PRIMARY IMMUNODEFICIENCY DISEASES AND MALIGNANCY IN TURKEY; A MULTICENTER STUDY
- Sara Sebnem Kilic, Turkey
Abstract
Background and Aims
There is no data regarding the prevalence of malignancies in patients with PID in Turkey. Along with the frequency of malignancy, we aimed to present the types of cancer and define the underlying immune deficiency of patients.
Methods
Between 1992 and 2018 years, from 5 tertiary immunology clinics, fifty-nine patients with PID who developed malignancy were included. All patients were evaluated for demographics, clinical features and prognosis. The data were obtained from the hospital records retrospectively.
Results
The prevalence of malignancy in our cohort was found as 0.9% (59/6538). The male/female ratio was 1.8 (38/21) and the median age of patients was 14 years (range 2- 51). The median age at diagnosis of malignancy was 10 years (range 2-51). The highest number of patients with malignancy had ataxia telangiectasia (n=17,28.8%), non-Hodgkin lymphoma was the leading malignancy (n=27,45.7%), the highest cancer rate was observed in patients with DOCK8 deficiency (16.3%) in all patients. The frequency and subtypes of malignancies are given in Table 1, Table 2 respectively. EBV quantitative PCR was positive in 16 out of 53 patients (30.2%). Two patients had dual malignancies. Remission was detected in 24 patients (40.6%), however 31 patients (52.5%) died.
Conclusions
The incidence of PIDs-associated malignancy has increased in recent years. While lymphoid malignancies are the most common cancer and observed more frequently in AT patients, higher susceptibility to cancer was detected in patients with DOCK8 deficiency. This study is the largest cohort investigating the association of malignancy in patients with PID in Turkey.