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clinical data were available, were excluded (see flowchart, Figure 1). Twenty-five of the remaining 209 cases (12%) were identified in patients with an immunodeficiency. Table 1 and Table 2 give an overview of the clinical char- acteristics, ordered by the underlying disorder in the latter. The majority of cases of T-NHL were found in patients with an auto-immune disease (56%). Other underlying disorders were hematologic malignancies (24%), solid organ transplantation (16%) and HIV infection (4%). Previously published case reports and case series are sum- marized in Online Supplementary Table S1, also ordered by the underlying disorder. For comparison, in our PubMed search 605 cases of immunodeficiency-related T-NHL were identified, of which 201 occurred in patients with HIV infection (33%), 197 in transplant recipients (33%), 143 in patients with underlying autoimmune diseases (24%), 55 following previously treated hematologic malig- nancies (9%) and nine in patients with a primary immun- odeficiency (1%).
In our series, T-NHL occurred at a median age of 52 years (interquartile range 39.5-73), which is nine to ten years younger than that observed in studies on T-NHL in the general population.25,26 Patients with Crohn’s disease and HIV were younger than patients with hematologic malignancies and solid organ transplantations. This is con-
sistent with the literature on HIV and PTLD, in which the mean/median ages reported were 38-39 and 43.5-57.5 respectively.14,15,17-20
In line with these studies, we found a male predomi- nance (80%).14,15,17-20,26,27 Most patients had been treated with either immunosuppressive therapy or chemotherapy prior to the diagnosis of lymphoma (88%). For patients for whom information was available regarding the exact start date of the therapy with immunosuppressive or chemotherapeutic agents, the median interval between the start of the immunosuppressive treatment and the time of diagnosis was 76 months (interquartile range 14.3- 171; N=16). In all patients for whom the duration of the drug use could be deduced from the clinical records, 60% had used one or more immunosuppressive or chemother- apeutic agents for at least two years (N=20). In all 25 patients, most (60%) had used prednisolone for varying periods. Azathioprine was used by 32%, including 86% of the patients with Crohn’s disease; those for whom it was documented (7 out of 8) used this drug for longer than two years. Drugs somewhat less frequently used were chlo- rambucil and cyclophosphamide (both 16%), mostly by patients with hematologic malignancies, and tacrolimus (12%) by solid organ transplant recipients. A few patients had been treated with adalimumab, infliximab, rituximab,
Figure 1. Cases included. Flowchart of inclusion of cases of T-NHL in patients with immunodeficiencies due to an under- lying disorder or immunosuppressive drugs in the period 1999-2014. AI: autoim- mune; Hem. malignancy: hematologic malignancy; HIV: human immunodeficien- cy virus; SOT: solid organ transplantation; T-NHL: T-cell non-Hodgkin lymphoma.
haematologica | 2018; 103(3)