Schwartz R A, Rezuke W N, Cartun R W, Hasson J
Department of Pathology, University of Connecticut School of Medicine, USA.
Conn Med. 1998 Nov;62(11):643-7.
We describe a case of a 73-year-old male with a rare T-cell lymphoma that presented deceptively as progressive hepatic failure with fever, weight loss, pancytopenia, mental confusion, splenomegaly, and no lymphadenopathy. An alcoholic history supported the diagnosis of cirrhosis, but a liver biopsy was not performed. A bone marrow biopsy was considered unremarkable. Death occurred after a course of four months. Postmortem examination showed hepatic, splenic, lymph node, and marrow infiltration by characteristically sparse, isolated, bizarre, medium-to-large sized neoplastic cells with extensive hepatic centrilobular necrosis, steatosis, and predominant splenic involvement. Immunohistochemical markers indicated a T-cell lymphoma consistent with either an alpha/beta peripheral T-cell lymphoma or a gamma/delta lymphoma. Definitive immunotyping was not available. However, the pathologic features are most consistent with a gamma/delta T-cell lymphoma. This case is an example of a rare, rapidly progressive lymphoma, which is a recognized clinical entity, easily missed, and treatable. Its diagnostic consideration must be explicitly communicated to pathologists, because the isolated or sparse tumor cells in a lymph node, liver, or bone marrow biopsy may easily be mistaken for variants of megakaryocytes or histiocytes.
我们描述了一例73岁男性患者，患有罕见的T细胞淋巴瘤，其表现具有欺骗性，为进行性肝衰竭，伴有发热、体重减轻、全血细胞减少、精神错乱、脾肿大且无淋巴结病。有酗酒史支持肝硬化的诊断，但未进行肝活检。骨髓活检结果无明显异常。病程四个月后患者死亡。尸检显示肝脏、脾脏、淋巴结和骨髓有特征性稀疏、孤立、怪异的中到大尺寸肿瘤细胞浸润，伴有广泛的肝小叶中心坏死、脂肪变性，且脾脏受累为主。免疫组化标记显示为T细胞淋巴瘤，符合α/β外周T细胞淋巴瘤或γ/δ淋巴瘤。无法进行明确的免疫分型。然而，病理特征最符合γ/δ T细胞淋巴瘤。该病例是一种罕见的、快速进展的淋巴瘤的实例，这是一种公认的临床实体，容易漏诊但可治疗。必须明确告知病理学家对其进行诊断性考虑，因为在淋巴结、肝脏或骨髓活检中孤立或稀疏的肿瘤细胞可能很容易被误认为巨核细胞或组织细胞的变体。
