背景:TAFRO综合征,这是2010年在日本首次报道的,是一种以血小板减少为特征的相对罕见的疾病,Anasarca,发烧,肾功能损害,网状蛋白纤维化,和器官肿大。虽然这种疾病被认为与多中心Castleman病相似,一些临床特征,比如血小板减少症,与典型的多中心Castleman病不同。此外,TAFRO综合征的病因仍然未知且存在争议。只有少数TAFRO综合征并发肾上腺病变,他们都有出血。
方法:本报告描述了一名46岁亚裔男子发烧的病例,上腹痛,背部疼痛1个月。计算机断层扫描显示腹水,轻度淋巴结肿大,左肾上腺坏死无出血。验血显示血小板减少症,贫血,C反应蛋白升高,碱性磷酸酶,和肌酐水平。基于水肿,严重的血小板减少症,发烧,骨髓活检显示网状蛋白骨髓纤维化,轻度淋巴结肿大,和进行性肾功能不全,我们诊断该患者患有TAFRO综合征。他通过立即给予糖皮质激素和托珠单抗成功治疗。
结论:以前没有一例TAFRO综合征并发肾上腺坏死的报道。因为左肾上腺活检显示坏死,没有任何出血迹象,我们得出的结论是,在这种情况下,单侧肾上腺坏死是由严重的高细胞因子血症下梗死或器官肥大引起的。这种不寻常的临床过程可用于进一步分析TAFRO综合征的病因。
BACKGROUND: TAFRO syndrome, which was first reported in 2010 in Japan, is a relatively rare disease characterized by thrombocytopenia, anasarca, fever, renal impairment,
reticulin fibrosis, and organomegaly. Although this disease is considered similar to multicentric Castleman disease, some of the clinical features, such as thrombocytopenia, are different from typical cases of multicentric Castleman disease. In addition, the etiology of TAFRO syndrome remains unknown and controversial. There have only been a few cases of TAFRO syndrome complicated with adrenal gland lesions, and all of them have had hemorrhagic involvement.
METHODS: This report describes the
case of a 46-year-old Asian man who presented with fever, epigastric pain, and back pain for 1 month. A computed tomographic scan revealed ascites, mild lymphadenopathy, and left adrenal necrosis without hemorrhage. A blood test showed thrombocytopenia, anemia, and elevated C-reactive protein, alkaline phosphatase, and creatinine levels. Based on the edema, severe thrombocytopenia, fever,
reticulin myelofibrosis shown by bone marrow biopsy, mild lymphadenopathy, and progressive renal insufficiency, we diagnosed this patient as having TAFRO syndrome. He was successfully treated by immediate administration of glucocorticoids and tocilizumab.
CONCLUSIONS: There have been no previous
reports of a
case of TAFRO syndrome complicated with adrenal necrosis. Because the biopsy of the left adrenal gland revealed necrosis without any evidence of hemorrhage, we concluded that the unilateral adrenal necrosis in this
case was caused by either ischemia from infarction or organomegaly itself under severe hypercytokinemia. This unusual clinical course is useful for further analysis of the etiology of TAFRO syndrome.