PRIOR MALIGNANCY DOES NOT CONFER INFERIOR OUTCOMES IN TEENAGERS AND ADULTS WITH PRIMARY IMMUNODEFICIENCIES UNDERGOING ALLOGENEIC STEM CELL TRANSPLANTATION.
- Emma C. Morris, United Kingdom
Abstract
Background and Aims
Primary immunodeficiency disorders (PIDs) result in recurrent infections and a predisposition to malignancy due to impaired immune surveillance.1Large registry studies have demonstrated a 1.42-fold excess relative risk of malignancy, with lymphoproliferative disorders the most common.2Allogeneic haematopoietic stem cell transplantation (alloHSCT) is the only curative option for most PIDs.3Recently, reduced intensity conditioned alloHSCT has been shown to be effective and safe in adults with PID.4,5PID associated malignancy is often a trigger for alloHSCT in adult PID patients.
Methods
Evidence from the paediatric population suggests the presence of malignancy in PID patients should not preclude alloHSCT.6 There is scant data on alloHSCT outcome in adult PID patients with a history of malignancy. We compared overall survival (OS) between patients with a history of a malignancy (n= 12) and those without (n=35) in our cohort of (n=47) teenagers and young adults (aged >13, median 22) with PID (Fig 1). The patients in the malignant group had haematological (6 MDS, 3 Hodgkin Lymphoma, 1 B-cell NHL, 1 T-cell NHL, 1 autoimmune lymphoproliferative disorder) and epithelial malignancies (2 VIN/CIN/AIN and 1 poorly differentiated squamous cell carcinoma). Patients with lymphoma were in remission prior to transplant.
Results
OS in the patients with malignancy was 100% (median follow up 4 years) with no recurrence of malignancy post-transplant(vs 82.9% in the non-malignant group, p=0.147).
Conclusions
AlloHSCT may be particularly useful in adult patients with PID-associated malignancy as the restoration of immune surveillance and provision of a life-long graft-vs-tumour effect may reverse the inherited predisposition to malignancy.