Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Leuk Res. 2011 Dec;35(12):1605-10. doi: 10.1016/j.leukres.2011.04.021. Epub 2011 May 18.
The diagnosis of therapy-related myelodysplastic syndrome (t-MDS) in the absence of increased myeloblasts or cytogenetic abnormalities is challenging. The presence of ring sideroblasts (RS) in this setting is often used to support the diagnosis of t-MDS. In this study, we reviewed 843 patients initially classified as therapy-related myeloid neoplasm in our hospital over 10 years. Nineteen (2.3%) patients had a normal karyotype, <5% bone marrow (BM) blasts, and ≥15% RS (17-70%), forming this study group. After reviewing clinical charts and follow-up BM specimens, we confirmed the diagnosis of MDS in 13 patients, but in 6 patients the blood counts returned to normal and RS and associated dyserythropoiesis disappeared in the follow-up BM biopsy. With a median follow-up of 21 months, none of these 6 patients died of BM causes. Compared with t-MDS cases, the 6 patients with transient/reversible RS showed comparable numbers of RS and BM blasts, but infrequent dysplasia involving non-erythroid lineages. We conclude that the presence of ≥15% RS in the post-therapy setting is not necessarily indicative of a clonal stem cell neoplasm. Four patients with transient/reversible RS received α-interferon therapy which may contribute to RS formation in this setting.
在没有增加的髓样前体细胞或细胞遗传学异常的情况下，诊断治疗相关骨髓增生异常综合征（t-MDS）具有挑战性。在这种情况下，环形铁幼粒细胞（RS）的存在通常用于支持 t-MDS 的诊断。在这项研究中，我们回顾了 10 年来我院最初分类为治疗相关髓系肿瘤的 843 例患者。19 例（2.3%）患者具有正常核型、<5%骨髓（BM）原始细胞和≥15%RS（17-70%），形成了本研究组。在回顾临床图表和随访 BM 标本后，我们在 13 例患者中确认了 MDS 的诊断，但在 6 例患者中，血细胞计数恢复正常，随访 BM 活检中 RS 和相关的红细胞生成异常消失。中位随访 21 个月，这 6 例患者均未因 BM 原因死亡。与 t-MDS 病例相比，6 例具有一过性/可逆性 RS 的患者具有相似数量的 RS 和 BM 原始细胞，但非红细胞谱系的发育不良较少见。我们得出结论，在治疗后出现≥15%RS 并不一定表明存在克隆性干细胞肿瘤。4 例具有一过性/可逆性 RS 的患者接受了α-干扰素治疗，这可能有助于该情况下 RS 的形成。
