Division of Nephrology and Endocrinology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Department of Hematology and Oncology, The University of Tokyo Hospital, Tokyo, Japan.
BMC Endocr Disord. 2023 Jun 5;23(1):128. doi: 10.1186/s12902-023-01384-5.
Bilateral adrenal infarction is rare and only a small number of cases have been reported so far. Adrenal infarction is usually caused by thrombophilia or a hypercoagulable state, such as antiphospholipid antibody syndrome, pregnancy, and coronavirus disease 2019. However, adrenal infarction with myelodysplastic/myeloproliferative neoplasm (MDS/MPN) has not been reported.
An 81-year-old man with a sudden severe bilateral backache presented to our hospital. Contrast-enhanced computed tomography (CT) led to the diagnosis of bilateral adrenal infarction. Previously reported causes of adrenal infarction were all excluded and a diagnosis of MDS/MPN-unclassifiable (MDS/MPN-U) was reached, which was considered to be attributed to adrenal infarction. He developed a relapse of bilateral adrenal infarction, and aspirin administration was initiated. Partial primary adrenal insufficiency was suspected as the serum adrenocorticotropic hormone level was persistently high after the second bilateral adrenal infarction.
This is the first case of bilateral adrenal infarction with MDS/MPN-U encountered. MDS/MPN has the clinical characteristics of MPN. It is reasonable to assume that MDS/MPN-U may have influenced bilateral adrenal infarction development, considering the absence of thrombosis history and a current comorbid hypercoagulable disease. This is also the first case of recurrent bilateral adrenal infarction. It is important to carefully investigate the underlying cause of adrenal infarction once adrenal infarction is diagnosed, as well as to assess adrenocortical function.
双侧肾上腺梗死罕见，迄今为止，仅有少数病例报道。肾上腺梗死通常由血栓形成倾向或高凝状态引起，如抗磷脂抗体综合征、妊娠和 2019 年冠状病毒病。然而，骨髓增生异常/骨髓增殖性肿瘤（MDS/MPN）伴发的肾上腺梗死尚未见报道。
一名 81 岁男性突发双侧剧烈腰痛，就诊于我院。增强 CT 提示双侧肾上腺梗死。既往报道的肾上腺梗死病因均被排除，诊断为 MDS/MPN 不能分类（MDS/MPN-U），考虑为肾上腺梗死所致。他出现双侧肾上腺梗死复发，给予阿司匹林治疗。第二次双侧肾上腺梗死发生后，患者血清促肾上腺皮质激素水平持续升高，疑为部分原发性肾上腺皮质功能不全。
本例是首例 MDS/MPN-U 伴双侧肾上腺梗死的病例。MDS/MPN 具有 MPN 的临床特征。鉴于无血栓形成病史和当前合并的高凝性疾病，合理假设 MDS/MPN-U 可能影响双侧肾上腺梗死的发生。这也是首例双侧肾上腺梗死复发的病例。一旦诊断为肾上腺梗死，应仔细调查其潜在病因，并评估肾上腺皮质功能。
