Bernini J C, Timmons C F, Sandler E S
Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas 75235-9063.
Cancer. 1995 Jan 1;75(1):110-4. doi: 10.1002/1097-0142(19950101)75:1<110::aid-cncr2820750118>3.0.co;2-i.
Acute de novo basophilic leukemia (ABL) is uncommon in adults, and extremely rare in children. To the authors' knowledge, there have been no previous reports of anaphylactoid reactions from basophilic degranulation in children with this condition.
This report describes the clinicopathologic profile and complications of a patient with de novo ABL.
Immediately after the first induction dose of intravenous vincristine, the patient developed an anaphylactoid reaction and disseminated intravascular coagulation with massive pulmonary hemorrhage. A normal serum tryptase level suggested that this life-threatening event was secondary to tumor lysis (basophilic degranulation), rather than to a mast-cell mediated anaphylactic reaction to vincristine. This interpretation is supported by the coagulation studies, which suggested release of heparin from the blast granules.
Although de novo ABL is rare, it should be considered when cytoplasmic basophilic granules are seen in the leukemic cells of patients with what otherwise appears to be undifferentiated leukemia, and the pertinent diagnostic procedures should be undertaken. During the treatment of ABL, potential complications related to basophilic degranulation should be anticipated, and antihistamine prophylaxis may be of value.
急性原发性嗜碱性粒细胞白血病（ABL）在成人中并不常见，在儿童中极为罕见。据作者所知，此前尚无关于患有这种疾病的儿童因嗜碱性粒细胞脱颗粒引起类过敏反应的报道。
本报告描述了一名原发性ABL患者的临床病理特征及并发症。
在首次静脉注射长春新碱诱导剂量后，患者立即出现类过敏反应，并发生弥散性血管内凝血伴大量肺出血。正常的血清类胰蛋白酶水平表明，这一危及生命的事件是由肿瘤溶解（嗜碱性粒细胞脱颗粒）继发引起的，而非肥大细胞介导的对长春新碱的过敏反应。凝血研究支持了这一解释，该研究提示有肝素从原始细胞颗粒中释放出来。
尽管原发性ABL很罕见，但当在其他方面看似未分化白血病患者的白血病细胞中发现细胞质嗜碱性颗粒时，应考虑到该病，并应进行相关诊断程序。在ABL的治疗过程中，应预见到与嗜碱性粒细胞脱颗粒相关的潜在并发症，抗组胺药预防可能有价值。
