Management of Extramedullary Leukemia as a Presentation of Acute Myeloid Leukemia
Management of Extramedullary Leukemia as a Presentation of Acute Myeloid Leukemia
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DOI：
10.6004/jnccn.2012.0120
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发表时间：
2012-09-01
影响因子：
13.4
通讯作者：
Shami, Paul J.
中科院分区：
文献类型：
--
作者：
Slomowitz, Samuel J.;Shami, Paul J.
髓外受累被认为是急性髓系白血病（AML）的一种罕见表现，尽管一些数据表明它可能存在于高达30%的患者中。急性髓细胞白血病的髓外受累可表现为多种临床表现，最显著的是髓样肉瘤、皮肤白血病和中枢神经系统受累。每一种都在症状和管理方面呈现出独特的临床情况。任何形式的髓外疾病，如果没有骨髓疾病的证据，仍然被认为是全身性疾病的证据，通常按此治疗。最常见的是，髓外疾病与骨髓疾病同时出现，尽管可能需要鞘内化疗或放疗形式的额外局部治疗，但全身治疗的原则保持不变。髓外疾病的预后影响尚不清楚。具体而言，造血干细胞移植是否应考虑在第一次缓解，而不考虑其他预后因素尚未建立。接受移植的患者与没有髓外疾病的患者结局相似，尽管他们确实有更高的髓外复发率。需要更多的研究来确定髓外疾病的分子基础，其预后影响和最佳管理。（JNCCN 2012;10：1065-1169）
Extramedullary involvement is considered to be an uncommon presentation of acute myeloid leukemia (AML), although some data suggest it may be present in up to 30% of patients. Extramedullary involvement by AML can present in a variety of clinical manifestations, most notably in the form of myeloid sarcoma, leukemia cutis, and central nervous system involvement. Each presents a unique clinical scenario in terms of symptoms and management. Extramedullary disease in any form presenting without evidence of bone marrow disease is still considered evidence of systemic disease and is usually treated as such. Most commonly, extramedullary disease presents concurrently with bone marrow disease, and although it may require additional local therapy in the form of intrathecal chemotherapy or radiation, the principles of systemic treatment remain unchanged. The prognostic impact of extramedullary disease is unclear. Specifically, whether hematopoietic stem cell transplantation should be considered in first remission irrespective of other prognostic factors has not been established. Patients who undergo transplantation have similar outcomes as patients without extramedullary disease, although they do have a higher rate of extramedullary relapse. More research is needed to define the molecular basis for extramedullary disease, its prognostic impact, and optimal management. (JNCCN 2012;10:1065-1169)