Jacobs P, Knottenbelt E, Jacobs L
S Afr Med J. 1982 Oct 9;62(16):559-61.
Pancytopenia with massive splenomegaly, minimal lymphadenopathy and circulating atypical lymphoid cells with cytoplasmic projections characterize the lymphoproliferative disorder known as hairy cell leukemia, or leukaemic reticulo-endotheliosis. Many of the features of hairy cell leukaemia are shared with a malignant lymphoma of the B-cell type, from which it can be distinguished on a number of criteria, including histological examination of bone marrow, lymph nodes, liver and spleen. Recognition that this syndrome is not homogeneous is important, since in the B-cell tumour the clinical course is progressive, requiring chemotherapy which is usually effective; the disease is indolent in leukaemic reticulo-endotheliosis and drug treatment may actually shorten survival. The patient presented illustrates the ease with which this diagnostic error can arise, and the features separating these two unusual entities are reviewed.
全血细胞减少伴巨脾、轻度淋巴结病以及循环中出现带有胞质突起的非典型淋巴细胞是毛细胞白血病（又称白血病性网状内皮细胞增生症）这种淋巴增殖性疾病的特征。毛细胞白血病的许多特征与B细胞型恶性淋巴瘤相同，但可根据一些标准将二者区分开来，包括对骨髓、淋巴结、肝脏和脾脏进行组织学检查。认识到这种综合征并非单一类型很重要，因为在B细胞肿瘤中，临床病程呈进行性，需要化疗且化疗通常有效；而白血病性网状内皮细胞增生症的病情发展缓慢，药物治疗实际上可能会缩短生存期。本文所介绍的患者说明了这种诊断错误是多么容易出现，并对区分这两种不同疾病实体的特征进行了综述。
