Nagasaka T, Nakamura S, Medeiros L J, Juco J, Lai R
Division of Pathology, Clinical Laboratory, Nagoya University Hospital, Japan.
Mod Pathol. 2000 Oct;13(10):1143-9. doi: 10.1038/modpathol.3880211.
Non-Hodgkin's lymphomas uncommonly present as bone lesions. Most of these tumors are diffuse large B-cell lymphomas. Anaplastic large cell lymphoma (ALCL) presented as bone lesions is exceedingly rare. In this study, we describe six cases of ALCL that presented as solitary or multiple bone lesions. The average patient age was 33 years (range, 4 to 63 years) and the male to female ratio was 2:1. Fever and localized bone pain were the most frequent presenting symptoms. Radiologic examinations revealed osteolytic lesions in all cases, with three (50%) being multiple lesions and five (83%) involving the axial bones. All patients were initially assessed to have only bone involvement. Staging studies revealed mild cervical lymphadenopathy in one patient and no evidence of extraskeletal disease in the other five patients. Histologically, there was diffuse infiltration of one or more bones by large pleomorphic lymphoma cells. Immunohistochemical studies showed all six neoplasms were positive for CD30, EMA, and granzyme B. One case was of T-cell lineage, positive for CD3. One case was positive for the T-cell-associated antigen CD4. The remaining four cases were of null-cell type. In-situ hybridization for EBV was performed in five cases; all were negative. Despite the relatively low International Prognostic Index (IPI) of these patients (mean, 1.67; range, 1 to 3), the overall prognosis was relatively poor: three of six died of disease within 2 years of diagnosis, and two of six were alive with evidence of disease (follow-up, 6 mo to 2 years). Thus, compared to their nodal counterparts, ALCLs that present as bone lesions are distinguished by their uniform expression of EMA and granzyme B, and a relatively poor clinical outcome. Our results also suggest that ALK-1 expression in this clinical setting is not a favorable prognostic indicator.
非霍奇金淋巴瘤很少表现为骨病变。这些肿瘤大多为弥漫性大B细胞淋巴瘤。表现为骨病变的间变性大细胞淋巴瘤（ALCL）极为罕见。在本研究中，我们描述了6例表现为孤立性或多发性骨病变的ALCL。患者平均年龄为33岁（范围4至63岁），男女比例为2:1。发热和局部骨痛是最常见的首发症状。放射学检查显示所有病例均有溶骨性病变，其中3例（50%）为多发性病变，5例（83%）累及中轴骨。所有患者最初评估仅累及骨骼。分期检查显示1例患者有轻度颈部淋巴结肿大，其他5例患者无骨骼外疾病证据。组织学上，可见一个或多个骨骼被大的多形性淋巴瘤细胞弥漫浸润。免疫组化研究显示所有6例肿瘤CD30、EMA和颗粒酶B均为阳性。1例为T细胞谱系，CD3阳性。1例T细胞相关抗原CD4阳性。其余4例为空细胞型。5例进行了EBV原位杂交；均为阴性。尽管这些患者的国际预后指数（IPI）相对较低（平均1.67；范围1至3），但总体预后相对较差：6例中有3例在诊断后2年内死于疾病，6例中有2例仍有疾病证据存活（随访6个月至2年）。因此，与淋巴结型ALCL相比，表现为骨病变的ALCL的特点是EMA和颗粒酶B表达一致，临床结局相对较差。我们的结果还表明，在这种临床情况下，ALK-1表达不是一个良好的预后指标。
