Shibayama H, Machii T, Yamaguchi M, Tokumine Y, Kitani T
Department of Hematology and Oncology, Osaka University Medical School, Japan.
Int J Hematol. 1996 Oct;64(3-4):287-90. doi: 10.1016/0925-5710(96)00497-5.
Hairy cell leukemia (HCL) is an uncommon type of chronic B cell leukemia mainly affecting middle-aged adults. HCL presenting with pancytopenia is rare in Japan and a distinct subtype of HCL termed HCL-Japanese variant is predominantly seen. We describe a HCL patient with unusual presentation. The patient was a 26-year-old male, such early onset of HCL being quite rare. The patient showed leukocytosis with many circulating hairy cells and cellular bone marrow. These findings were preferentially seen in HCL-Japanese variant, but, cytomorphologic, cytochemical and immunophenotypical studies on the pathologic cells were consistent with those of typical HCL seen in Western countries. Interferon-alpha therapy was very effective in this case. Differentiation of the subtype of HCL appears to be important for the choice of the treatment. The cytological findings were useful for the differential diagnosis of HCL presenting with leukocytosis.
毛细胞白血病(HCL)是一种不常见的慢性B细胞白血病,主要影响中年成年人。在日本,以全血细胞减少为表现的HCL较为罕见,主要可见一种称为HCL-日本变异型的独特HCL亚型。我们描述了一名表现不寻常的HCL患者。该患者为一名26岁男性,HCL如此早发相当罕见。患者表现为白细胞增多,有许多循环毛细胞和细胞性骨髓。这些发现多见于HCL-日本变异型,但对病理细胞进行的细胞形态学、细胞化学和免疫表型研究与西方国家所见典型HCL的研究结果一致。α干扰素治疗在该病例中非常有效。HCL亚型的区分似乎对治疗选择很重要。细胞学发现有助于鉴别以白细胞增多为表现的HCL。