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.
Description:
Pneumology and Alergology Discipline, State University of Medicine and Pharmacy "Nicolae Testemitanu", Chișinău, Republic of Moldova, Congresul consacrat aniversării a 75-a de la fondarea Universității de Stat de Medicină și Farmacie „Nicolae Testemițanu” din Republica Moldova, Ziua internațională a științei pentru pace și dezvoltare