Department of Hematology, Shengjing Hospital of China Medical University, Shenyang, China.
Department of Hematology, Jiangsu Province Hospital, Nanjing, China.
Ann Palliat Med. 2021 Dec;10(12):12431-12440. doi: 10.21037/apm-21-3210.
The coexistence of plasma cell neoplasia such as multiple myeloma (MM) or monoclonal gammopathy of underdetermined significance (MGUS) with myeloid neoplasia such as myelodysplastic syndrome with excess blasts (MDS-EB) is exceedingly rare. Seeking to understand the clinical features of this dual hematological neoplasm and exploration of novel therapeutic approaches is warranted.
The cases of 7 patients diagnosed with both MGUS/MM coexisting with MDS-EB were reported. Moreover, this study reviewed and summarized 34 published cases of MDS including 7 cases of MDS-EB, describing the coexistence with plasma cell disease, and analyzed the clinical characteristics and survival of these cases.
In total, 14 cases (7 reported here and 7 previously published) of MGUS/MM coexisting with MDS-EB were analyzed. Of these 14 patients, the median age was 65.5 years. Almost all (85.7%) participants had severe anemia or pancytopenia, and nearly half (42.9%) of the cases developed into acute myeloid leukemia (AML). Half of the participants showed osteolytic lesions. The median bone marrow plasma cell count was 23.0%, and the median myeloid blast count was 7.5%. Immunological analysis using flow cytometry confirmed the coexistence of 2 different clones, malignant myeloid clone (CD34+, CD117+, HLA-DR+, CD33+, and CD13+) and plasma clone (CD38+, CD138+, and CD56+). Patients with MGUS/MM and MDS-EB experience very poor therapeutic responses. A great number of patients (64%) were reported to have no response or rapid relapse. The median overall survival (OS) was only 8 months for patients with MGUS/MM and MDS-EB, which was significantly shorter than that of those with MGUS/MM and MDS-other type (median OS of 52 months) (P=0.0009).
Herein, a type of malignant myeloid clone concurrent with plasma clone was reported, without previous exposure to chemotherapy, and poor prognosis of these patients was observed. However, standard treatment methods are still absent, which therefore heightens our awareness of this type of disease and the urgent need for further investigation to prolong survival.
浆细胞肿瘤，如多发性骨髓瘤（MM）或意义未明单克隆丙种球蛋白血症（MGUS），与骨髓增生异常综合征伴原始细胞增多-Ⅰ型（MDS-EB）等髓系肿瘤同时存在的情况极为罕见。因此，有必要了解这种双重血液系统肿瘤的临床特征，并探索新的治疗方法。
报告了 7 例诊断为 MGUS/MM 合并 MDS-EB 的患者病例。此外，本研究还回顾和总结了 34 例 MDS 病例，包括 7 例 MDS-EB，描述了与浆细胞疾病的共存情况，并分析了这些病例的临床特征和生存情况。
总共分析了 14 例 MGUS/MM 合并 MDS-EB 的病例（这里报告了 7 例，之前发表的 7 例）。这 14 名患者的中位年龄为 65.5 岁。几乎所有患者（85.7%）均有严重贫血或全血细胞减少，近一半（42.9%）的病例进展为急性髓系白血病（AML）。半数患者出现溶骨性病变。骨髓浆细胞计数的中位数为 23.0%，髓性原始细胞计数的中位数为 7.5%。采用流式细胞术进行免疫分析证实存在 2 个不同的克隆，恶性髓性克隆（CD34+、CD117+、HLA-DR+、CD33+和 CD13+）和浆细胞克隆（CD38+、CD138+和 CD56+）。MGUS/MM 合并 MDS-EB 的患者治疗反应非常差。大量患者（64%）无反应或快速复发。MGUS/MM 合并 MDS-EB 患者的中位总生存期（OS）仅为 8 个月，明显短于 MGUS/MM 合并 MDS-其他类型（中位 OS 52 个月）（P=0.0009）。
在此，报告了一种无先前化疗暴露的恶性髓性克隆与浆细胞克隆同时存在的病例，观察到这些患者的预后较差。然而，目前仍然缺乏标准的治疗方法，这因此提高了我们对这种疾病的认识，迫切需要进一步研究以延长生存时间。
