Lymphoblastic lymphoma is a rare and aggressive form of non-Hodgkin’s lymphoma (NHL), occurring in adolescents or young adults with a male:female sex ratio of 3:1(1). T-cell lymphoblastic lymphoma accounts for about 90% of NHLs [1, 2]. Its incidence is estimated to be about 0.1–3 per 100,000 population per year [2, 3]. Although pericardial involvement due to malignancies has been described, it remains a rare and late manifestation of lymphomas and leukemias [4, 5]. Malignant pericardial effusion due to lymphoma is even more rare, with only a few cases having been reported [4,5,6]. Here we report a case of a 17-year-old adolescent with no medical history who presented with a lymphoma revealed by pericardial tamponade. Pericardial effusion may be due to direct tumor infiltration or related to venous–lymphatic compression or hematogenous dissemination. It may also be secondary to autoimmune disorders or to chemotherapy or radiotherapy used for the treatment of this malignancy [3, 4, 7].
The classic presentation of lymphoma is lymphadenopathy and B symptoms (fever, weight loss, night sweats), usually associated with a mediastinal mass that can be manifested by compressive signs [1, 3, 6, 7]. Dyspnea is often the main symptom leading to consultation, and may be related to compression of the superior vena cava by the mediastinal mass and pleural and/or pericardial effusion [1]. Pericardial localization of lymphoma is rare and even rarer in the form of cardiac tamponade [3, 6, 8, 9].
Pericardial involvement of non-Hodgkin’s lymphoma is a rare and usually late manifestation, approximately 20 months after diagnosis of the lymphoma [3, 8]. It accounts for 0.5% of cardiac involvement and only 1% of extracardiac involvement [4, 10].
Transthoracic echocardiography is the first-line modality for the diagnosis of tamponade, which will be confirmed by the right heart catheterization [3, 8]. Echocardiography allows for diagnosis of tamponade to be made by specifying the elements that have good sensitivity, of at least 90%, such as the appearance of a swinging heart, Right Atrial (RA) systolic collapse, early Right Ventricular (RV) collapse, and a dilated vena cava that cannot be modulated by breathing [8]. In our case, dyspnea associated with chest pain led to electrocardiogram being carried out. This showed sinus tachycardia with electrical alternans. The transthoracic echocardiography confirmed the diagnosis of large pericardial effusion and tamponade. The presence of a pericardial effusion is associated with a significant decrease in survival and the occurrence of major adverse events such as cardiac tamponade, cardiovascular collapse, and death [1, 4].
CT is one of the scans carried out as part of the etiological workup of any pericardial effusion. Its interest is to confirm or rule out a possible tumoral process [6]. Mediastinal mass is not uncommon in non-Hodgkin’s lymphoma; it is found in 50–75% of cases [1,2,3, 6, 7]. Thoracic CT scan carried out in our adolescent patient allowed for the highlighting of the compressive mediastinal mass. The diagnosis of lymphoblastic lymphoma was made by histological examination. In our case, the histology and immunohistochemical markers confirmed T-lymphoblastic lymphoma.
After pericardiocentesis, patient was no longer dyspneic and was started on chemotherapy treatment by the clinical hematology team for his lymphoma.
