Primer İmmün Yetmezlik ve Malignite Birlikteliği: İki Olgu Sunumu

Year 2022, Volume: 6 Issue: 2, 196 - 200, 30.08.2022

Ümmügülsüm Dikici , Öner Özdemir , Emine Aylin Yılmaz , Mehmet Fatih Orhan

https://doi.org/10.34084/bshr.1095949

Abstract

Primer immun yetmezlikler tekrarlayan enfeksiyon dışında malignitelere yatkınlık oluşturan hastalıklardır. Primer immun yetmezlikli hastalarda bazı bilinmeyen mekanizmalar ve kusurlu DNA onarım süreçleri nedeniyle oluşan genomik instabilite, kanser riskinde artışa neden olur ve bu hastalarda genel olarak kanser gelişme riskinin % 4,7- 5,7 arasında değiştiği düşünülmektedir. Primer immun yetmezlikli hastaların enfeksiyonlardan sonra en sık ölüm nedeni malignitelerdir. Bu makalede sunmuş olduğumuz ilk olgumuz öksürük şikâyetiyle hastaneye başvuran ve takibinde Burkitt Lenfoma tanısı alan, bu aşamada tetkiklerinde immünglobülinlerinde düşüklük saptanarak lenfoma tanısına eş zamanlı olarak yaygın değişken immün yetmezlik tanısı eklenen hastamızdır. İkinci olgumuz primer immun yetmezlik nedeniyle takipliyken akut lenfoblastik lösemi gelişen hastamızdır. Burada primer immun yetmezlikli hastaların olağanın dışında ilk başvurusunun maligniteyle beraber de olabileceğine dikkat çekmeyi ve primer immun yetmezlikli hasta takip eden klinisyenlerin malignite gelişimine karşı farkındalıklarını arttırmayı amaçladık.

Keywords

Akut Lenfoblastik Lösemi, Burkitt Lenfoma, Yaygın Değişken İmmun Yetmezlik

Primary Immunodeficiency and Malignant Diseases: Two Case Report

Abstract

Primary immunodeficiencies are diseases that predispose to malignancies other than recurrent infections. Genomic instability due to some unknown mechanisms and defective DNA repair processes in patients with primary immunodeficiency causes an increase in cancer risk, and it is thought that the risk of developing cancer in these patients varies between 4.7-5.7%. After infections, the most common cause of death in patients with primary immunodeficiency is malignancies. In this article, our first case is the patient who applied to the hospital with the complaint of cough and was diagnosed with Burkitt's lymphoma in the follow-up, and at this stage, low immunoglobulins were detected in the tests and the diagnosis of lymphoma was simultaneously diagnosed as common variable immunodeficiency. Our second case is our patient who developed acute lymphoblastic leukemia while being followed up due to primary immunodeficiency. Here, we aimed to draw attention to the fact that the unusual first presentation of patients with primary immunodeficiency may be associated with malignancy and to increase the awareness of clinicians following patients with primary immunodeficiency about the development of malignancy.

Keywords

Acute Lymphoblastic Leukemia, Burkitt Lymphoma, Common Variable Immunodeficiency

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