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

Chinese Journal of Contemporary Pediatrics ›› 2022, Vol. 24 ›› Issue (7) : 817-820.

PDF(499 KB)
PDF(499 KB)
Chinese Journal of Contemporary Pediatrics ›› 2022, Vol. 24 ›› Issue (7) : 817-820. DOI: 10.7499/j.issn.1008-8830.2203060
CASE ANALYSIS

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
Author information +
History +

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.

Key words

Hypoprothrombinemia-lupus anticoagulant syndrome / Coagulation function / Epistaxis / Child

Cite this article

Download Citations
LI Jia-Zhuo, TIAN Xin, LIAO Chu-Shu, HE Xiang-Ling, ZHU Cheng-Guang. Recurrent epistaxis with coagulation disorders in a boy aged 2 years[J]. Chinese Journal of Contemporary Pediatrics. 2022, 24(7): 817-820 https://doi.org/10.7499/j.issn.1008-8830.2203060

References

1 中华医学会血液学分会血栓与止血学组, 中国血友病协作组. 罕见遗传性出血性疾病诊断与治疗中国专家共识(2021年版)[J]. 中华血液学杂志, 2021, 42(2): 89-96. PMID: 33858037. PMCID: PMC8071661. DOI:10.3760/cma.j.issn.0253- 2727.2021.02.001.
2 中华医学会血液学分会血栓与止血学组, 中国血友病协作组. 凝血因子Ⅷ/Ⅸ抑制物诊断与治疗中国指南(2018年版)[J]. 中华血液学杂志, 2018, 39(10): 793-799. PMID: 30369197. PMCID: PMC7348289. DOI: 10.3760/cma.j.issn.0253- 2727.2018.10.001.
3 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.
4 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.
5 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.
6 Kim JS, Kim MJ, Bae EY, et al. Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome[J]. Korean J Pediatr, 2014, 57(4): 202-205. PMID: 24868219. PMCID: PMC4030123. DOI: 10.3345/kjp.2014.57.4.202.
7 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.
8 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.
9 华宝来, 范连凯, 李梦涛, 等. 低凝血酶原血症-狼疮抗凝物综合征: 1例报道并文献复习[J]. 血栓与止血学, 2010, 16(2): 82-85. DOI: 10.3969/j.issn.1009-6213.2010.02.011.
10 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.
11 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.
12 Fleck RA, Rapaport SI, Rao LV. Anti-prothrombin antibodies and the lupus anticoagulant[J]. Blood, 1988, 72(2): 512-519. PMID: 2456797.
PDF(499 KB)

Accesses

Citation

Detail

Sections
Recommended

/