Ouassou Safaa, Herrak Laila, Achachi Leila, Nachite Fatima, Znati Kaoutar, Ftouh Mustapha El
Service de Pneumologie, Hôpital Ibn Sina, CHU Rabat, Maroc.
Laboratoire d'Anatomo-pathologie, Hôpital Ibn Sina, CHU Rabat, Maroc.
Pan Afr Med J. 2016 Jun 30;24:181. doi: 10.11604/pamj.2016.24.181.9906. eCollection 2016.
Primary mediastinal large B-cell lymphoma (PMBL) is a lymphoma occurring in the anterior mediastinum starting from the cells B of the thymique medullary zone. This is a rare entity characterized by epidemiological, clinical and evolutionary peculiarities as well as by pathological and immunohistochemical peculiarities. We report a case series of 5 patients with diagnosed PMBL hospitalized in Pulmonology Department of Ibn Sina Hospital between January 2012 and May 2016. The average age was 34 years, the median of consultation time was 2 months. Reported symptoms were dyspnea, chest pain, dry cough; two patients suffered from superior vena cava syndrome. LDH level was high in 4 patients. Thoracic imaging showed an anterior mediastinal tissue processing in 5 patients. Histological diagnosis was based on ultrasound-guided transparietal puncture biopsy in 5 patients. The contribution of immunohistochemistry was decisive in all cases. Patients were sent to the National Institute of Oncology for therapeutic management. PMBL prognosis is reserved, it most commonly occurs in young women, which increases the need of aggressive therapy to improve survival rate.
原发性纵隔大B细胞淋巴瘤(PMBL)是一种发生在前纵隔的淋巴瘤,起源于胸腺髓质区的B细胞。这是一种罕见的疾病,具有流行病学、临床和演变特征,以及病理和免疫组化特征。我们报告了2012年1月至2016年5月期间在伊本·西那医院肺病科住院的5例确诊为PMBL的病例系列。平均年龄为34岁,就诊时间中位数为2个月。报告的症状为呼吸困难、胸痛、干咳;2例患者患有上腔静脉综合征。4例患者乳酸脱氢酶水平升高。胸部影像学检查显示5例患者前纵隔组织有病变。5例患者的组织学诊断基于超声引导下经胸壁穿刺活检。免疫组化在所有病例中起决定性作用。患者被送往国家肿瘤研究所进行治疗管理。PMBL的预后不容乐观,它最常见于年轻女性,这增加了采用积极治疗以提高生存率的必要性。