FROM INBORN ERRORS OF IMMUNITY TO LYMPHOMA – A HEMATOLOGIST’S POINT OF VIEW OD UROĐENIH GREŠAKA IMUNITETA DO LIMFOMA – PERSPEKTIVA HEMATOLOGA
Journal
Medicinski Pregled
Date Issued
2022-10
Author(s)
Goran MARJANOVIĆ
Tanja Dzopalic
Milos Kostic
Milan Lazarevic
Branka Bonaci Nikolic
DOI
10.2298/MPNS22S1066M
Abstract
After infections, malignancies, lymphomas especially, are the second most frequent cause of death in patients with inborn errors of
immunity. Factors predetermining the appearance and aggressiveness
of lymphomas include gene defects, defects of immune surveillance
and regulation as well as infections with oncogenic viruses. Aggressive non-Hodgkin lymphomas, mostly diffuse large B-cell and Bukit
subtypes are predominant in deoxyribonucleic acid repair defects,
while Hodgkin lymphoma becomes equally present in patients with
defects of immune regulation. Marginal zone and mucosa-associated
lymphoid tissue lymphomas, appear to be frequent in defects of antibody production, especially in patients with common variable immune deficiency. The prevalence of Epstein-Barr virus may vary
within entities, but there is no entity without at least a few cases of
lymphoma and Epstein-Barr virus co-infection. Standard treatment
of lymphomas associated with deoxyribonucleic acid repair defects
and severe combined deficiencies, is stem cell transplantation. Lymphomas in inborn errors of immunity with a less severe clinical
presentation, should be treated with immunochemotherapy and
monoclonal antibodies (Brentuximab, Rituximab) wherever feasible. There is no data about the usefulness of checkpoint inhibitors,
bi-specific antibodies and T-cells with chimeric antigen receptor.
Allogeneic stem cell transplantation represents a major indication
for treatment of relapse/refractory lymphomas in any inborn error
of immunity. Potential benefit of therapy with Chimeric antigen
receptor Natural-killer cells in lymphomas associated with inborn
errors of immunity, remains to be seen in future studies.
Key words: Immune System Diseases; Metabolism, Inborn Errors;
Lymphoma; Hodgkin Disease; Lymphoma, Non-Hodgkin; Therapeutics
immunity. Factors predetermining the appearance and aggressiveness
of lymphomas include gene defects, defects of immune surveillance
and regulation as well as infections with oncogenic viruses. Aggressive non-Hodgkin lymphomas, mostly diffuse large B-cell and Bukit
subtypes are predominant in deoxyribonucleic acid repair defects,
while Hodgkin lymphoma becomes equally present in patients with
defects of immune regulation. Marginal zone and mucosa-associated
lymphoid tissue lymphomas, appear to be frequent in defects of antibody production, especially in patients with common variable immune deficiency. The prevalence of Epstein-Barr virus may vary
within entities, but there is no entity without at least a few cases of
lymphoma and Epstein-Barr virus co-infection. Standard treatment
of lymphomas associated with deoxyribonucleic acid repair defects
and severe combined deficiencies, is stem cell transplantation. Lymphomas in inborn errors of immunity with a less severe clinical
presentation, should be treated with immunochemotherapy and
monoclonal antibodies (Brentuximab, Rituximab) wherever feasible. There is no data about the usefulness of checkpoint inhibitors,
bi-specific antibodies and T-cells with chimeric antigen receptor.
Allogeneic stem cell transplantation represents a major indication
for treatment of relapse/refractory lymphomas in any inborn error
of immunity. Potential benefit of therapy with Chimeric antigen
receptor Natural-killer cells in lymphomas associated with inborn
errors of immunity, remains to be seen in future studies.
Key words: Immune System Diseases; Metabolism, Inborn Errors;
Lymphoma; Hodgkin Disease; Lymphoma, Non-Hodgkin; Therapeutics
File(s)![Thumbnail Image]()
Loading...
Name
From inborn errors of immunity to lymphoma 0025-810522066M.pdf
Size
424.42 KB
Format
Adobe PDF
Checksum
(MD5):cbf219cbb2d329214ea1bce56e276d60