2' DEOXYCOFORMYCIN IN IFN-RESISTANT HCL OR T GAMMA LYMPHOPROLIFERATIVE DISORDER

脱氧考福霉素治疗毛细胞病的研究 白血病或T-γ淋巴增生性疾病被写成 抢救方案，用于治疗这些患者，如果他们没有反应 在其他正在进行的CRB方案中使用低剂量阿尔法干扰素。 已知脱氧考福霉素在毛细胞中具有显著活性。 白血病，据报道可产生高完全应答率 以前未接受治疗的患者的发病率，并导致在 先前干扰素治疗失败的患者。它还没有得到证实 对T-γ淋巴增生性疾病的疗效。七 在这项研究中，有4名患者患有毛细胞 白血病和三名T-γ淋巴增生性疾病患者。全 四例毛细胞性白血病患者对 干扰素α-2a治疗。这四名患者中有三名患者有 对DCF治疗有显著的部分反应，目前已有 骨髓中仅有极少量残留毛细胞。 他们已经将外周血细胞计数正常化，所有人都 停用脱氧考福霉素至少六个月； 所有患者在所有治疗后均保持稳定的部分缓解状态。一 一例毛细胞白血病患者在第二剂DCF治疗后死亡 由于严重的血小板减少和对人类白细胞抗原- 匹配的血小板输注和广泛的胃肠道出血。在… 尸检发现死者的数量已大幅减少 这个病人骨髓中的毛细胞。三名患者中的 T-γ淋巴增生性疾病，1例完全 应答者，两名患者为无应答者。一个完整的人 应答者是一名患有严重粒细胞减少症的老年妇女。我们 结论：脱氧康福霉素具有抗干扰素活性 毛细胞性白血病和部分T-γ患者 淋巴增生性疾病。治疗与.相关 可以接受的毒性，但会加剧与以下疾病相关的全血细胞减少症 这两种疾病中的一种。

This study of deoxycoformycin therapy in patients with hairy cell leukemia or T-gamma lymphoproliferative disorder was written as a salvage protocol to treat these patients if they failed to respond to low-dose alfa interferon on other ongoing CRB protocols. Deoxycoformycin is known to have significant activity in hairy cell leukemia and has been reported to produce high complete response rates in previously untreated patients and to lead to responses in patients failing prior interferon therapy. It has unproven efficacy in the T-gamma lymphoproliferative disorder. Seven patients have been admitted to this study, four with hairy cell leukemia and three with T-gamma lymphoproliferative disorder. All four patients with hairy cell leukemia were refractory to interferon alfa-2a therapy. Three of these four patients had a substantial partial response to dCF therapy and have at this moment only minimal residual hairy cell infiltrate in the bone marrow. They have normalized their peripheral blood counts, and all have been off of deoxycoformycin therapy for a minimum of six months; all remain in stable partial remission off all therapy. One patient with hairy cell leukemia died after the second dose of dCF because of profound thrombocytopenia and refractoriness to HLA- matched platelet transfusions and extensive GI bleeding. At autopsy there had been a substantial reduction in the number of hairy cells in this patient's bone marrow. Of three patients with T-gamma lymphoproliferative disorder, one patient was a complete responder, and two patients were nonresponders. The one complete responder was an elderly woman with severe granulocytopenia. We conclude that deoxycoformycin has activity in interferon-resistant hairy cell leukemia and in some patients with T-gamma lymphoproliferative disorder. Treatment is.associated with acceptable toxicity but can worsen the pancytopenia associated with either of these diseases.