Schafer A I, Rosenthal D S, Moloney W C
Am J Med Sci. 1978 May-Jun;275(3):297-308. doi: 10.1097/00000441-197805000-00007.
Long-term follow-up of 18 patients with hairy cell leukemia seen at a single institution is reported: diagnostic and therapeutic modalities are described. Although the clinical features of this disease are homogeneous, it is frequently misdiagnosed initially. Bone marrow biopsy was diagnostic in all 17 patients studied prior to splenectomy. The presence of circulating "hairy" cells is greatly variable. The cause of pancytopenia is unclear: splenic sequestration was not demonstrated by 51Cr red cell studies in three of four patients. In most patients, splenectomy resulted in amelioration of pancytopenia. No clinical features prior to splenectomy could predict response to surgery. The results of chemotherapy and splenic irradiation were variable and unpredictable. Although bone marrow infiltration persists following splenectomy, this remains the only established therapy in hairy cell leukemia and is usually followed by long, uncomplicated survival.
本文报告了在同一机构就诊的18例毛细胞白血病患者的长期随访情况，并描述了诊断和治疗方式。尽管该疾病的临床特征具有同质性，但最初常被误诊。在17例接受脾切除术之前接受研究的患者中，骨髓活检均具有诊断价值。循环“毛”细胞的存在差异很大。全血细胞减少的原因尚不清楚：4例患者中有3例的51Cr红细胞研究未证实存在脾扣押现象。在大多数患者中，脾切除术可改善全血细胞减少的状况。脾切除术之前的临床特征无法预测手术效果。化疗和脾区照射的结果各不相同且无法预测。尽管脾切除术后骨髓浸润仍然存在，但这仍是毛细胞白血病唯一已确立的治疗方法，通常随后会有较长时间的无并发症生存期。
