Division of Hematology-Oncology, Chang Gung Memorial Hospital, Taipei, Taiwan, ROC.
Acta Haematol. 2011;126(3):186-93. doi: 10.1159/000328887. Epub 2011 Aug 13.
Classical hairy cell leukemia (HCL-C) and its variant (HCL-V) are rare chronic B-cell lymphoproliferative disorders. Only a few reports in Chinese patients are available.
We retrospectively reviewed 16 patients with HCL-C and HCL-V in Taiwan over a 17-year period.
Eight were HCL-C and 8 were HCL-V. All HCL accounted for 0.7% of all adult leukemias. Compared to HCL-V, HCL-C was characterized by profound leukopenia, monocytopenia, thrombocytopenia and fewer circulating hairy cells. One HCL-C and 2 HCL-V patients had second malignancies. Seven HCL-C patients achieved hematological remission after splenectomy (n = 1) or 2-chlorodeoxyadenosine (n = 6). Of the 8 HCL-V patients, 6 received splenic irradiation. Only one achieved complete remission and another had partial remission; relapse or disease progression was noted 13.4 or 25.7 months later, respectively. Two of three HCL-V patients who underwent splenectomy had stable disease. All patients with HCL-C were alive while 3 with HCL-V expired. Compared to HCL-C, HCL-V had a significantly shorter leukemia-free survival.
A relatively higher proportion of HCL-V in all HCL comparing to Westerners is observed. Second malignancies are common. With an inferior outcome and dismal response to most treatment, enrollment in a clinical trial should be considered for HCL-V.
经典型毛细胞白血病（HCL-C）及其变异型（HCL-V）是罕见的慢性 B 细胞淋巴增生性疾病。仅有少数关于中国患者的报告。
我们回顾性分析了台湾 17 年间的 16 例 HCL-C 和 HCL-V 患者。
8 例为 HCL-C，8 例为 HCL-V。所有 HCL 占成人白血病的 0.7%。与 HCL-V 相比，HCL-C 表现为严重的白细胞减少、单核细胞减少、血小板减少和较少的循环毛细胞。1 例 HCL-C 和 2 例 HCL-V 患者患有第二恶性肿瘤。7 例 HCL-C 患者在脾切除术后（n=1）或 2-氯脱氧腺苷（n=6）后达到血液学缓解。8 例 HCL-V 患者中，6 例接受了脾照射。仅 1 例达到完全缓解，另 1 例部分缓解；分别在 13.4 和 25.7 个月后复发或疾病进展。行脾切除术的 3 例 HCL-V 患者中，2 例疾病稳定。所有 HCL-C 患者均存活，而 3 例 HCL-V 患者死亡。与 HCL-C 相比，HCL-V 的无白血病生存期明显缩短。
与西方人相比，所有 HCL 中 HCL-V 的比例相对较高。第二恶性肿瘤很常见。HCL-V 对大多数治疗反应不佳，预后较差，应考虑入组临床试验。
