Tomimori Kayo, Kodama Yuki, Tanaka Hiroyuki, Yamashita Atushi, Gi Toshihiro, Asada Yujiro, Doi Koutarou, Katsuragi Shinji, Sato Yuichiro
Department of Obstetrics and Gynecology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
Department of Diagnostic Pathology, Faculty of Medicine, Miyazaki University Hospital, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki, 889-1692, Japan.
Virchows Arch. 2022 Jun;480(6):1181-1187. doi: 10.1007/s00428-022-03289-5. Epub 2022 Feb 24.
Transient abnormal myelopoiesis (TAM), also known as transient myeloproliferative disorder or transient leukemia, is a self-regressing neoplasia that afflicts infants with trisomy 21. A recent review article documented "myeloid cell thrombus (MCT)" and "fetal vascular malperfusion (FVM)" in placentas with TAM, although the characteristic TAM placental findings have not been clarified. Here, we compared the clinical and pathological placental findings between trisomy 21 patients with or without TAM. In 13 cases of trisomy 21, we identified six placentas with TAM and seven placentas without TAM. The six placentas with TAM included two stillborn cases. Microscopically, MCT was noted in all the cases, and a high incidence of FVM (50%) was observed in TAM cases. Immunohistochemically, MCT was found to be a platelet-rich thrombus. The placentas were grouped according to the presence or absence of TAM and subsequently compared. Clinically, the incidences of abnormal fetal heart rate pattern and fetal or neonatal death were significantly higher in TAM cases. Pathologically, placenta in TAM cases weighted more than those in cases without TAM, and the incidence of MCT was significantly higher in placentas with TAM. Moreover, the incidence of FVM was higher in placentas with TAM, but this difference was not statistically significant. We propose that MCT is a diagnostic feature of placentas with TAM and may be associated with poor fetal outcomes.
短暂性异常髓系造血（TAM），也称为短暂性骨髓增殖性疾病或短暂性白血病，是一种会影响21三体综合征婴儿的自限性肿瘤。最近一篇综述文章记录了患有TAM的胎盘出现“髓系细胞血栓（MCT）”和“胎儿血管灌注不良（FVM）”，尽管TAM胎盘的特征性表现尚未明确。在此，我们比较了患有或未患有TAM的21三体综合征患者的临床和病理胎盘表现。在13例21三体综合征病例中，我们确定了6例伴有TAM的胎盘和7例不伴有TAM的胎盘。6例伴有TAM的胎盘包括2例死产病例。显微镜下，所有病例均观察到MCT，并且在伴有TAM的病例中观察到FVM的高发生率（50%）。免疫组化显示，MCT是富含血小板的血栓。根据是否存在TAM对胎盘进行分组，随后进行比较。临床上，伴有TAM的病例中胎儿心率异常模式以及胎儿或新生儿死亡的发生率显著更高。病理上，伴有TAM的病例的胎盘重量超过不伴有TAM的病例，并且伴有TAM的胎盘中MCT的发生率显著更高。此外，伴有TAM的胎盘中FVM的发生率更高，但这种差异无统计学意义。我们认为MCT是伴有TAM的胎盘的一个诊断特征，并且可能与不良胎儿结局相关。
