de Campos Fernando Peixoto Ferraz, Zerbini Maria Claudia Nogueira, Felipe-Silva Aloisio, Simões Angélica Braz, Lovisolo Silvana Maria, da Fonseca Leonardo Gomes, Laborda Lorena
Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Autops Case Rep. 2014 Mar 31;4(1):21-27. doi: 10.4322/acr.2014.004. eCollection 2014 Jan-Mar.
Anaplastic large cell lymphoma (ALCL), a well-recognized entity, presents a varied clinical picture and epidemiological characteristics associated with the expression of the anaplastic lymphoma kinase (ALK) protein. When classic symptoms are present (weight loss, fever, and night sweats) and combine with enlarged and easily accessible peripheral lymph nodes, diagnosis is not that difficult. But when the clinical presentation is nonspecific, a tough diagnostic task is required. HIV infection is highly associated with neoplastic disorders-mainly with those of hematological origin. However, ALCL is exceptionally associated with HIV infection, and the few reported cases are ALK ALCL. The authors report two cases of ALK ALCL with the unusual clinical presentation: one is associated with the HIV infection and the other presents as a fever of unknown origin (FUO) without peripheral lymphadenopathy. The latter was autopsied and was characterized by nodal and extra nodal involvement. The authors call attention to the plurality of clinical presentation of this group of lymphomas, and the early indication of bone marrow examination in cases of an FUO with elevated hepatic enzymes and lactic dehydrogenase.
间变性大细胞淋巴瘤(ALCL)是一种已被充分认识的疾病,其临床表现多样,流行病学特征与间变性淋巴瘤激酶(ALK)蛋白的表达相关。当出现典型症状(体重减轻、发热和盗汗)并伴有易于触及的外周淋巴结肿大时,诊断并不困难。但当临床表现不具有特异性时,则需要进行艰巨的诊断工作。HIV感染与肿瘤性疾病高度相关——主要与血液系统来源的疾病相关。然而,ALCL与HIV感染的关联极为罕见,且报道的少数病例均为ALK阳性的ALCL。作者报告了两例具有不寻常临床表现的ALK阳性的ALCL病例:一例与HIV感染相关,另一例表现为不明原因发热(FUO)且无外周淋巴结病。后者进行了尸检,其特征为淋巴结和结外受累。作者提醒注意这类淋巴瘤临床表现的多样性,以及在不明原因发热且肝酶和乳酸脱氢酶升高的病例中早期进行骨髓检查的指征。