Parathymic syndromes in a young patient with thymoma: bronchiectasis, erythroblastopenia and ulcerative colitis

Abstract

Introduction: Thymomas are among the rare forms of mediastinal tumors, with more than 20 associated parathymic syndromes being described. The connection between autoimmune and thymoma manifestations has long been known, although the mechanisms involved are still a mystery. Methods and Materials: A case of a 41-year-old patient diagnosed with thymoma at the age of 30 is presented. Erythroblastopenia was confirmed by the reduction of erythrocyte series in bone marrow. Ulcerative colitis confirmed by histological examination. Thoracic HRCT (at age of 30-33-39-40 years; B, E, H, I) allowed pulmonary lesions assessment. Good’s syndrome was ruled out by normal serum immunoglobulins values. Results: The onset of the disease with low grade fever and retrosternal pain at age 27, misdiagnosed as pericarditis (mediastinal enlargement on chest X-ray) led to treatment with methylprednisolone. Pregnancy at 28 years old (birth of a healthy child) was the reason for the refusal of further examinations and surgical treatment, but contributed to the significant tumor growth with clinical manifestations of parathymic syndromes (erythroblastopenia with severe anaemia, ulcerative colitis), which did not disappear after thymectomy and splenectomy (over 2 years). Lung lesions have progressed from centrilobular nodules to bilateral extensive cystic bronchiectasis. Discussion: Around 1-8% of adults with bronchiectasis are diagnosed with humoral immune deficiency. Systemic inflammatory disorders or immune deficiencies, such as hypogammaglobulinemia or HIV infection is often related to be the cause of diffuse bronchiectasis. Patients that have thymic neoplasms sometimes may present with some thoracic symptoms or be clinically indolent. There are known multiple autoimmune parathymic syndromes associated with thymic neoplasms. Understanding the potential etiologies of bronchiectasis in thymic neoplasms helps the management of these patients. Thymomectomy should be an indication for these kind of patients in order to reduce the parathymic implications. Conclusions: The case illustrates rare forms of parathymic syndromes as well as the impact of thymectomy and splenectomy on their evolution. Thymoma-associated bronchiectasis predisposes to recurrent respiratory infections and progressive worsening of lung function, including in young patients.