Nakahashi Hirotaka, Hashimoto Yoko, Yamane Arito, Irisawa Hiroyuki, Yokohama Akihiko, Saitoh Takayuki, Hanada Hiroshi, Matsushima Takafumi, Tsukamoto Norifumi, Karasawa Masamitsu, Murakamai Hirokazu, Nojima Yoshihisa
Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine.
Rinsho Ketsueki. 2007 Aug;48(8):647-51.
A 72-year-old woman was referred to our hospital for evaluation of leukocytosis revealed by a medical examination. Her physical examination demonstrated no splenomegaly and no palpable lymph nodes. Her white cell count was 10,900/microl with atypical lymphocytosis (84.5%). Her hemoglobin concentration was 10.4 g/dl, and platelet count 151,000/microl. On peripheral blood smears, the atypical lymphocytes had a hairy cell-like appearance, and phase-contrast microscopic and transmission electron microscopic findings revealed the lymphocytes had many long surface microvilli. Flowcytometric analysis of peripheral blood lymphocytes identified expanded B-lymphocytes as having the IgG+, CD5- CD10- CD11c+ CD19+ CD20+ CD23- CD25- and CD103- cell surface phenotype. Serum electrophoresis disclosed polyclonal elevation of IgG and IgM (2620 mg/dl and 840 mg/dl, respectively). No light-chain restriction and a polyclonal VH gene rearrangement pattern indicated the polyclonal proliferation of B cells. The patient was a nonsmoker and had HLA-DR4, as in previous reports which have suggested an association between hairy B-cell lymphoproliferative disorder (HBLD) and HLA-DR4. No chromosome 3 abnormality was observed. These findings were consistent with the characteristics of HBLD, but differed in some respects from those of persistent polyclonal B-cell lymphocytosis (PPBL). Therefore, we diagnosed this patient as having HBLD.
一名72岁女性因体检发现白细胞增多而转诊至我院。体格检查未发现脾肿大及可触及的淋巴结。她的白细胞计数为10,900/微升,伴有非典型淋巴细胞增多(84.5%)。血红蛋白浓度为10.4克/分升,血小板计数为151,000/微升。外周血涂片显示,非典型淋巴细胞呈毛细胞样外观,相差显微镜和透射电子显微镜检查结果显示这些淋巴细胞有许多长的表面微绒毛。外周血淋巴细胞的流式细胞术分析确定,扩增的B淋巴细胞具有IgG+、CD5 - 、CD10 - 、CD11c+、CD19+、CD20+、CD23 - 、CD25 - 和CD103 - 的细胞表面表型。血清电泳显示IgG和IgM多克隆升高(分别为2620毫克/分升和840毫克/分升)。无轻链限制和多克隆VH基因重排模式表明B细胞多克隆增殖。该患者不吸烟,且具有HLA - DR4,如先前报道所示,HLA - DR4与毛细胞B淋巴细胞增殖性疾病(HBLD)之间存在关联。未观察到3号染色体异常。这些发现与HBLD的特征相符,但在某些方面与持续性多克隆B细胞淋巴细胞增多症(PPBL)不同。因此,我们诊断该患者患有HBLD。