Ho A D, Thaler J, Willemze R, Lauria F, Derossi G, Kuse R, Stryckmans P, Blanc C M, Cataldo F, McVie G
Bone Marrow Transplant. 1989 Jan;4 Suppl 1:60-2.
Knowledge of the vital role of adenosine deaminase in lymphatic tissues has led to the development of enzyme inhibitors for treatment of lymphoid neoplasms. Deoxycoformycin is a potent ADA inhibitor and has been shown to be active in acute lymphoblastic leukemia at high doses but associated with unpredictable toxicity. In indolent lymphocytic leukemia or lymphoma with low ADA concentrations, this drug is effective at low doses with mild toxicity. The on-going EORTC trial shows that pentostatin is highly effective in hairy cell leukemia and can achieve durable complete remissions even if interferon alpha has failed. It will probably play an important role in the treatment of prolymphocytic leukemia, T- and B-cell chronic lymphocytic leukemia and Sézary syndrome.
对腺苷脱氨酶在淋巴组织中重要作用的认识促使了用于治疗淋巴肿瘤的酶抑制剂的研发。脱氧助间型霉素是一种强效的腺苷脱氨酶抑制剂，已表明高剂量时对急性淋巴细胞白血病有活性，但伴有不可预测的毒性。在腺苷脱氨酶浓度低的惰性淋巴细胞白血病或淋巴瘤中，该药物低剂量时有效且毒性轻微。正在进行的欧洲癌症研究与治疗组织（EORTC）试验表明，喷司他丁在毛细胞白血病中非常有效，即使α干扰素治疗失败也能实现持久的完全缓解。它可能在原淋巴细胞白血病、T细胞和B细胞慢性淋巴细胞白血病以及Sezary综合征的治疗中发挥重要作用。
