Groopman J E
Semin Oncol. 1985 Dec;12(4 Suppl 5):30-4.
Hairy-cell leukemia is a well-characterized, pathologic disorder. The majority of cases are B lymphocyte in origin with circulating hairy cells, splenomegaly, and, not infrequently, pancytopenia included in the clinical findings. Observation, splenectomy, glucocorticoids (ie, for vasculitic manifestations), and alkylating agents are the currently recommended treatment for this disease. Recombinant interferon alpha may become the treatment of choice for individuals with progressive disease after splenectomy. An understanding of the mechanism of action of recombinant interferon alpha may shed light on the pathways of interferons in general, as well as provide insights into the pathogenesis of this particular proliferative disorder.
毛细胞白血病是一种特征明确的病理紊乱疾病。大多数病例起源于B淋巴细胞,临床表现包括循环中的毛细胞、脾肿大,且全血细胞减少也并不罕见。观察、脾切除术、糖皮质激素(如用于血管炎表现)和烷化剂是目前针对该疾病推荐的治疗方法。重组干扰素α可能会成为脾切除术后病情进展患者的首选治疗药物。了解重组干扰素α的作用机制可能会有助于总体上阐明干扰素的作用途径,同时也能为这种特定增殖性疾病的发病机制提供见解。