Bagilet D, Diab M, Aristiquí R, Magaró J, Sívori O, Naves A
Servicio de Clínica Médica, Sanatorio Delta, Rosario, Argentina.
Medicina (B Aires). 1990;50(6):548-52.
Angioimmunoblastic lymphadenopathy (AILD), first discovered in the 70's, is an infrequent disease which generally proves fatal within a short time. It is characterized by lymphadenopathies, hepatosplenomegaly, fever and rash. The most frequent laboratory findings are: anemia, leukocytosis with lymphopenia and non specific hypergammaglobulinemia. In spite of being considered a non malignant disease, it produces important immunity disorders which predispose the patient to serious infections, frequently fatal. In the course of time patients are likely to develop malignant lymphomas or other types of tumors. We describe a ganglionar proliferation and general symptoms in a patient who had been diagnosed as AILD by ganglionar biopsy. She was treated with corticosteroids during 8 months after which she had a complete recovery. Three months later the patient was readmitted with enterrorhagias and clear deterioration of her general condition. Tests showed the existence of a colon tumor and absence of adenomegaly in the areas previously affected by AILD. A colon surgery was carried out and a specimen examined. The anatomopathologic examination confirmed the existence of an immunoblastic lymphoma infiltrating the regional ganglionar area. Colon lymphomas constitute only 4% of all colon tumors; they are related to chronic gastrointestinal disease such as Crohn disease, ulcerative colitis, malabsorption syndromes, tumors and others. We conclude that in this patient AILD and prednisone administration constituted favoring factors for the development of an extranodal lymphoma.
血管免疫母细胞性淋巴结病(AILD)于20世纪70年代首次被发现,是一种罕见疾病,通常在短时间内致命。其特征为淋巴结病、肝脾肿大、发热和皮疹。最常见的实验室检查结果为:贫血、白细胞增多伴淋巴细胞减少以及非特异性高丙种球蛋白血症。尽管被认为是非恶性疾病,但它会引发重要的免疫紊乱,使患者易患严重感染,且往往致命。随着时间推移,患者可能会发展为恶性淋巴瘤或其他类型的肿瘤。我们描述了一名经淋巴结活检诊断为AILD的患者的淋巴结增生和全身症状。她接受了8个月的皮质类固醇治疗,之后完全康复。三个月后,患者因便血和全身状况明显恶化再次入院。检查显示存在结肠肿瘤,且先前受AILD影响的区域无淋巴结肿大。进行了结肠手术并检查了标本。解剖病理学检查证实存在浸润局部淋巴结区域的免疫母细胞性淋巴瘤。结肠淋巴瘤仅占所有结肠肿瘤的4%;它们与慢性胃肠道疾病如克罗恩病、溃疡性结肠炎、吸收不良综合征、肿瘤等有关。我们得出结论,在该患者中,AILD和泼尼松治疗是结外淋巴瘤发生的促成因素。