Matsuda Kensuke, Matsumoto Yosuke, Yoshida Mihoko, Shimura Kazuho, Kaneko Hiroto, Inaba Tohru, Horiike Shigeo, Kuroda Junya, Taniwaki Masafumi
Department of Hematology and Oncology, Tokyo University Hospital, Japan.
Departments of Hematology and Laboratory Medicine, Aiseikai Yamashina Hospital, Japan.
Mediterr J Hematol Infect Dis. 2017 Sep 1;9(1):e2017054. doi: 10.4084/MJHID.2017.054. eCollection 2017.
Hairy B-cell lymphoproliferative disorder (HBLD) is one of chronic polyclonal B-cell lymphocytosis. We report a 47-year-old female Japanese patient diagnosed as having HBLD based on lymphocytosis with hairy cell appearance and characteristic phenotypes including CD11c+ and without B-cell monoclonality. She was a non-smoker and possessed HLA-DR4. She has been closely followed up without treatment and lymphoma development for over five years. Although this disease is quite rare and has been reported, to our knowledge, in only 13 Japanese cases, an accurate diagnosis, particularly differential diagnosis from persistent polyclonal B-cell lymphocytosis or hairy cell leukemia-Japanese variant is essential for the prevention of unnecessary treatments.
毛细胞B淋巴细胞增殖性疾病（HBLD）是慢性多克隆B淋巴细胞增多症之一。我们报告了一名47岁的日本女性患者，根据淋巴细胞增多伴毛细胞外观以及包括CD11c+在内的特征性表型且无B细胞单克隆性，被诊断为HBLD。她不吸烟，拥有HLA-DR4。她在未接受治疗的情况下接受了超过五年的密切随访，未发生淋巴瘤。尽管这种疾病非常罕见，据我们所知，仅在13例日本病例中有报道，但准确诊断，尤其是与持续性多克隆B淋巴细胞增多症或日本变异型毛细胞白血病进行鉴别诊断，对于避免不必要的治疗至关重要。
