Creutzig U, Ritter J, Budde M, Sutor A, Schellong G
University Children's Hospital, Muenster, Federal Republic of Germany.
Cancer. 1987 Dec 15;60(12):3071-9. doi: 10.1002/1097-0142(19871215)60:12<3071::aid-cncr2820601235>3.0.co;2-y.
There were 294 children with acute myelogenous leukemia (AML) entered into the German AML Berlin, Frankfurt, and Münster hospitals (BFM) 78 and 83 studies. Thirty (10%) died as a result of hemorrhage and/or leukostasis prior to or in the first 12 days of therapy. The risk of early death due to hemorrhage and/or leukostasis is significantly greater when certain features are initially present: acute monocytic leukemia (French-American-British [FAB] M5), hyperleukocytosis (greater than or equal to 100,000/microliter), and extramedullary organ involvement (P less than 0.001). The risk increases sharply when these factors exist in combination: 72% mortality with FAB M5 and hyperleukocytosis, and 43% with FAB M5 and extramedullary organ involvement. In 11 patients leukostasis alone or in combination with hemorrhage was probably the cause of death during the first 3 days after diagnosis. All 11 children presented with hyperleukocytosis and were classified as monocytic subtype FAB M4 or M5. After induction, a close temporal association between rapid blast reduction and occurrence of fatal hemorrhage was established in five patients. Thrombocytopenic hemorrhages were controllable and, therefore, responsible for death only in exceptional cases. It is difficult to avoid these early fatal complications with current therapeutic measures. Early exchange transfusion together with special supportive care may be useful.
294名急性髓性白血病(AML)患儿进入了德国AML柏林、法兰克福和明斯特医院(BFM)的78和83项研究。30名(10%)患儿在治疗前或治疗的前12天内死于出血和/或白细胞淤滞。当初始存在某些特征时,因出血和/或白细胞淤滞导致早期死亡的风险显著更高:急性单核细胞白血病(法美英[FAB] M5型)、白细胞增多症(大于或等于100,000/微升)和髓外器官受累(P<0.001)。当这些因素同时存在时,风险急剧增加:FAB M5型和白细胞增多症的死亡率为72%,FAB M5型和髓外器官受累的死亡率为43%。在11名患者中,单独的白细胞淤滞或与出血合并可能是诊断后前3天内死亡的原因。所有11名儿童均表现为白细胞增多症,被归类为单核细胞亚型FAB M4或M5。诱导治疗后,在5名患者中确定了快速原始细胞减少与致命性出血发生之间密切的时间关联。血小板减少性出血是可控的,因此仅在特殊情况下才导致死亡。用目前的治疗措施很难避免这些早期致命并发症。早期换血联合特殊支持治疗可能有用。