Lupus Anticoagulant Hypoprothrombinemia Syndrome Associated with Bilateral Adrenal Hemorrhage in a Child: Early Diagnosis and Intervention

Abstract

Background: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients, usually associated with autoimmunity or infections. Pediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal hemorrhage due to LAHPS and its long-term clinical course have not been sufficiently described. Case presentation: A 9‐year‐old boy presented with prolonged abdominal pain and abnormal coagulation screening tests. The laboratory tests showed prolonged activated partial thromboplastin time and subsequently revealed the presence of lupus anticoagulant, anti-nuclear antibodies, and hypoprothrombinemia, leading to diagnosis of LAHPS. An enhanced computed tomogram demonstrated nodular lesions in the adrenal glands bilaterally, suggestive of adrenal hemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. Conclusions: The patient with LAHPS developed rare adrenal failure due to adrenal hemorrhage, a life-threatening event that should be recognized and treated early. In our case, renal dysfunction was also observed when systemic lupus erythematosus was diagnosed one year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful observation for patients with autoantibodies are required.

Lupus Anticoagulant-Hypoprothrombinemia Syndrome: Literature Review and Description of Local Case in a 3-Year-Old Chinese Girl

“…The detection rates of LA, aPT, aPS/PT, aCL and aβ2GPI in LAHPS patients were 100% (53/53), 58.3% (14/24), 1,5,15,22,24 50% (12/24), 1,5,6,15,21 47.8% (22/46), 1,2,5,[9][10][11][12][13]15,19,[22][23][24]26,27 and 47.4% (18/38), 1,5,9,11,15,19,[22][23][24]26,27 respectively. There were no significant differences in the detection rates of each of these antiphospholipid antibodies before versus after the age of 18 years (p > 0.05; ►Table 3).…”

“…Underlying Etiology ►Table 3 presents 33 valid cases with information, excluding 20 cases without a description of the underlying disease. Among these cases, 20 (60.6%) were associated with autoimmune diseases, 2,4,7,9,10,12,14,18,22,24-27 11 (33.3%) were related to infections, 1,6,[11][12][13]16,17,20,21,23 and 2 (6.1%) were linked to tumors. 8,15 The most prevalent autoimmune disease was SLE (14/20, 70.0%), while viruses were the most common etiologic agent of infectious diseases (6/11, 54.5%).…”

“…In contrast to the prothrombotic risk more often associated with LA, patients with LAHPS may experience varying degrees of bleeding, ranging from no bleeding to mild skin and mucosal bleeding and up to life-threatening bleeding. 1,2 The first report of LAHPS was published by Rapaport et al 3 in 1960, describing a severe bleeding case in an 11-year-old girl with systemic lupus erythematosus (SLE) and confirming significantly reduced prothrombin activity. Due to its rarity, there have been limited reports on LAHPS, with less than 200 cases documented in the literature over the past 60 years.…”

Association between Severe Acute Respiratory Syndrome Coronavirus 2 Infection (Coronavirus Disease 2019) and Lupus Anticoagulant-Hypoprothrombinemia Syndrome: A Case Report and Literature Assessment

“…LAHS is a rare acquired disorder caused by prothrombin antibodies. This condition should be suspected in a bleeding patient, more frequently in children 3 4 5 and women, 4 with prolonged PT and aPTT and a reduced factor II activity in combination with LA. The presence of antiphosphatidyl-serine/prothrombin antibodies explains the reduced level of factor II, likely due to a rapid clearance of the prothrombin-antibody complex.…”