Tre-O1-01 - Characteristics, management and course of mycosis fungoides in solid organ transplant recipients: a retrospective cohort from two cutaneous lymphoma outpatient clinics

Abstract

<h3>Background:</h3> Primary cutaneous lymphoproliferative disorders in solid organ recipients are rare. Reports of mycosis fungoides (MF) diagnosed in these patients are limited to less than 30 cases, with a heterogeneous disease course. Furthermore, management in terms of type and modulation of the preceding immunosuppressive treatments after MF diagnosis was not uniform. <h3>Objective:</h3> To report the characteristics of MF, treatment response, disease course, and status of immunosuppressive therapy in patients who are solid organ recipients, or under similar immunosuppression. <h3>Methods:</h3> The medical files were retrospectively reviewed for all solid organ transplant patients/patients who are under similar immunosuppression for other indications, who were also diagnosed with MF, and were followed at the cutaneous lymphoma outpatient clinics of 2 tertiary medical centers from 1/2010 to 12/2020. <h3>Results:</h3> The cohort included 11 patients (8 male; median age at organ transplantation/immunosuppressive state, 33 years). Transplanted organs involved the liver (n=5), kidney (n=3), liver and kidney (n=1), or heart (n=1). Patients were treated with mycophenolic acid, tacrolimus, and prednisone (n=5) or tacrolimus (n=5). One patient with nephrotic syndrome received cyclosporine. Median age at diagnosis of MF was 48 years, 45% were diagnosed before the age of 18 years, median time from organ transplantation/immunosuppressive state to diagnosis of MF was 8 years (range 0.5–22 years). Ten patients had early stage, (IA, 5; IB, 5), 50% of whom had early folliculotropic MF (FMF), (in some combined with classic/hypopigmented MF). One patient had advanced MF (IIIA) with folliculotropic erythroderma. Early-stage patients were treated mainly with skin-directed therapies, achieving either partial (50%) or complete response (50%). The erythrodermic patient achieved partial response after being treated with ultraviolet A and narrow-band ultraviolet B with acitretin. Immunosuppressive treatment was modulated in 4/11 patients, (cyclosporine switched to mycophenolic acid, 1; mycophenolic acid stopped and tacrolimus continued, 1; tacrolimus continued at a lower dose, combined with mycophenolic acid and prednisone, 1; tacrolimus stopped, 1). Median time of follow-up was 4 years (range 1–8 years), with no stage progression noted. At the last visit, 6 had no evidence of disease, and 5 had active disease (IA, 4; III, 1). <h3>Conclusions:</h3> Most solid organ recipient MF patients present with early-stage disease, similar to MF in the general population, with over representation of FMF, and over representation among children. Treatment is challenging due to the notorious higher risk of non-melanoma skin cancer in organ transplant recipients. Furthermore, immunosuppressive therapy modifications to improve MF should be balanced against the risk of solid organ rejection. Response to treatment and course do not seem to differ from MF in the general population, although longer follow-up is needed.