Zinzani P L, Lauria F, Buzzi M, Raspadori D, Gugliotta L, Bocchia M, Macchi S, Algeri R, Tura S
Istituto di Ematologia L. e A. Seràgnoli Università, Bologna, Italy.
Haematologica. 1990 Jan-Feb;75(1):54-7.
Hairy cell leukemia (HCL), a well-recognized chronic lymphoproliferative disorder, is frequently characterized by pancytopenia, monocytopenia, splenomegaly and marrow fibrosis, which typically leads to an unsuccessful bone marrow aspiration (dry tap). Patients with a high white cell count without neutropenia and/or monocytopenia, with an aspirable and hypercellular marrow, splenomegaly and neoplastic cells with hairy cell features have been recently recognized and classified as HCL variants. We report here the clinical, hematological and immunological features of 7 such cases. All patients presented splenomegaly with a high leukocyte count; 2 were anemic and only 1 thrombocytopenic. Five patients were treated with alpha-Interferon (alpha-IFN) but 4 failed to achieve any significant response; two of these were subsequently splenectomized and successfully treated with Chlorambucil. Splenectomy, followed by Chlorambucil, was performed at diagnosis in the remaining 2 cases, both of which achieved a partial response and are alive and well. Six out of the 7 patients are still alive. The recognition of these peculiar patients is also important because they most often do not respond to alpha-IFN, while splenectomy, followed by Chlorambucil, may be a reasonable therapeutic option for them.
毛细胞白血病（HCL）是一种公认的慢性淋巴细胞增殖性疾病，其特征通常为全血细胞减少、单核细胞减少、脾肿大和骨髓纤维化，这通常导致骨髓穿刺失败（干抽）。近期，白细胞计数高但无中性粒细胞减少和/或单核细胞减少、骨髓可穿刺且细胞增多、脾肿大以及具有毛细胞特征的肿瘤细胞的患者已得到确认并被归类为HCL变异型。我们在此报告7例此类病例的临床、血液学和免疫学特征。所有患者均有脾肿大及白细胞计数高的表现；2例贫血，仅1例血小板减少。5例患者接受了α干扰素（α-IFN）治疗，但4例未取得任何显著疗效；其中2例随后接受了脾切除术，并成功接受苯丁酸氮芥治疗。其余2例在诊断时即接受了脾切除术，随后使用苯丁酸氮芥治疗，均取得部分缓解，目前仍存活且状况良好。7例患者中有6例仍存活。认识到这些特殊患者也很重要，因为他们通常对α-IFN无反应，而脾切除术后再使用苯丁酸氮芥可能是适合他们的合理治疗选择。
