Malignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat
Ayca Kiykim
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorNursah Eker
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorOzlem Surekli
Department of Pediatrics, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorErcan Nain
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorNurhan Kasap
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorHacer Aktürk
Department of Pediatrics, Division of Pediatric Infectious Diseases, Koc University, Istanbul, Turkey
Search for more papers by this authorOmer Dogru
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorAylin Canbolat
Pediatric Hematology and Oncology, Goztepe Training and Research Hospital, Medeniyet University, Istanbul, Turkey
Search for more papers by this authorAyper Somer
Department of Pediatrics, Division of Pediatric Infectious Diseases, Istanbul University, Istanbul, Turkey
Search for more papers by this authorAhmet Koc
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorGulnur Tokuc
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorSuheyla Bozkurt
Department of Pathology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorKemal Turkoz
Department of Pathology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorElif Karakoc-Aydiner
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorAhmet Ozen
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorCorresponding Author
Safa Baris
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Correspondence
Safa Baris, Marmara University, Division of Pediatric Allergy/Immunology, Istanbul, Turkey.
Email: [email protected]; [email protected]
Search for more papers by this authorAyca Kiykim
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorNursah Eker
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorOzlem Surekli
Department of Pediatrics, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorErcan Nain
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorNurhan Kasap
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorHacer Aktürk
Department of Pediatrics, Division of Pediatric Infectious Diseases, Koc University, Istanbul, Turkey
Search for more papers by this authorOmer Dogru
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorAylin Canbolat
Pediatric Hematology and Oncology, Goztepe Training and Research Hospital, Medeniyet University, Istanbul, Turkey
Search for more papers by this authorAyper Somer
Department of Pediatrics, Division of Pediatric Infectious Diseases, Istanbul University, Istanbul, Turkey
Search for more papers by this authorAhmet Koc
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorGulnur Tokuc
Department of Pediatrics, Division of Pediatric Hematology and Oncology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorSuheyla Bozkurt
Department of Pathology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorKemal Turkoz
Department of Pathology, School of Medical, Marmara University, Istanbul, Turkey
Search for more papers by this authorElif Karakoc-Aydiner
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorAhmet Ozen
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Search for more papers by this authorCorresponding Author
Safa Baris
Department of Pediatrics, Division of Pediatric Allergy and Immunology, School of Medical, Marmara University, Istanbul, Turkey
Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, Istanbul, Turkey
Correspondence
Safa Baris, Marmara University, Division of Pediatric Allergy/Immunology, Istanbul, Turkey.
Email: [email protected]; [email protected]
Search for more papers by this authorAbstract
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.
CONFLICTS OF INTEREST
The authors declare no conflict of interest.
Open Research
DATA AVAILABILITY STATEMENT
The data that support the findings of this study are available from the corresponding author upon reasonable request.
Supporting Information
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Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
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