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Relationship of immunodeficiency to lymphoid malignancy
J H Kersey1, R S Shapiro, A H Filipovich
1Bone Marrow Transplantation Program, University of Minnesota, Minneapolis.
The Pediatric Infectious Disease Journal
|May 1, 1988
Summary
Individuals with immunodeficiencies face increased risks of lymphoid malignancies. Non-Hodgkin's lymphomas are most common in primary immunodeficiencies and after bone marrow transplantation, with varied histopathology across disorders.
Area of Science:
- Immunology
- Oncology
- Hematology
Background:
- Primary and secondary immunodeficiencies increase cancer risk, particularly lymphoid malignancies.
- Malignancies in immunodeficiency syndromes are a significant concern, especially in naturally occurring conditions and post-bone marrow transplantation (BMT).
Purpose of the Study:
- To review malignancies developing in immunodeficiency syndromes.
- To analyze the types and characteristics of cancers in patients with primary immunodeficiencies and following BMT.
Main Methods:
- Analysis of 514 cases from the Immunodeficiency Cancer Registry for naturally occurring immunodeficiencies.
- Review of lymphoid malignancy cases following bone marrow transplantation.
Main Results:
- Non-Hodgkin's lymphomas (NHL) are the predominant malignancy (48.6%) in patients with primary immunodeficiencies.
- NHL is common in ataxia-telangiectasia, common variable immunodeficiency, Wiskott-Aldrich syndrome (WAS), and severe combined immunodeficiency (SCID), with distinct histopathological features.
- Post-BMT immunodeficiency also predisposes to lymphoid malignancies, particularly in recipients of partially mismatched bone marrow.
Conclusions:
- Immunodeficiency syndromes significantly elevate the risk of lymphoid malignancies.
- Non-Hodgkin's lymphomas are the most frequent cancers observed in these patient groups.
- Further research into the specific mechanisms and management of these malignancies is warranted.