Chang Sheng-Tsung, Hsieh Yen-Chuan, Lu Yi-Hsuan, Tzeng Ching-Cherng, Lin Ching-Nan, Chuang Shih-Sung
Department of Pathology, Chi-Mei Medical Center, Tainan, Taiwan.
Pathol Res Pract. 2008;204(1):23-6. doi: 10.1016/j.prp.2007.08.008. Epub 2007 Oct 29.
There are three clinicopathological entities of marginal zone lymphoma (MZL), including extranodal or mucosa-associated lymphoid tissue (MALT) lymphoma and MZL of nodal (NMZL) or splenic (SMZL) type. Of these, leukemic presentation, usually as small or villous lymphocytes, is more common in SMZL, while leukemic change in NMZL is rare, and the morphology has not been characterized. We present a stage 4 MZL involving lymph node, spleen, and bone marrow with two relapses after chemotherapy. The leukemic cells at the second relapse revealed irregular nuclear contours with multilobated nuclei (so-called flower cells or floral cells) mimicking the neoplastic cells in adult T-cell leukemia/lymphoma (ATLL). The absence of leukemic change and splenic hilar lymphadenopathy at initial presentation, expression of IgD by tumor cells, and cytogenetic changes of +7 suggested that this tumor might be a NMZL. Although the cytomorphologic features of floral leukemic cells might suggest ATLL, thorough clinical and laboratory workup helped to reach a correct diagnosis. Our findings broaden the cytological spectra of leukemic cells in MZL and illustrate the importance of immunophenotyping.
边缘区淋巴瘤(MZL)有三种临床病理类型,包括结外或黏膜相关淋巴组织(MALT)淋巴瘤以及结内(NMZL)或脾(SMZL)型MZL。其中,白血病表现通常为小淋巴细胞或绒毛状淋巴细胞,在SMZL中更为常见,而NMZL中的白血病改变罕见,且其形态尚未明确。我们报告1例4期MZL,累及淋巴结、脾脏和骨髓,化疗后复发2次。第二次复发时的白血病细胞显示核轮廓不规则,有多个分叶核(所谓的花细胞或花瓣样细胞),类似于成人T细胞白血病/淋巴瘤(ATLL)中的肿瘤细胞。初诊时无白血病改变及脾门淋巴结肿大、肿瘤细胞表达IgD以及+7的细胞遗传学改变提示该肿瘤可能为NMZL。尽管花细胞样白血病细胞的细胞形态学特征可能提示ATLL,但全面的临床和实验室检查有助于做出正确诊断。我们的发现拓宽了MZL中白血病细胞的细胞学谱,并说明了免疫表型分析的重要性。