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伴有侵袭性白血病性皮肤病变且对维奈克拉和地西他滨反应迅速的IDH2突变型近ETP-ALL

IDH2-mutated near ETP-ALL with aggressive leukemia cutis and brisk response to venetoclax and decitabine.

作者信息

Vaidya Poorva, Wang Huan-You, Don Michelle D, Hinds Brian R, Mangan James K

机构信息

Department of Internal Medicine, Division of Hematology-Oncology, Moores Cancer Center, University of California, San Diego, La Jolla, CA, United States.

Department of Pathology, University of California, San Diego, La Jolla, CA, United States.

出版信息

Leuk Res Rep. 2023 Dec 30;21:100408. doi: 10.1016/j.lrr.2023.100408. eCollection 2024.

Abstract

Near early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a rare hematologic malignancy, for which second line therapeutic options are limited. T-cell leukemias are also rarely associated with leukemia cutis, which is more often seen in leukemias of myeloid origin. We present the case of an adult male diagnosed with near ETP-ALL, with IDH2 and DNMT3A mutations, suggestive of a myeloid origin, and leukemia cutis. After the patient progressed on hyper-CVAD and nelarabine, we treated him with the BCL-2 inhibitor venetoclax and the hypomethylating agent decitabine. The regimen induced a rapid bone marrow response and resolution of the leukemia cutis.

摘要

近早期T细胞前体急性淋巴细胞白血病(ETP-ALL)是一种罕见的血液系统恶性肿瘤,其二线治疗选择有限。T细胞白血病也很少与皮肤白血病相关,后者在髓系起源的白血病中更为常见。我们报告了一例成年男性患者,诊断为近ETP-ALL,伴有IDH2和DNMT3A突变,提示髓系起源,并患有皮肤白血病。在患者接受Hyper-CVAD和奈拉滨治疗病情进展后,我们用BCL-2抑制剂维奈克拉和去甲基化药物地西他滨对其进行治疗。该方案诱导了快速的骨髓反应并使皮肤白血病消退。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fe/10805932/b2f61d2c2438/gr1.jpg

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