Recurrent bilateral adrenal infarction with myelodysplastic/myeloproliferative neoplasm-unclassifiable (MDS/MPN-U): a case report

Abstract

Background 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. Case presentation 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. Conclusion 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.

Adrenal infarction with latent myelodysplastic/myeloproliferative neoplasm, unclassifiable with JAK2V617F mutation

“…20 With reference to these case reports, other factors as well as JAK2V617F 2, including the current case, developed complications such as adrenal insufficiency and thromboses at other sites. Among the six patients, five had bilateral adrenal infarction 4,5,[20][21][22] and four developed primary adrenal insufficiency that required steroid replacement therapies. 4,[20][21][22] In our case, the adrenal infarction was unilateral and improved soon after edoxaban treatment.…”

“…adrenal gland. 4,22,25 When treating adrenal infarction, attention should be paid to adrenal hemorrhagic infarction. In particular, patients with MPN have an increased risk of bleeding owing to a decrease in vWF activity associated with an increase in the platelet count.…”

“… 4 , 5 Indeed, one patient developed adrenal infarction before the diagnosis of ET, indicating that JAK2 V617F‐CHIP was closely associated with adrenal infarction, as in our case. 4 However, one patient with MDS/MPN‐U did not harbor the JAK2 V617F mutation, 22 indicating that other gene mutations may contribute to the thrombogenesis in MDS/MPN‐U. Furthermore, one case with MDS had adrenal infarction with no other thrombotic predisposition, such as MPN, suggesting that the hypercoagulable state associated with MDS may contribute to thrombogenesis.…”

“…Among the six patients, five had bilateral adrenal infarction 4 , 5 , 20 , 21 , 22 and four developed primary adrenal insufficiency that required steroid replacement therapies. 4 , 20 , 21 , 22 In our case, the adrenal infarction was unilateral and improved soon after edoxaban treatment. Although our patient subsequently developed adrenal infarction contralaterally, the lesions of adrenal infarction were small; therefore, the patient did not develop adrenal insufficiency.…”

“…The mechanisms underlying adrenal infarction and adrenal hemorrhagic infarction are not fully understood; however, it is hypothesized that they are related to the unique vascular anatomy of the adrenal gland. 4 , 22 , 25 When treating adrenal infarction, attention should be paid to adrenal hemorrhagic infarction. In particular, patients with MPN have an increased risk of bleeding owing to a decrease in vWF activity associated with an increase in the platelet count.…”