Immunodeficiency and Cancer
Summary of Key Points
• Cancer remains a major cause of mortality among patients with primary and acquired immunodeficiencies.
• Cancer in immunocompromised hosts is frequently associated with infectious agents, including the following:
Epstein-Barr virus (associated with lymphoproliferative disorders and leiomyosarcoma)
Human papillomavirus (associated with skin anal and cervical carcinomas)
• Categories of genetic immunodeficiencies with increased risk of the development of cancer include the following:
Combined defects with T-cell dysfunction (e.g., severe combined immunodeficiency or Wiskott-Aldrich syndrome)
Defects that inhibit lymphoid apoptosis (e.g., autoimmune lymphoproliferative syndrome)
Defects of genomic instability (e.g., ataxia telangiectasia)
Acquired deficiencies in T-cell immunity with increased risk of developing cancer, such as:
• Cancer can occur de novo, preexisting cancer can recur, or cancer can be transmitted by a donor.
• In general, the outcome for immunocompromised patients with cancer is inferior to the outcome for the general cancer population.
1. Lack of function in which cells of the immune system is most associated with increased risk of cancer?
2. Which of the following infectious agents is not associated with cancers of immunodeficiency?
3. A history of which cancer in a living kidney donor puts the recipient at the highest risk of transmission of cancer to the recipient of the donated kidney?
1. Answer: C. Antigen presentation via macrophages (dendritic cells) or B cells are important in providing an appropriate immune response, and there is evidence that natural-killer cells can kill tumor cells. However, cancer development has been most strongly associated with defects in T-cell function in primary immunodeficiency and in suppression of T-cell function in secondary immunodeficiencies, such as human immunodeficiency virus and transplant recipients.
2. Answer: B. In immunodeficient patients, EBV is associated with lymphoma, gastric carcinoma, and leiomyosarcoma; HHV8 is associated with Kaposi sarcoma and pleural effusion lymphoma; HPV is associated with squamous cell carcinoma; and H. pylori is associated with mucosa-associated lymphoid tissue lymphoma. HSV has not been associated with cancers that immunodeficient patients are at increased risk to develop.
3. Answer: E. A history of resected low-grade cancer has not been associated with donor transmission to a recipient of an organ transplant. Although it is a rare event, cases have been reported of donor transmission of advanced stage hematologic cancers, such as large-cell lymphoma.
