Simonin Mathieu, Lainey Elodie, Rialland Fanny, Caye-Eude Aurélie, Reguerre Yves, Boutroux Hélène, Azarnoush Saba, Thouvenin Sandrine, Rohrlich Pierre Simon, Baruchel André
Service d'hématologie et oncologie pédiatrique, hôpital Armand-Trousseau, Assistance publique des Hôpitaux de Paris (AP-HP), Sorbonne université, Paris, France.
Laboratoire d'hématologie, Hôpital Robert-Debré, Assistance publique des Hôpitaux de Paris (AP-HP), Université Paris-Cité, Paris, France.
Bull Cancer. 2023 Oct 27. doi: 10.1016/j.bulcan.2023.09.004.
Acute leukemias of ambiguous lineage (ALAL) represent between 3 and 5% of childhood AL. This term encompasses many subtypes of AL that have been defined according to the immunophenotypic profile based on the expression of various lineage markers. This classification has been modified and enriched during the last decade thanks to the improvement of molecular biology techniques, which have led to reconsider the ontogenic proximity existing between certain forms of ALAL. This increasing diagnostic complexity justifies the establishment of a close communication between clinicians and biologists in the management of these rare forms of AL. Indeed, the initial classification remains the cornerstone of their management since it conditions the future choice of therapeutic protocol. Thus, with the notable exception of undifferentiated forms of AL or AUL (for acute undetermined leukemia), it is now accepted that ALAL benefit from a lymphoid-based therapy approach. As with the management of "classic" acute lymphoblastic leukemias (ALL), the evaluation of response to treatment will determine the modalities of therapeutic intensification. The objective of improving the prognosis of ALAL justifies, in the long term, their future inclusion in the international ALLTogether protocol while continuing in-depth molecular exploration of these patients to identify targeted therapies.
急性混合细胞白血病（ALAL）占儿童急性白血病（AL）的3%至5%。该术语涵盖了许多根据基于各种谱系标志物表达的免疫表型特征所定义的AL亚型。在过去十年中，由于分子生物学技术的进步，这一分类得到了修改和丰富，这些技术促使人们重新考虑某些形式的ALAL之间存在的起源相近性。这种日益增加的诊断复杂性证明了临床医生和生物学家在管理这些罕见形式的AL时建立密切沟通的合理性。事实上，初始分类仍然是其管理的基石，因为它决定了未来治疗方案的选择。因此，除了未分化形式的AL或AUL（急性未定型白血病）这一显著例外，现在人们公认ALAL可从基于淋巴细胞的治疗方法中获益。与“经典”急性淋巴细胞白血病（ALL）的管理一样，对治疗反应的评估将决定治疗强化的方式。从长远来看，改善ALAL预后的目标证明了将它们纳入国际ALLTogether方案的合理性，同时继续对这些患者进行深入的分子探索以确定靶向治疗方法。
