Bilateral Adrenal Infarction That Developed in Latent Essential Thrombocythemia

Abstract

Bilateral adrenal infarction is an extremely rare disease, and it has been reported that some coagulation abnormalities, including essential thrombocythemia (ET), exist in the background. We herein report a 76-year-old patient in whom the platelet count had been in the normal range at the onset of adrenal infarction but subsequently increased to 102×10 4 /μL at 7 months later, leading to the diagnosis of JAK2V617F-positive ET. As the presence of the JAK2V617F mutation increases the risk of thrombosis, Janus kinase 2 (JAK2) genetic testing should be considered in some cases of nonspecific unknown thrombosis, even if there are no obvious hematological findings, such as clonal hematopoiesis of indeterminate potential (CHIP).

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

“…Among these cases, two patients with ET had JAK2V617F mutations. 4,5 Indeed, one patient developed adrenal infarction before the diagnosis of ET, indicating that JAK2V617F-CHIP was closely associated with adrenal infarction, as in our case. 4 However, one patient with MDS/MPN-U did not harbor the JAK2V617F mutation, 22 indicating that other gene mutations may contribute to the thrombogenesis in MDS/MPN-U.…”

“…Previously, five cases of myeloid neoplasms that developed into adrenal infarctions have been reported (Table 2). Among these cases, two patients with ET had JAK2 V617F mutations 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 .…”

“…Although the six cases in Table 2 did not develop adrenal hemorrhagic infarction, they sometimes occurred after adrenal infarction. 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.…”

“…Antiphospholipid antibody syndrome, COVID‐19 infection, and heparin‐induced thrombocytopenia have been reported as underlying diseases of adrenal infarction 1–3 . Adrenal infarctions have also been rarely linked to myeloproliferative neoplasms (MPN), such as polycythemia vera (PV) and essential thrombocythemia (ET) 4,5 …”

“…[1][2][3] Adrenal infarctions have also been rarely linked to myeloproliferative neoplasms (MPN), such as polycythemia vera (PV) and essential thrombocythemia (ET). 4,5 Myelodysplastic/myeloproliferative neoplasm (MDS/MPN) is a hematological disorder that exhibits characteristics of both MDS and MPN. 6,7 According to the revised version of the fourth edition of the World…”