Child Kidney Dis.  2024 Jun;28(2):80-85. 10.3339/ckd.24.007.

Lupus anticoagulant hypoprothrombinemia syndrome associated with a hemorrhagic ovarian cyst in a girl with systemic lupus erythematosus: a case report

• Son MH
• Yim HE

• KMID: 2557074
• DOI: http://doi.org/10.3339/ckd.24.007

Abstract

Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare entity characterized by the presence of lupus anticoagulant (LA) and prothrombin (factor II) deficiency. It may cause severe bleeding contrary to classical antiphospholipid syndrome. Here, we report a case of LAHPS presenting with a hemorrhagic ovarian cyst in a 17-year-old girl with systemic lupus erythematosus (SLE) nephritis. She had been followed up for 8 years. Her first manifestation of SLE was prolonged gingival bleeding after tooth extraction at 9 years of age. During the follow-up period, she had neither severe bleeding nor thrombotic complications despite a positive LA and a prolonged activated partial thromboplastin time (aPTT). At this visit, the patient presented with colicky abdominal pain, a hemorrhagic ovarian cyst, a prolonged prothrombin time, a prolonged aPTT, a low factor II level, and a positive LA, leading to the diagnosis of LAHPS. While a hemorrhagic ovarian cyst resolved completely in 3 months, she received oral pill, transfusions of red blood cells and plasma, and intravenous cyclophosphamide pulse therapy in combination with glucocorticoids due to persistent menorrhagia, anemia, prolonged aPTT, and lupus flaring. Thus, LAHPS needs to be considered in SLE patients with positive LA and prolonged aPTT.

Keyword

Case reports; Hemorrhage; Hypoprothrombinemias; Lupus coagulation inhibitor; Lupus nephritis

Figure

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  Fig. 1. Abdominal ultrasonography and pelvic computed tomography. (A) A 4.9×4.1×5 cm heterogeneous mass (arrow) in the uterus on abdominal ultrasonography. (B) A hemorrhagic cyst (arrow) in the right ovary on pelvic computed tomography. (C) After 3 months, the size of right ovarian cystic lesion (arrow) is reduced on abdominal ultrasonography.

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  Fig. 2. Second kidney biopsy findings on October 2020. (A) Immunofluorescence images showing a full house pattern in the mesangial region and capillary walls. (B-D) Segmental endocapillary hypercellularity (red arrow) and double contouring with subendothelial deposits in the glomerular capillary walls (blue arrow). (E-G) Electron microscopic findings with the presence of subendothelial and subepithelial deposits and foot process effacement (red arrow). B, C: Hematoxylin and eosin stain, ×400; D: Masson-Trichrome stain, ×400; E: ×2,000; F: ×5,000; G: ×10,000.

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  Fig. 3. Clinical courses of the patient. Proteinuria (orange line), aPTT (blue line), factor II levels, the presence of LA, and bleeding episodes of the patient from April 2012 to March 2022. Epistaxis on July 2014 and September 2020, a hemorrhagic ovarian cyst on December 2018, and menorrhagia from December 2018.12 to April 2019. MMF, mycophenolate mofetil; aPTT, activated partial thromboplastin time; CPM, cyclophosphamide; LA, lupus anticoagulant.

Reference

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