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Treatment options for lymphoproliferative disease associated with primary immunodeficiency
Source: Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 17, 2025 · Captured 2026-09-09.
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Treatment options for lymphoproliferative disease associated with primary immunodeficiency include the following:
Chemotherapy with or without rituximab.
Allogeneic hematopoietic stem cell transplant (HSCT).
Patients with primary immunodeficiency can achieve complete and durable remissions with standard chemotherapy regimens for NHL, although toxicity is increased.[2]; [5][Level of evidence C1] Recurrences in these patients are common and may not represent the same clonal disease.[6] Immunologic correction through allogeneic HSCT is often required to prevent recurrences.
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