PIDs are characterized by recurrent infections and increased risk of malignancies because of the reduced immunological surveillance against cancer cells and oncogenic viruses.
We report the incidence and the characteristics of the treatment of tumors among 690 patients with PID, diagnosed from 1990 until 2017 in Brescia.
Out of 690 patients, 25 patients(3.6%) developed 33 tumors.Of the 25 affected patients, 8 patients suffered from CVID, 5 from CID, 3 from AT, 2 from HSP2, 2 from XLA, 2 from WAS, 2 from HIES, 1 from SCID.The age at diagnosis ranged from 1 to 52 years, with a median age of 19.6 years. The time between diagnosis of PID and onset of tumor was short, often<1 year between diagnosis and appearance of cancer in case of CID.Moreover, in two cases of CID, the diagnosis of cancer was made before the diagnosis of PID, so cancer was the onset clinical manifestation.Hematological malignancies were prevalent(22/33,66.7%) with a minority of solid tumors(11/33,33.33%).In particular Non-Hodgkin lymphomas were the most frequent(16/33,48.48%).In total 13 patients survived(52%) and tumor was the main cause of death(7 cases).Two patients underwent BMT once the disease was in remission.
Malignancies in PID patients can be successful treated if a prompt diagnosis is posed.Moreover tailored treatment is often necessary.Our data suggest an encouraging survival rate(52%) in lymphomas.
ESID Subregistry on PID and Malignancies was created with aim to analyze more in depth the phenomenon and to understand how to improve the prognosis of these patients often considered at poor prognosis.