DiGiuseppe J A, Hartmann D P, Freter C, Cossman J, Mann R B
Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA.
Mod Pathol. 1997 Jan;10(1):33-7.
Intravascular lymphomatosis (IVL) is an extremely rare variant of aggressive non-Hodgkin's lymphoma characterized by confinement of neoplastic lymphocytes within vascular spaces. Although IVL is potentially curable with combination chemotherapy, diagnosis is often delayed, in part owing to the negative bone marrow biopsy specimens that are typical of this disorder. We hypothesized that use of a more sensitive method of analysis might identify small clonal B-cell populations in histologically negative bone marrow biopsy specimens from patients with IVL. With use of a recently described assay for immunoglobulin heavy chain gene rearrangement based on the polymerase chain reaction, we demonstrated clonal B-cell populations in histologically negative marrow specimens from five (100%) of five patients with IVL. None of these specimens demonstrated molecular evidence of the t(14;18) associated with follicular lymphoma, providing no evidence for a common derivation of IVL and follicular lymphoma. In summary, molecular analysis of routine bone marrow biopsy sepcimens from patients in whom the diagnosis of IVL is entertained may facilitate prompt recognition of a lympho-proliferative disorder and thereby permit timely therapeutic intervention. Moreover, these findings suggest that despite histologically negative staging bone marrow biopsy specimens, IVL typically disseminates early in its course, thus arguing against the use of localized therapy in this disorder.
血管内淋巴瘤（IVL）是侵袭性非霍奇金淋巴瘤的一种极其罕见的变体，其特征是肿瘤性淋巴细胞局限于血管腔内。尽管IVL有可能通过联合化疗治愈，但诊断往往延迟，部分原因是该疾病典型的骨髓活检标本呈阴性。我们推测，使用更敏感的分析方法可能会在IVL患者组织学阴性的骨髓活检标本中识别出小的克隆性B细胞群体。通过使用最近描述的基于聚合酶链反应的免疫球蛋白重链基因重排检测方法，我们在5例IVL患者中5例（100%）组织学阴性的骨髓标本中证实了克隆性B细胞群体。这些标本均未显示与滤泡性淋巴瘤相关的t(14;18)的分子证据，这表明IVL和滤泡性淋巴瘤没有共同的起源。总之，对疑似IVL患者的常规骨髓活检标本进行分子分析可能有助于迅速识别淋巴细胞增殖性疾病，从而及时进行治疗干预。此外，这些发现表明，尽管分期骨髓活检标本组织学呈阴性，但IVL通常在病程早期就发生播散，因此不主张对该疾病采用局部治疗。
