Hematology, Graduate School of Medicine, Osaka City University, Japan.
Intern Med. 2020 Nov 1;59(21):2751-2756. doi: 10.2169/internalmedicine.4912-20. Epub 2020 Jul 7.
A 53-year-old man presented with uncontrolled bleeding caused by acquired platelet dysfunction accompanied by calreticulin-mutated primary myelofibrosis. Based on the detection of abnormal platelets, including large gray platelets, under light microscopy and the loss of the second wave of aggregation observed by light transmission aggregometry, the patient was diagnosed with platelet dysfunction accompanied by myeloproliferative neoplasms (MPNs). In addition, the absence of platelet α-granules was confirmed by electron microscopy. Therefore, this condition may be termed "acquired gray platelet syndrome." Acquired platelet dysfunction must be ruled out when abnormal platelets are observed in patients with MPNs.
一位 53 岁男性因获得性血小板功能障碍伴钙网织蛋白突变原发性骨髓纤维化引起的无法控制的出血而就诊。基于光镜下观察到异常血小板，包括大灰色血小板，以及光传输聚集测定法观察到的第二波聚集缺失，诊断为伴有骨髓增生性肿瘤（MPNs）的血小板功能障碍。此外，电子显微镜证实血小板α 颗粒缺失。因此，这种情况可能被称为“获得性灰色血小板综合征”。当 MPN 患者观察到异常血小板时，必须排除获得性血小板功能障碍。
