Transient abnormal myelopoiesis (TAM) causing multiple organ failure
Transient abnormal myelopoiesis (TAM) causing multiple organ failure
复制标题
短暂性骨髓生成异常 (TAM) 导致多器官衰竭
DOI：
10.1007/s00134-002-1305-7
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发表时间：
2002
影响因子：
38.9
通讯作者：
C. Pierce
中科院分区：
文献类型：
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作者：
A. Hoskote;J. Chessells;C. Pierce
目的：对入住我们重症监护病房 (ICU) 的所有患有短暂性骨髓生成异常 (TAM) 的新生儿进行检查，TAM 是一种与唐氏综合症相关的自限性先天性骨髓增生性疾病。我们开始担心，在需要重症监护的新生儿中，TAM 的病程可能不会自限，从而导致预后不良。方法。对 1996 年至 2000 年间入住我们重症监护病房 (ICU) 的 TAM 和 21 三体性新生儿进行回顾性分析。记录临床表现、疾病进展和实验室检查结果，以确定任何预后特征。结果。收治了 4 例 TAM 和 21 三体性新生儿。所有四人均在出生后第一周内出现继发于肺动脉高压的呼吸衰竭。所有患者均出现水肿并显示出高粘滞血症的证据。就诊时白细胞计数中位数为 282，范围为 56–471×109/l。除一名外，所有患者均接受换血和低剂量阿糖胞苷治疗。尽管进行了积极治疗，仍有三人死于多器官衰竭。对婴儿的尸检证实了诊断，并显示肺部和肝脏有多处血栓栓塞。结论。在需要 ICU 的 TAM 婴儿中，这种“自限性”疾病造成的疾病严重程度是惊人的，由此产生的发病率和死亡率以前从未有过报道。致命结果与 TAM 自发消退的标志尚不清楚。对于白细胞计数高的畸形、水肿新生儿，应怀疑 TAM，并应尽早开始积极的换血和同步化疗。
Objective.To review all neonates admitted to our intensive care unit (ICU) with Transient Abnormal Myelopoiesis (TAM) – a self-limiting congenital myeloproliferative disorder seen in association with Down's syndrome. We have become concerned that in neonates requiring intensive care, TAM may not be self-limiting in its course resulting in a poor prognosis.Method.A retrospective review of neonates admitted to our intensive care unit (ICU) with TAM and trisomy 21 between 1996 and 2000. Clinical presentation, disease progression and laboratory findings were recorded to identify any prognostic features.Results.Four cases of TAM and trisomy 21 were admitted. All four presented within the 1st week of life in respiratory failure secondary to pulmonary hypertension. All were hydropic and showed evidence of hyperviscosity. Median white cell count at presentation was 282, range 56–471×109/l. All excepting one were treated with exchange transfusion and low dose cytarabine. Three died of multi-organ failure in spite of aggressive treatment. Post mortem examination of the infants confirmed the diagnoses and showed multiple thrombo-emboli in the lungs and livers.Conclusion.In infants with TAM requiring ICU, the severity of illness produced by this 'self-limiting' disease is striking and the resultant morbidity and mortality has not been reported previously. The markers for fatal outcome versus spontaneous resolution of TAM are not clear. In a dysmorphic, hydropic neonate with a high white cell count TAM should be suspected and early, aggressive exchange transfusion and simultaneous chemotherapy should be started.
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