Abstract：
TAFRO syndrome is a rare systemic inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. The diagnosis of TAFRO syndrome can be challenging; however, prompt diagnosis is vital because TAFRO syndrome is a progressive and life-threatening disease. We have showcased five patients with TAFRO syndrome who had similar bone marrow (BM) findings that could be considered the findings that characterize TAFRO syndrome. All patients were treated with corticosteroids and tocilizumab; three of the five patients (60 %) responded positively to the treatment. The unique BM findings observed in this study were megakaryocytes with distinct multinuclei and three-dimensional and indistinct bizarre nuclei (\"dysmorphic megakaryocyte\"), similar to the megakaryocyte morphology observed in myeloproliferative neoplasms (MPNs). Notably, dysmorphic megakaryocytes were observed in all five cases, whereas only two of the five patients tested positive for reticulin myelofibrosis, and three of the five patients had megakaryocytic hyperplasia, which are considered typical findings of TAFRO syndrome. Thus, the BM findings of dysmorphic megakaryocytes could help in the correct and immediate diagnosis of TAFRO syndrome.
摘要：
TAFRO综合征是一种病因不明的罕见全身性炎症性疾病，以血小板减少为特征。Anasarca,发烧,网状蛋白骨髓纤维化，肾功能不全,和器官肿大。TAFRO综合征的诊断可能具有挑战性；然而，及时诊断至关重要,因为TAFRO综合征是一种进行性和危及生命的疾病.我们已经展示了五名TAFRO综合征患者，他们具有相似的骨髓（BM）发现，可以认为是TAFRO综合征的特征。所有患者均接受皮质类固醇和托珠单抗治疗；五名患者中有三名（60％）对治疗反应积极。在这项研究中观察到的独特的BM发现是具有不同多核和三维和模糊的奇异核的巨核细胞（“异形巨核细胞”），与骨髓增殖性肿瘤（MPN）中观察到的巨核细胞形态相似。值得注意的是,在所有5例病例中均观察到异形巨核细胞，而五名患者中只有两名检测出网状蛋白骨髓纤维化呈阳性，五名患者中有三名患有巨核细胞增生，这些被认为是TAFRO综合征的典型发现。因此，变形巨核细胞的BM发现有助于TAFRO综合征的正确和即时诊断。
