Spontaneous heparin-induced thrombocytopenia syndrome presenting as bilateral adrenal infarction after knee arthroplasty

Abstract

Adrenal gland infarction resulting from adrenal vein thrombosis is an infrequently recognised entity with a limited differential diagnosis. When bilateral, it can result in acute life-threatening adrenal failure. Heparin-induced thrombocytopenia (HIT) is an antibody-mediated, prothrombotic state that represents an important cause of adrenal vein thrombosis leading to associated infarction. Sometimes, the clinical picture of HIT—including the presence of HIT antibodies—occurs despite absence of proximate heparin exposure (‘spontaneous HIT syndrome’). We report a case of nearly missed adrenal failure secondary to bilateral adrenal infarction that evolved during the second week following knee arthroplasty (a known trigger of spontaneous HIT syndrome). The combination of bilateral adrenal infarction, thrombocytopenia and presence of platelet-activating HIT antibodies not explainable by preceding heparin exposure led to a diagnosis of postknee arthroplasty spontaneous HIT syndrome. The case also highlights the clinical and laboratory findings associated with rapidly progressive acute adrenal failure.

“Spontaneous” Heparin-Induced Thrombocytopenia and Thrombosis After Total Knee Arthroplasty: A Report of 2 Cases

“…Why spontaneous HIT seems to occur preferentially in knee replacement cases is not established, but a plausible hypothesis is that during the surgery, polyanionic glycosaminoglycans from the knee joint are released into the systemic circulation; tourniquet release may conceivably deliver a bolus [10,11,13]. Polyanionic glycosaminoglycans from the knee joint are presumed to mimic UFH in structure, may bind platelet factor 4, and stimulate the antibody response triggering the cascade of intravascular platelet activation, aggregation, and thrombosis [13,15].…”

Unilateral suprarenal hemorrhage in a teenager: A case of TAFRO

“…HIT, though a very unusual entity in pediatric patients, 12 has been anecdotally reported in adults as a cause of suprarenal hemorrhage. [13][14][15][16][17] This however is an extremely rare diagnosis in the absence of heparin exposure (i.e., spontaneous HIT). HIT results in a hypercoagulable state 18,19 that required prompt management with alternative anticoagulants to control thrombi generation.…”

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

“…Adrenal infarction is a rare condition that occasionally develops in a hypercoagulable state 1 . 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 Antiphospholipid antibody syndrome, COVID-19 infection, and heparininduced thrombocytopenia have been reported as underlying diseases of adrenal infarction. [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.…”