Katagiri Takayuki, Ushiki Takashi, Masuko Masayoshi, Tanaka Tomoyuki, Miyakoshi Shukuko, Fuse Kyoko, Shibasaki Yasuhiko, Takizawa Jun, Aoki Sadao, Sone Hirohito
Department of Hematology Department of Transfusion Medicine and Regenerative Medicine, Niigata University Medical and Dental Hospital Department of Pathophysiology, Faculty of Pharmaceutical Sciences, Niigata University of Pharmacy and Applied Life Sciences, Niigata, Japan.
Medicine (Baltimore). 2017 Sep;96(36):e7975. doi: 10.1097/MD.0000000000007975.
Myeloid sarcoma (MS) and leukemia cutis (LC) are extramedullary tumors comprising myeloid blasts. They can occur de novo or concurrently with hematological disorders, usually acute myeloid leukemia (AML). AML chemotherapy is generally the initial therapy for MS and LC, and hematopoietic stem cell transplantation (HSCT) can be considered as additional therapy. However, treatment for older patients who are unable to continue intensive chemotherapy is not currently standardized.
A 71-year-old Japanese woman was diagnosed with multiple MSs associated with myelodysplastic syndrome (MDS), using bone marrow aspiration and lymph node biopsy.
Additionally, LC was diagnosed by skin biopsy. Extramedullary MS and LC lesions were formed by massive infiltration of myeloblastic cells.
Twenty courses of 5-azacytidine (5-Aza) were administrated as maintenance therapy after induction therapy with daunorubicin and cytarabine.
Myeloblasts decreased in the bone marrow and the LC disappeared after induction therapy. The MSs completely disappeared, except for the palatine tonsil lesion, after 5-Aza maintenance therapy. 5-Aza treatment provided long-term partial response for more than 21 months.
5-Aza was well tolerated and may be a good option for the treatment of MS and LC associated with MDS, especially in older patients who cannot receive HSCT.
髓系肉瘤(MS)和皮肤白血病(LC)是由髓母细胞组成的髓外肿瘤。它们可原发发生,或与血液系统疾病(通常为急性髓系白血病(AML))同时出现。AML化疗通常是MS和LC的初始治疗方法,造血干细胞移植(HSCT)可作为辅助治疗。然而,对于无法继续进行强化化疗的老年患者,目前尚无标准化的治疗方案。
一名71岁的日本女性通过骨髓穿刺和淋巴结活检被诊断为与骨髓增生异常综合征(MDS)相关的多发性MS。
此外,通过皮肤活检诊断为LC。髓母细胞大量浸润形成了髓外MS和LC病变。
在用柔红霉素和阿糖胞苷进行诱导治疗后,给予20个疗程的5-氮杂胞苷(5-Aza)作为维持治疗。
诱导治疗后骨髓中的髓母细胞减少,LC消失。5-Aza维持治疗后,除腭扁桃体病变外,MS完全消失。5-Aza治疗提供了超过21个月的长期部分缓解。
5-Aza耐受性良好,可能是治疗与MDS相关的MS和LC的一个好选择,特别是对于无法接受HSCT的老年患者。