Sato Kazuya, Tsukada Nodoka, Inamura Junki, Komatsu Shigetsuna, Sato Keisuke, Yamamoto Masayo, Shindo Motohiro, Moriichi Kentaro, Mizukami Yusuke, Fujiya Mikihiro, Torimoto Yoshihiro, Okumura Toshikatsu
Department of Hematology/Oncology, Asahikawa Kosei General Hospital, 1-24, Asahikawa 078-8211, Japan.
Department of Dermatology, Asahikawa Kosei General Hospital, 1-24, Asahikawa 078-8211, Japan.
Case Rep Hematol. 2021 Apr 21;2021:6640597. doi: 10.1155/2021/6640597. eCollection 2021.
Myeloid sarcoma (MS), which involves extramedullary lesions, is classified as a unique subtype of acute myeloid leukemia (AML). At present, no standard treatments for MS have been established. The patient was an 89-year-old man with myelodysplastic syndrome-excess blast-2 (MDS-EB-2) with a 2-year history of intermittent treatment with azacitidine (AZA) during a 4-year history of MDS. He developed painful cutaneous tumors 8 months after the second discontinuation of AZA. They were refractory for antibiotics and topical tacrolimus hydrate. A tumor biopsy was performed, and the histological findings of the tumor lesion showed a proliferation of tumor cells that were positive for myeloperoxidase and CD68 and negative for CD4 and CD123. The patient was diagnosed with MDS-associated MS. MDS-EB-2 quickly progressed to AML with the appearance of peripheral blood blasts and 25% bone marrow blasts. Monotherapy with reduced-dose AZA (37.5 mg/m for 7 days, every 4-6 weeks) was restarted, and the MS quickly disappeared. The patient's MS was successfully treated with 16 cycles of AZA treatment over a 22-month period. There have been 10 reported cases in which MS was successfully treated with AZA. Among the 10 cases, the patient in the present case was the oldest. Treatment with reduced-dose AZA should be considered as a therapeutic option for MS in elderly patients with MDS, especially patients who are ineligible for intensive chemotherapy.
髓系肉瘤（MS）累及髓外病变，被归类为急性髓系白血病（AML）的一种独特亚型。目前，尚未确立MS的标准治疗方法。该患者为一名89岁男性，患有骨髓增生异常综合征伴过多原始细胞-2（MDS-EB-2），在4年的MDS病史中曾接受过2年的阿扎胞苷（AZA）间歇治疗。在第二次停用AZA 8个月后，他出现了疼痛性皮肤肿瘤。这些肿瘤对抗生素和外用他克莫司水合物均无效。进行了肿瘤活检，肿瘤病变的组织学检查结果显示肿瘤细胞增殖，髓过氧化物酶和CD68呈阳性，CD4和CD123呈阴性。该患者被诊断为MDS相关的MS。MDS-EB-2迅速进展为AML，外周血原始细胞出现，骨髓原始细胞占25%。重新开始使用低剂量AZA单药治疗（37.5mg/m²，持续7天，每4 - 6周一次），MS迅速消失。在22个月的时间里，该患者的MS通过16个周期的AZA治疗成功治愈。已有10例报道称MS通过AZA治疗成功治愈。在这10例病例中，本病例的患者年龄最大。对于患有MDS的老年患者，尤其是不符合强化化疗条件的患者，应考虑使用低剂量AZA进行治疗作为MS的一种治疗选择。
