Dunn-Walters D K, Boursier L, Spencer J, Isaacson P G
Department of Histopathology, UMDS St Thomas' Campus, UCLMS, London, United Kingdom.
Hum Pathol. 1998 Jun;29(6):585-93. doi: 10.1016/s0046-8177(98)80007-5.
Splenic marginal zone lymphoma (SMZL) is a low-grade primary splenic B cell lymphoma, originally thought to be related to splenic marginal zone B cells. Later studies showed that SMZL sometimes may be accompanied by villous lymphocytes in the peripheral blood, a condition previously characterized as splenic lymphoma with villous lymphocytes (SLVL). The relationship between SMZL and splenic marginal zone B cells has recently been called into question. We report four further cases of SMZL, two of which were associated with villous lymphocytes in the peripheral blood. In addition to immunophenotypical analysis, we have studied the IgV(H) genes in each case, because the extent and patterns of their mutation can indicate the normal B cell counterpart of lymphomas. The IgV(H) genes in the four cases of SMZL studied are mutated, which is consistent with their origin from postfollicular marginal zone B cells. Evidence of ongoing mutation was also observed. This contrasts with a study showing that blood-borne tumor cells in SLVL show no sign of ongoing mutation. It is possible that the ongoing mutations in the cases studied here are acquired in a splenic microenvironment, such as that found in the follicle center.
脾边缘区淋巴瘤（SMZL）是一种低度恶性的原发性脾B细胞淋巴瘤，最初被认为与脾边缘区B细胞有关。后来的研究表明，SMZL有时可能伴有外周血中的绒毛状淋巴细胞，这种情况以前被称为伴有绒毛状淋巴细胞的脾淋巴瘤（SLVL）。最近，SMZL与脾边缘区B细胞之间的关系受到了质疑。我们报告了另外4例SMZL病例，其中2例伴有外周血中的绒毛状淋巴细胞。除了免疫表型分析外，我们还研究了每个病例中的IgV(H)基因，因为其突变的程度和模式可以表明淋巴瘤的正常B细胞对应物。所研究的4例SMZL病例中的IgV(H)基因发生了突变，这与它们起源于滤泡后边缘区B细胞一致。还观察到了持续突变的证据。这与一项研究形成对比，该研究表明SLVL中的血源性肿瘤细胞没有持续突变的迹象。此处所研究病例中的持续突变可能是在脾微环境中获得的，例如在滤泡中心发现的微环境。
