实体器官移植后的骨髓增生性肿瘤：23 例临床病理研究。

Myeloid Neoplasms Following Solid Organ Transplantation: Clinicopathologic Studies of 23 Cases.

机构信息

Division of Hematology, Department of Medicine, Shengjing Hospital affiliated to China Medical University, Shenyang, China.

Department of Pathology, Duke University Medical Center, Durham, NC.

出版信息

Am J Clin Pathol. 2017 Dec 20;149(1):55-66. doi: 10.1093/ajcp/aqx133.

DOI:10.1093/ajcp/aqx133

PMID:29228125

Abstract

OBJECTIVES

Myeloid neoplasms (MNs) after solid organ transplant are rare, and their clinicopathologic features have not been well characterized.

METHODS

We retrospectively analyzed 23 such cases.

RESULTS

The ages ranged from 2 to 76 years, with a median of 59 years at the diagnosis. The median interval between the transplant and diagnosis was 56 months (range, 8-384 months). The transplanted organs included liver in five, kidney in six, lung in five, heart in six, and heart/lung in one case(s). The types of MN included acute myeloid leukemia (AML) in 12, myelodysplastic syndrome (MDS) in five, chronic myelogenous leukemia (CML) in four, and myeloproliferative neoplasms (MPNs) in two cases. Cytogenetics demonstrated clonal abnormalities in 18 (78.3%) cases, including unbalanced changes in 10 (55.6%), Philadelphia chromosome in four (22.2%), and other balanced aberrations in four (22.2%) cases. Thirteen (56.5%) patients died, with an estimated median survival of 9 months. With disease stratification, AML and MDS have short median survivals (3.5 and 7 months, respectively), with an initial precipitous decline of the survival curve.

CONCLUSIONS

Posttransplant MNs have a latency period between that seen in AML/MDS related to alkylators and that associated with topoisomerase II inhibitors. The cytogenetic profile suggests a mutagenic effect on leukemogenesis. The clinical outcome for AML/MDS is dismal, with death occurring at an early phase of treatment.

摘要

目的

实体器官移植后发生的骨髓增生性肿瘤（MNs）较为罕见，其临床病理特征尚未得到充分描述。

方法

我们回顾性分析了 23 例此类病例。

结果

患者年龄为 2 至 76 岁，诊断时的中位年龄为 59 岁。移植与诊断之间的中位间隔为 56 个月（范围为 8-384 个月）。移植的器官包括肝脏 5 例、肾脏 6 例、肺 5 例、心脏 6 例和心脏/肺 1 例。MN 的类型包括急性髓细胞白血病（AML）12 例、骨髓增生异常综合征（MDS）5 例、慢性髓细胞白血病（CML）4 例和骨髓增生性肿瘤（MPNs）2 例。细胞遗传学显示 18 例（78.3%）存在克隆异常，包括 10 例（55.6%）非平衡改变、4 例费城染色体（22.2%）和 4 例其他平衡异常（22.2%）。13 例（56.5%）患者死亡，估计中位生存期为 9 个月。根据疾病分层，AML 和 MDS 的中位生存期较短（分别为 3.5 和 7 个月），生存期曲线初始急剧下降。