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Fludarabine 是一种新型腺嘌呤核苷类药物。作者首次报导了用其治疗3例HCL。计男2例,女1例。年龄分别为33、40及69岁。前2例为典型的HCL,例3为其变异型。后者的诊断系依据在光镜与电镜下于骨髓及脾脏中发现非典型淋巴样细胞,该细胞较典型之毛细胞为大,抗酒石酸磷酸酶(TRAP)染色阳性者<10%。电镜下可见为数众多的微绒毛状突起和中等量的细胞器,并可见核糖体板层复合体,核不规则,伴有大而显著之核仁。该类细胞的免疫学表型呈CD25阴性,而CD11及CD22均阳性。3例病人接受Fludarabine 治疗前均曾用α-干扰素,例2、3曾经切脾治疗,例2还用过强的松及2′-去氧助间型霉素(DCF)。
Fludarabine is a new adenine nucleoside drug. The authors reported for the first time 3 cases of HCL treated with it. Male 2 cases, female 1 case. The ages are 33, 40 and 69 respectively. The first two cases of typical HCL, 3 cases of its variant. The latter diagnosis was based on the finding of atypical lymphoid cells in the bone marrow and spleen under light and electron microscopy. The cells were larger than the typical hair cells and were <10% positive for tartaric acid phosphatase (TRAP) staining. A large number of microvilli-like processes and a moderate number of organelles were seen under the electron microscope. Ribosome lamellar complexes with irregular nuclei and large and prominent nucleoli were observed. The immunological phenotype of these cells was CD25 negative, while CD11 and CD22 were positive. All 3 patients received a-interferon before treatment with Fludarabine. Cases 2 and 3 were treated with splenectomy. In case 2, prednisone and 2’-deoxycoformycin (DCF) were also used.