lenfosit Predominant Hodgkin Lenfoma Tanılı Hastalarda Tek Merkez Deneyimi

Abstract

Nodüler lenfosit predominant Hodgkin lenfoma (NLPHL) az görülen ve prognozu oldukça iyi olan bir hastalıktır. NLPHL tüm Hodgkin lenfomalı (HL) hastaların %5’ini oluşturmaktadır. Hastalıkla ilgili en önemli sorunlar hastalık nüksü, Hodgkin dışı lenfomaya transformasyon ve tedavi ilişkili yan etkilerdir. Erken evre hastalıkta tedavisiz izlem, cerrahi, tutulu alan radyoterapisi ve tek başına ritüksimab tercih edilirken, yüksek tümör yükü olan olgularda ve ileri evre hastalıkta kemoimmünoterapi kullanılır, radyoterapi eklenebilir. Hastalık nüksü sonrasında dahi tedavi yanıtları oldukça iyidir. Tedaviye yönelik verilerin tamamı retrospektif çalışmalardan gelmektedir. Çalışmamızda kliniğimizde Aralık 2011-Aralık 2020 tarihleri arasında NLPHL tanısı alan 10 hasta değerlendirildi. Hastaların medyan yaşı 36 (28-60) yıldı. NLPHL tanılı hastalar HL tanılı hastaların %2.08’ini oluşturmakta idi. Hastaların %80’ni (n=8) erkekti. Tanı anında hastalarımızın %70’ni (n=7) erken evre idi ve tüm hastaların ECOG (Doğu Kooperatif Onkoloji Grubu) performans skoru 0’dı. Hastalarımızın hepsinde başvuru şikayeti ele gelen lenfadenopatiydi. Tanı anındaki hemogram ve biyokimyasal parametreler normal referans aralığındaydı. Tüm hastalarımızın bakılan immünhistokimyasal boyamalarında CD20 pozitifliği mevcuttu ve yalnız 1 (%10) hastanın CD30 pozitifliği mevcuttu. Dokuz (%90) hastamıza ilk sıra tedavi olarak ABVD (doksorubisin, bleomisin, vinblastin, dakarbazin) kemoterapisi uygulandı. Bir (%10) hastamıza ise kombine modalite tedavisi (CMT) olan ABVD ile birlikte RT uygulandı. Bir hastamıza hastalık progresyonu nedeni ile diğer hastamıza ise geç nüks sebebi ile kurtarma tedavisi sonrası otolog kök hücre nakli yapıldı. Kliniğimizde tanı konulan NLPHL hastalarının Amerika ve Almanya gibi yabancı ülkelerde yayınlanan literatürlerde belirtilen insidans oranları ve verilen ilk basamak tedavi seçimi ile uyumsuz bir tablo oluşturmaktadır. Bu farklılığın Türkiye’den bildirilecek diğer verilerle karşılaştırılması ve tartışılması uygun olacaktır.

Keywords

• Nodüler lenfosit predominant Hodgkin lenfoma
• Klasik Hodgkin Lenfoma
• prognoz

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