Introduction: Patients with systemic sclerosis may develop cancer. The development of malignant hematological diseases has also been described in patients with systemic sclerosis. These patients may also be prone to the development of lymphoma, in most cases a B-cell lymphoma.
Aim: The aim was to describe the case of a patient with systemic sclerosis who had a non-Hodgkins lymphoma and developed primary hypothyroidism.
Case report: A patient, female, aged 39, presented with anemia and fever. Laboratory investigations revealed Hb 10 g/dl, Ht 30.01% and Fe 19 μg/dl. A diagnostic evaluation was performed for the presence of an infection and antibiotics were administered. Four years earlier a non-Hodgkin T cell lymphoma had been diagnosed and was followed up. She had sclerodactyly, telangiectasias in the area of the face and the posterior surface of the trunk. Gastroscopy was performed and showed impaired peristaltic movement of the esophagus and esophagitis. A chest CT revealed fibrosis and pleural effusions bilaterally. Further laboratory evaluation revealed TSH 20.45 μIU/ml (normal values 0.354.94 μIU/ml), FT4 0.6 ng/dl (normal values 0.71.48 ng/dl), FT3 1.5 pg/ml (normal values 1.83.7 pg/ml) and positive antithyroid antibodies. Thyroxine was administered.
Conclusions: Systemic sclerosis is a fibrosing systemic inflammatory disease possibly of autoimmune etiology. In the course of autoimmune diseases lymphoma may develop. The development of hematological malignancies has been described in patients with systemic sclerosis, in particular B cell lymphoma. The extremely rare coexistence of primary hypothyroidism, systemic sclerosis and T cell lymphoma is described. This observation shows that follow up and therapeutic management of patients with systemic autoimmune diseases should be performed with caution as these patients are prone to the development of hematological and other autoimmune diseases.
18 - 21 May 2019
European Society of Endocrinology