BI17 Generalized morphoea associated with anaplastic lymphoma and Hodgkin lymphoma: a report of two cases

Abstract Managing generalized morphoea in the context of lymphoma poses significant challenges due to the complexities of balancing immunosuppressive therapy with the need to treat malignancy. These issues are further compounded by the increased risk of transformation or recurrence in patients with autoimmune conditions who require immunosuppression treatment. Here, we present two cases that highlight these challenges. The first case involves a 59-year-old woman with a history of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), previously treated with six cycles of rituximab, cyclophosphamide, vincristine sulfate and prednisolone chemotherapy. After achieving remission, she remained under active surveillance. Her medical history also included acromegaly secondary to a pituitary microadenoma, treated with transsphenoidal adenectomy, and Sjögren syndrome. Seven years following treatment for lymphoma, she presented with progressive skin hardening involving the arms, submammary region, thighs and lower legs. Examination revealed widespread indurated plaques, significant movement restriction, and active erythematous areas, which severely impacted her activities of daily living. Initial management included methotrexate, pulsed methylprednisolone, hydroxychloroquine and ultraviolet (UV)A1 phototherapy. However, a year later, she developed recurrent NLPHL, with high-grade transformation to T-cell-rich B-cell lymphoma. She was treated with six cycles of cyclophosphamide, doxorubicin, prednisone, rituximab and vincristine chemotherapy and remains in remission. Her morphoea has since recurred, requiring hydroxychloroquine and further UVA1 therapy, with a good clinical response. The second case involved a 54-year-old woman with a background of long-standing psoriasis and Sjögren syndrome, who presented with generalized morphoea characterized by extensive skin thickening, induration and joint mobility restriction. Treatment was initiated with hydroxychloroquine and methotrexate. However, within 4 months, she developed cervical lymphadenopathy, and a biopsy confirmed stage IVB anaplastic large cell lymphoma. Immunosuppressive medications for morphoea were discontinued, and she had a poor response to the initial chemotherapy regimen of brentuximab, cyclophosphamide, doxorubicin and prednisolone. She is due to begin an alternative chemotherapy regimen, with plans for autologous stem cell transplantation. These cases highlight the intricate interplay between generalized morphoea and lymphoma, emphasizing the need for a tailored approach to therapy. The heightened risk of lymphoma in autoimmune conditions necessitates careful balancing of immunosuppression and oncological treatment, with a close multidisciplinary approach to optimize patient outcomes.