Fishel R S, Farnen J P, Hanson C A, Silver S M, Emerson S G
Department of Internal Medicine, University of Michigan, Ann Arbor.
Medicine (Baltimore). 1990 Jul;69(4):232-43. doi: 10.1097/00005792-199007000-00005.
Acute lymphoblastic leukemia with eosinophilia is a rare but distinctive clinical entity. The eosinophilia in these patients can present before, concomitantly, or after the diagnosis of leukemia. Patients with this syndrome often suffer from the cardiovascular complications of severe eosinophilia, suffering excess morbidity and mortality as a result of their eosinophilia. Treatment of the eosinophilia in this syndrome consists of administration of induction chemotherapy, followed by prednisone and hydroxyurea if required for persistent eosinophilia. Eosinophilia often resolves with remission of leukemia, only to return at the time of relapse in a high percentage of cases. Patients with this syndrome characteristically have cytogenetic abnormalities involving the long arms of chromosomes 5 and 14. These cytogenetic abnormalities are not commonly seen in acute lymphoblastic leukemia and suggest that this syndrome may have a distinct pathophysiology and etiology. The affected region on chromosome 5 contains genes that control hematopoiesis, including eosinophilopoiesis. Further investigations into these cytogenetic abnormalities may provide insight into the etiology of the leukemia and eosinophilia characteristic of this syndrome.
伴有嗜酸性粒细胞增多的急性淋巴细胞白血病是一种罕见但独特的临床实体。这些患者的嗜酸性粒细胞增多可在白血病诊断之前、同时或之后出现。患有该综合征的患者常因严重嗜酸性粒细胞增多而出现心血管并发症,其嗜酸性粒细胞增多导致发病率和死亡率过高。该综合征嗜酸性粒细胞增多的治疗包括诱导化疗,如果持续性嗜酸性粒细胞增多需要,随后给予泼尼松和羟基脲。嗜酸性粒细胞增多常随着白血病缓解而消退,但在高比例病例中会在复发时再次出现。患有该综合征的患者特征性地具有涉及5号和14号染色体长臂的细胞遗传学异常。这些细胞遗传学异常在急性淋巴细胞白血病中并不常见,提示该综合征可能具有独特的病理生理学和病因。5号染色体上的受累区域包含控制造血的基因,包括嗜酸性粒细胞生成。对这些细胞遗传学异常的进一步研究可能有助于深入了解该综合征白血病和嗜酸性粒细胞增多的病因。