Yamada Yukika, Miura Miyoko, Tagari Mayu, Oshimi Kazuo, Shiragata Tomokazu, Suga Wataru, Takahashi Tatsurou, Shimizu Kazuyoshi, Ohshima Kouichi, Kajiwara Keizou
Department of Clinical Laboratory, Kushiro Central Hospital.
Department of Medicine, Kushiro Central Hospital.
Rinsho Ketsueki. 2018;59(3):281-286. doi: 10.11406/rinketsu.59.281.
A 62-year-old man presented to the hospital with thrombocytopenia, and splenomegaly was detected. His blood films prepared by natural air drying revealed medium-sized lymphocytes with unevenly distributed large and small villous projections. The cytoplasm was basophilic, nuclei were oval with clumped chromatin, and nucleoli were absent in most cells. Immune phenotypes CD19+, CD20+, CD11c+, FMC7+, IgM+, and Igκ+ were detected. TRAP stain appeared negative, IgH JH chain genes were monoclonally rearranged, and BRAF V600E mutation was not detected. On the basis of these findings, hairy cell leukemia-Japanese variant (HCL-JV) was strongly suspected. The patient was followed up for >4 years without treatment. However, because thrombocytopenia and splenomegaly gradually progressed, splenectomy was performed. Microscopic examination confirmed that the splenic white pulp was atrophic. Moreover, infiltrates comprising small-to-medium-sized atypical lymphocytes with inconspicuous nucleoli were predominantly detected in the congested red pulp. On the basis of these results and immune histochemical findings, the patient was diagnosed with splenic diffuse red pulp small B-cell lymphoma (SDRPL). Here we discussed whether the aforementioned diseases (HCL-JV and SDRPL) are the same; however, further accumulation of cases is essential to draw a definite conclusion.
一名62岁男性因血小板减少症入院，检查发现脾脏肿大。自然风干制备的血涂片显示有中等大小的淋巴细胞，其大小不一的绒毛状突起分布不均。细胞质呈嗜碱性，细胞核呈椭圆形，染色质聚集，大多数细胞无核仁。检测到免疫表型CD19+、CD20+、CD11c+、FMC7+、IgM+和Igκ+。TRAP染色呈阴性，IgH JH链基因单克隆重排，未检测到BRAF V600E突变。基于这些发现，强烈怀疑为日本变异型毛细胞白血病（HCL-JV）。该患者未经治疗随访超过4年。然而，由于血小板减少症和脾脏肿大逐渐进展，遂行脾切除术。显微镜检查证实脾白髓萎缩。此外，在充血的红髓中主要检测到由核仁不明显的中小非典型淋巴细胞组成的浸润。基于这些结果和免疫组化结果，该患者被诊断为脾弥漫性红髓小B细胞淋巴瘤（SDRPL）。在此我们讨论了上述疾病（HCL-JV和SDRPL）是否相同；然而，要得出明确结论，必须进一步积累病例。
