ecancermedicalscience

Research

Low incidence of primary immunodeficiency-associated cancers in children at a tertiary care pediatric hospital in Pakistan: a blessing in disguise or wet behind the ears?

30 Jul 2024
Rahat Ul Ain, Mahwish Faizan

Scarce data is available regarding primary immunodeficiency-associated cancers in children in low-middle-income countries. This study aimed to determine the incidence, clinical features and outcomes of primary immunodeficiencies (PIDs)-associated cancers in children presenting to Pakistan’s largest public-sector specialised pediatric oncology center. Among 5,748 children with cancers registered over 5 years, only eight patients were found to have PID-associated pediatric malignancies with an incidence of 1.4 per 1,000 cases. The median age at the time of diagnosis was 6.5 years with a male-to-female ratio of 7:1. Only four types of PIDs were found to be associated with cancer in children at our center: Ataxia Telangiectasia in 37.5% (n = 3), hyper-IgE syndrome and IgG deficiency in 25% (each n = 2) and one case (12.5%) of common variable immune deficiency. Six different types of pediatric cancers were associated with PID with a predisposition towards hematological malignancies (n = 7, 87.5%). Only two patients (25%) survived. The median survival of the cohort was 3.5 months. Infection-related mortality was the cause of death in four patients (66%), and the type of PID was the only statistically significant factor associated with the outcome. It is concluded that a lesser proportion of PID-associated pediatric cancers are found in our center as compared to the reported data from high-income countries. PID-associated cancers in children have an abysmal prognosis and infection-related mortality is the major cause of treatment failure. Sensitisation of oncologists to look for any underlying PID, the introduction of PID-screening programs in children and consideration of PID-associated malignancies as a high-risk group for treatment may help improve the outcomes.

Related Articles

Judith Naa Odey Tackie, Ernestina Schandorf, Patrick Bankah, Hafisatu Gbadamosi, Joseph Daniels, Mary Ann Dadzie
Pengkhun Nov, Yangfeng Zhang, Duanyu Wang, Syphanna Sou, Socheat Touch, Samnang Kouy, Virak Vicheth, Lilin Li, Xiang Liu, Changqian Wang, Peizan Ni, Qianzi Kou, Ying Li, Chongyang Zheng, Arzoo Prasai, Wen Fu, Wandan Li, Kunpeng Du, Jiqiang Li
Marceli de Oliveira Santos, Paulo Cesar Fernandes de Souza, Fernanda C da Silva de Lima, Nathalie V Balmant, Carolina Motta, Michele Gonçalves da Costa, Rejane de Souza Reis, Gemma Gatta, Beatriz de Camargo
Etienne Okobalemba Atenguena, Joseph Francis Nwatsock, Berthe Sabine Esson Mapoko, Lionel Fossa Tabola, Kenn Chi Ndi, Jérôme Boombhi, Paul Ndom