Background and Aims

Malignancy may be the first presentation of primary immunodeficiency (PID). Diagnosis of PID could alter management and delay in diagnosis may result in further organ damage.

This review aimed to determine:

whether immune investigations were undertaken for children with lymphoma.

number of children with abnormal immunoglobulins prior to or during treatment and whether immunoglobulin replacement was provided.

whether any child was diagnosed with PID.

Methods

A retrospective review was performed of children diagnosed with lymphoma from 1st March 2016 – 1st March 2019. Information was collected on: diagnosis, immunological investigation undertaken and whether immunoglobulin replacement was provided.

Results

64 children were diagnosed with lymphoma at median age 13 years 8 months (range 2 – 17 years). Ten had immunoglobulins measured prior to/at diagnosis, one was low. Nine had low IgG after diagnosis, including two that were previously normal. Five children received immunoglobulin replacement (IRT). Two had hypogammaglobulinaemia that was not rechecked after cessation of IRT. Two further children remain hypogammaglobulinaemic after treatment.

Ten patients were lymphopaenic at presentation. Two had lymphocyte subsets performed after commencing treatment, both were abnormal. Five further children had subsets measured prior to treatment, of which two were abnormal, however, both normalised after treatment. Four others had subsets only after initiation of treatment.

One child has persistent lymphopaenia and hypogammaglobulinaemia 2.5 years after treatment. Further immune investigation has been recommended. No child was diagnosed with PID.

Conclusions

Immune investigation was rarely undertaken and often normal. Further work is required to know who and when to investigate for PID.