Ayca Kiykim1,2, Nursah Eker3, Ozlem Surekli4, Ercan Nain1,2, Nurhan Kasap1,2, Hacer Aktürk5, Omer Dogru3, Aylin Canbolat6, Ayper Somer7, Ahmet Koc3, Gulnur Tokuc3, Suheyla Bozkurt8, Kemal Turkoz8, Elif Karakoc-Aydiner1,2, Ahmet Ozen1,2, Safa Baris1,2. 1. Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey. 2. Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey. 3. Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey. 4. Department of Pediatrics, School of Medical, Marmara University, Istanbul, Turkey. 5. Department of Pediatrics, Division of Pediatric Infectious Diseases, Koc University, Istanbul, Turkey. 6. Pediatric Hematology and Oncology, Goztepe Training and Research Hospital, Medeniyet University, Istanbul, Turkey. 7. Department of Pediatrics, Division of Pediatric Infectious Diseases, Istanbul University, Istanbul, Turkey. 8. Department of Pathology, School of Medical, Marmara University, Istanbul, Turkey.
Abstract
BACKGROUND: Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. METHODS: Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. RESULTS: The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). CONCLUSION: In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.
BACKGROUND: Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. METHODS: Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. RESULTS: The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PIDpatients with lymphoma (P = 0.001). CONCLUSION: In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.
Authors: Eun Sil Kim; Dongsub Kim; Yoonsun Yoon; Yiyoung Kwon; Sangwoo Park; Jihyun Kim; Kang Mo Ahn; Soomin Ahn; Yon Ho Choe; Yae-Jean Kim; Mi Jin Kim Journal: Front Immunol Date: 2021-08-12 Impact factor: 7.561