幼児における急性リンパ性白血病の産前発症の原因

J L Wiemels¹, G Cazzaniga, M Daniotti

¹Leukaemia Research Fund Centre, Institute of Cancer Research, Chester Beatty Laboratories, London, UK.

Lancet (London, England)

|November 7, 1999

まとめ

幼児期急性リンパ性白血病 (ALL) は,出産前に発症することが多い. ALLに共通するTEL-AML1融合は子宮内で発生しますが,完全な白血病の発症には後期のイベントが必要です.

科学分野:

遺伝学遺伝学とは
小児腫瘍学小児腫瘍学
分子生物学は分子生物学である.

背景:

幼児白血病の自然史に関する理解は限られている.
TEL-AML1遺伝子融合は,小児性急性リンパ性白血病 (ALL) で一般的です.
TEL-AML1異常の産前起源を調査する.

研究の目的:

幼児期のTEL-AML1遺伝子融合 ALLが産前発症するかどうかを判断する.
TEL-AML1融合につながる染色体転位イベントのタイミングを調査する.

主な方法:

RT-PCRを用いたALLの12人の子供におけるTEL-AML1融合のスクリーニング.
ゲノムTEL-AML1融合の増幅と配列決定.
長距離逆PCRと患者特有のプライマーを用いて.
新生児の血の斑点を分析して,産前融合を検出する.

主要な成果:

TEL-AML1融合配列は,ALLの同卵性双子の新生児の血液斑点で検出されました.
ある双子のTEL-AML1融合の産前起源が示唆されている.
評価可能な9人の患者のうち6人は,融合のための陽性な血の斑点を示した.

結論:

幼児ALLは,子宮内の染色体転位によって頻繁に開始されます.
TEL-AML1融合イベントだけでは,臨床白血病では不十分です.
臨床白血病の発症には,産後イベントが必要である.

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