Iordan Iuliana, Vladareanu Ana-Maria, Mambet Cristina, Dumitru Ion, Onisai Minodora, Cisleanu Diana, Bumbea Horia
Department of Hematology, "Carol Davila" University of Medicine and Pharmacy, Emergency University Hospital, Bucharest, Romania.
Department of Medical Semiology and Nephrology, "Carol Davila" University of Medicine and Pharmacy, Emergency University Hospital, Bucharest, Romania.
Maedica (Bucur). 2024 Jun;19(2):428-433. doi: 10.26574/maedica.2024.19.2.428.
Adult T-cell leukemia/lymphoma (ATLL) is a rare T-cell lymphoproliferative disease associated with human T-cell leukemia virus type 1. There are four subtypes of ATLL: smoldering, chronic, lymphoma and acute. All subtypes can exhibit extranodal involvement. Hepatic infiltration occurs in the chronic, lymphoma and acute types of ATLL, but symptoms are rare. We report the case of a 32-year-old patient with acute-type ATLL and atypical severe hepatic dysfunction at diagnosis. At first, the patient presented with non-specific signs and symptoms, including severe abdominal pain, jaundice, hepatosplenomegaly, ascites and small lymphadenopathies, as well as leukocytosis, which was initially considered reactive. After excluding acute abdomen, the patient was referred to the hematologist. The diagnosis of acute-type ATLL was unexpectedly suggested by peripheral blood smear and confirmed by immunophenotyping by flow cytometry. Multiple causes of liver dysfunction, including hepatic infiltration, paraneoplastic syndrome, infectious diseases, extrahepatic compression, hemophagocytic syndrome, alcoholic liver disease and drug- induced hepatotoxicity, were taken into account. Considering the concurrence of ATLL diagnosis with liver dysfunction, the favorable clinical and biological evolution after specific hematological treatment, and the absence of imaging supporting other possibilities, we concluded that diffuse hepatic infiltration was the most probable cause. When evaluating hepatic dysfunction, considering a broad differential diagnosis is crucial. While it is uncommon, lymphoma should be included in the list of potential causes.
成人T细胞白血病/淋巴瘤(ATLL)是一种与1型人类T细胞白血病病毒相关的罕见T细胞淋巴增殖性疾病。ATLL有四种亚型:冒烟型、慢性型、淋巴瘤型和急性型。所有亚型均可出现结外受累。慢性型、淋巴瘤型和急性型ATLL可发生肝浸润,但症状罕见。我们报告一例32岁急性型ATLL患者,诊断时伴有非典型严重肝功能障碍。起初,患者表现为非特异性体征和症状,包括严重腹痛、黄疸、肝脾肿大、腹水和小淋巴结肿大,以及白细胞增多,最初被认为是反应性的。排除急腹症后,患者转诊至血液科医生处。外周血涂片意外提示急性型ATLL诊断,并通过流式细胞术免疫表型分析得以确诊。考虑了肝功能障碍的多种原因,包括肝浸润、副肿瘤综合征、传染病、肝外压迫、噬血细胞综合征、酒精性肝病和药物性肝毒性。鉴于ATLL诊断与肝功能障碍同时存在、特定血液学治疗后良好的临床和生物学演变以及缺乏支持其他可能性的影像学表现,我们得出结论,弥漫性肝浸润是最可能的原因。评估肝功能障碍时,考虑广泛的鉴别诊断至关重要。虽然罕见,但淋巴瘤应列入潜在病因清单。