Atsushi Sakamoto1,2, Masao Ogura3, Atsushi Hattori4, Kinji Tada5, Reiko Horikawa4, Hisaya Nakadate6, Kimikazu Matsumoto7, Keiji Nogami8, Masahiro Ieko9, Akira Ishiguro5,6. 1. Center for Postgraduate Education and Training, National Center for Child Health and Development, NCCHD, Tokyo, Japan. sakamoto-a@ncchd.go.jp. 2. Children's Cancer Center, National Center for Child Health and Development, Setagaya-ku, Tokyo, 157-8535, Japan. sakamoto-a@ncchd.go.jp. 3. Division of Nephrology and Rheumatology, NCCHD, Tokyo, Japan. 4. Division of Endocrinology and Metabolism, NCCHD, Tokyo, Japan. 5. Center for Postgraduate Education and Training, National Center for Child Health and Development, NCCHD, Tokyo, Japan. 6. Division of Hematology, NCCHD, Tokyo, Japan. 7. Children's Cancer Center, National Center for Child Health and Development, Setagaya-ku, Tokyo, 157-8535, Japan. 8. Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan. 9. Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido, Ishikari-Tobetsu, Hokkaido, Japan.
Abstract
BACKGROUND: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients with autoimmune diseases or infections. Paediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal haemorrhage 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 haemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. CONCLUSIONS: This patient with LAHPS developed rare adrenal failure due to adrenal haemorrhage, 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 1 year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful long-term observation is required in patients with autoantibodies.
BACKGROUND:Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients with autoimmune diseases or infections. Paediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal haemorrhage 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 haemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. CONCLUSIONS: This patient with LAHPS developed rare adrenal failure due to adrenal haemorrhage, 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 1 year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful long-term observation is required in patients with autoantibodies.