Recurrent epistaxis with coagulation disorders in a boy aged 2 years
LI Jia-Zhuo, TIAN Xin, LIAO Chu-Shu, HE Xiang-Ling, ZHU Cheng-Guang
Department of Hematology and Oncology, First Affiliated Hospital of Hunan Normal University/Children's Medical Center, Hunan Provincial People's Hospital, Changsha 410005, China
Abstract A boy, aged 2 years and 5 months, had recurrent epistaxis, and the coagulation function examination showed that activated partial thromboplastin time (APTT) was significantly prolonged. Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test, with positive lupus anticoagulant and low prothrombin activity. The boy was diagnosed with hypoprothrombinemia-lupus anticoagulant syndrome. The condition was improved after treatment with glucocorticoid, immunoglobulin, and vitamin K1. The boy has been followed up for 6 months, and no epistaxis was observed. Prothrombin activity returned to normal, and lupus anticoagulant remained positive. This is a relatively rare disease, and for patients with bleeding symptoms and coagulation disorders, it is recommended to perform the tests such as APTT correction test, lupus anticoagulant testing, and coagulation factor dilution test, which can improve the detection rate of this disease, so as to achieve early diagnosis, provide rational treatment in the early stage, and improve the prognosis.
Meireles E, Machado F, Teles L, et al. A case report of severe bleeding due to lupus anticoagulant hypoprothrombinemia syndrome[J]. J Thromb Thrombolysis, 2020, 49(2): 334-336. PMID: 31515751. DOI: 10.1007/s11239-019-01955-1.
Sakamoto A, Ogura M, Hattori A, et al. Lupus anticoagulant hypoprothrombinemia syndrome associated with bilateral adrenal haemorrhage in a child: early diagnosis and intervention[J]. Thromb J, 2021, 19(1): 19. PMID: 33731125. PMCID: PMC7968225. DOI: 10.1186/s12959-021-00271-0.
Kocheril AP, Vettiyil GI, George AS, et al. Pediatric systemic lupus erythematosus with lupus anticoagulant hypoprothrombinemia syndrome—a case series with review of literature[J]. Lupus, 2021, 30(4): 641-648. PMID: 33509039. DOI: 10.1177/0961203321988934.
Jin J, Zehnder JL. Prozone effect in the diagnosis of lupus anticoagulant for the lupus anticoagulant-hypoprothrombinemia syndrome[J]. Am J Clin Pathol, 2016, 146(2): 262-267. PMID: 27473743. DOI: 10.1093/ajcp/aqw106.
Rapaport SI, Ames SB, Duvall BJ. A plasma coagulation defect in systemic lupus erythematosus arising from hypoprothrombinemia combined with antiprothrombinase activity[J]. Blood, 1960, 15: 212-227. PMID: 14436248.
Bajaj SP, Rapaport SI, Fierer DS, et al. A mechanism for the hypoprothrombinemia of the acquired hypoprothrombinemia-lupus anticoagulant syndrome[J]. Blood, 1983, 61(4): 684-692. PMID: 6403077.
Knobe K, Tedg?rd U, Ek T, et al. Lupus anticoagulants in two children—bleeding due to nonphospholipid-dependent antiprothrombin antibodies[J]. Eur J Pediatr, 2012, 171(9): 1383-1387. PMID: 22527568. DOI: 10.1007/s00431-012-1737-1.
Fleck RA, Rapaport SI, Rao LV. Anti-prothrombin antibodies and the lupus anticoagulant[J]. Blood, 1988, 72(2): 512-519. PMID: 2456797.
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