Kaur Varinder, Swami Arjun, Alapat Daisy, Abdallah Al Ola, Motwani Pooja, Hutchins Laura F, Jethava Yogesh
a Department of Medicine, Division of Hematology and Oncology, Emily Couric Cancer Center , University of Virginia , Charlottesville , VA , USA.
b Division of Hematology and Oncology , Winthrop P. Rockefeller Cancer Institute, University of Arkansas for Medical Sciences , Little Rock , AR , USA.
Hematology. 2018 Jan;23(1):17-24. doi: 10.1080/10245332.2017.1333275. Epub 2017 Jun 2.
Myeloid sarcoma (MS) is characterized by extramedullary infiltration by immature myeloid cells. Owing to rarity of this disease, the clinical features and overall outcomes are yet to be clarified.
To define clinical characteristics, epidemiology, pathologic findings, treatment options and outcomes in MS.
We conducted a retrospective review of 23 patients diagnosed with MS at our institute over a period of 13 years (2002-2015).
MS presented mostly as a manifestation of relapsed acute myeloid leukemia, seen in 39% of patients. Skin and subcutaneous soft tissues were the most common sites of anatomic involvement (69.5%). Ninety five percent (n = 19) were positive for classical myeloid markers with either cytochemical staining (chloracetate-esterase, MPO), flow-cytometry (CD33, CD34, CD13 and CD117), or immunohistochemistry (CD34, CD43, CD68 and lysozyme). Of these, 52% were positive for CD33 (n = 12), 35% for CD68 (n = 8), 30% for CD34 (n = 7), and 26% for lysozyme (n = 6). Cytogenetic abnormalities were seen in 63% (n = 12/19) patients on bone-marrow aspirate, with five patients displaying a complex (n = 3) or monosomal (n = 2) karyotype. Twenty seven percent patients with a normal karyotype had presence of deleterious mutations (FLT3, ASXL, STAG and JAK2) on further testing with myeloid mutation panel. The Median overall survival (OS) of the entire cohort was 15.9 months (95% CI, 7.4-24.4 months). The OS was significantly better for patients <65 years (24.6 vs. 3.4 months, p = 0.009) of age, and for those attaining a complete remission (CR) to induction therapy (25.7 vs. 0.8 months, p < 0.001). All patients who underwent allogeneic hematopoietic stem cell transplant attained long-term remissions, with a median follow-up of 54 (range 32-120) months.
Failure to achieve CR with induction therapy, and age >65 years are associated with poor outcomes in MS. Allogeneic stem-cell transplant in first remission appears to be the most effective modality for achieving long-term remissions.
髓系肉瘤（MS）的特征是未成熟髓系细胞的髓外浸润。由于这种疾病罕见，其临床特征和总体预后尚待阐明。
明确MS的临床特征、流行病学、病理表现、治疗选择及预后。
我们对13年间（2002 - 2015年）在我院确诊为MS的23例患者进行了回顾性研究。
MS大多表现为复发急性髓系白血病，39%的患者可见此表现。皮肤和皮下软组织是最常见的解剖学受累部位（69.5%）。95%（n = 19）的患者通过细胞化学染色（氯乙酸酯酶、MPO）、流式细胞术（CD33、CD34、CD13和CD117）或免疫组织化学（CD34、CD43、CD68和溶菌酶）检测，经典髓系标志物呈阳性。其中，52%（n = 12）的患者CD33呈阳性，35%（n = 8）的患者CD68呈阳性，30%（n = 7）的患者CD34呈阳性，26%（n = 6）的患者溶菌酶呈阳性。63%（n = 12/19）的患者骨髓穿刺可见细胞遗传学异常，5例患者表现为复杂核型（n = 3）或单倍体核型（n = 2）。27%核型正常的患者经髓系突变检测进一步发现存在有害突变（FLT3、ASXL、STAG和JAK2）。整个队列的中位总生存期（OS）为15.9个月（95%CI，7.4 - 24.4个月）。年龄<65岁的患者OS明显更好（24.6个月对3.4个月，p = 0.009），诱导治疗达到完全缓解（CR）的患者OS也更好（25.7个月对0.8个月，p < 0.001）。所有接受异基因造血干细胞移植的患者均获得长期缓解，中位随访时间为54（范围32 - 120）个月。
诱导治疗未达到CR以及年龄>·65岁与MS的不良预后相关。首次缓解期进行异基因干细胞移植似乎是实现长期缓解的最有效方式。
