Katayama I
Department of Pathology, Saitama Medical School, Japan.
Hematol Oncol Clin North Am. 1988 Dec;2(4):585-602.
HCL is a well-recognized entity among the lymphoproliferative disorders. With better appreciation of the wide variability in its clinical and hematologic manifestations, some authors have proposed several subtypes of HCL such as leukopenic and nonleukopenic subtypes and subtypes with and without massive splenomegaly. As opposed to such a clinical and hematologic variability, the pathology of HCL in the spleen and bone marrow is consistent and highly characteristic. Since the spleen becomes available for pathologic examination only after therapeutic splenectomy, the bone marrow pathology often plays the most important role in the differential diagnosis of HCL. It is characterized by focal or diffuse mononuclear cell infiltration with a wide spacing between individual nuclei in most patients and by a severely hypocellular marrow with individual hairy cells infiltrating between the marrow fat cells in the remaining minority of patients. The bone marrow biopsy also serves as one of the criteria in selecting the therapeutic modality as well as monitoring the therapeutic effect. Further, new insights into the pathogenesis of HCL are emerging from recent studies of the bone marrow microenvironment.
毛细胞白血病(HCL)是淋巴增殖性疾病中一种广为人知的疾病实体。随着对其临床和血液学表现的广泛变异性有了更深入的认识,一些作者提出了HCL的几种亚型,如白细胞减少型和非白细胞减少型亚型,以及有和无巨脾的亚型。与这种临床和血液学变异性相反,HCL在脾脏和骨髓中的病理学表现是一致且极具特征性的。由于脾脏只有在治疗性脾切除术后才能进行病理检查,因此骨髓病理学在HCL的鉴别诊断中往往起着最重要的作用。其特征是在大多数患者中表现为局灶性或弥漫性单核细胞浸润,单个核之间间距较宽,而在其余少数患者中表现为严重细胞减少的骨髓,单个毛细胞浸润于骨髓脂肪细胞之间。骨髓活检也是选择治疗方式以及监测治疗效果的标准之一。此外,最近对骨髓微环境的研究为HCL的发病机制带来了新的见解。