Lupus anticoagulant-hypoprothrombinemia syndrome: A cerebral bleeding case report as systemic lupus erythematosus debut

Pérez, Miriam Lopez; Laso, Rosa Vidal; Velasco-Rodríguez, Diego; Martín-Herrero, Sara; Alfonzo, Inés Martinez; García-Raso, Aránzazu; Llamas-Sillero, Pilar

doi:10.1016/j.reuma.2022.02.007

Cited by 3 publications

(9 citation statements)

References 13 publications

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“…A total of 27 relevant articles with 53 LAHPS cases were identified as being published in the last 5 years, including our own case. Of these, 13 articles (48.1%) were from Asia, 1,2,4-13 10 (37.0%) were from Europe, [14][15][16][17][18][19][20][21][22][23] and 4 (14.8%) were from North America. [24][25][26][27]…”

Section: Literature Search Strategymentioning

confidence: 99%

“…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).…”

Section: Antiphospholipid Antibodymentioning

confidence: 99%

“…Out of these, 24 had bleeding at a single site, while 10 had bleeding at multiple sites. The most common bleeding sites were subcutaneous (n ¼ 16, 47.1%), 2,4,5,7,8,10 12-14,16,20,21,27 followed by the urinary system (n ¼ 9, 26.5%), 5,7,9,10,15,18,27 nose (n ¼ 8, 23.5%), 2,9-12,18 intracranial (n ¼ 6, 17.6%), 9,19,[22][23][24][25] gynecological (n ¼ 4, 11.8%), 7,9,26 gingival (n ¼ 2, 5.9%), 9,14 and intramuscular (n ¼ 2, 5.9%). 10,27 Subcutaneous hemorrhage presented as petechiae, ecchymosis, and purpura, while urinary system hemorrhage exhibited gross hematuria, microscopic hematuria, and adrenal hemorrhage.…”

Section: Bleeding Manifestations and Thrombosismentioning

confidence: 99%

“…15 The remaining 24 patients with bleeding were treated with immunomodulatory, 1,2,7-12,14,16-19,22-27 of which 23 with corticosteroids and 3 with intravenous immunoglobulin (IVIG). 11,22,25 Indicators of improvement were considered as the improvement of bleeding symptoms, absence of active bleeding, and normalization of factor II levels or prothrombin time at discharge or during follow-up. Out of the 32 cases for outcome information (►Table 5), 29 (90.6%) showed improvement, with 2 patients without bleeding, 12 patients with nonsevere bleeding, 8 patients with severe bleeding, and 7 patients with both degrees of bleeding.…”

Section: Treatment and Outcomementioning

confidence: 99%

“…Corticosteroids are commonly used as the first choice for immunosuppressive agents, and IVIG has been used as the initial therapy before elective surgery or after failure of the initial regimen. 21,22,25,36,42 It is important to note that thrombosis can occur in LAHPS patients who undergo steroid therapy and have their prothrombin time and factor II levels return to normal. Therefore, it is crucial to carefully balance the risks of thrombosis and bleeding.…”

Section: Treatment and Outcomementioning

confidence: 99%

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

Wang,

Tang,

Zhang

2024

Semin Thromb Hemost

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Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare, acquired coagulopathy syndrome. Here, we aim to summarize the clinical features of LAHPS to improve the understanding of the disease. The clinical data of 52 patients with LAHPS retrieved through PubMed from 2019 to 2023, supplemented with a local case of a child with LAHPS, were retrospectively analyzed, and the clinical characteristics were summarized. 56.6% of LAHPS patients were female, the median age at onset was 13.0 years (range, 1.2–85 years), and the median activity of factor II was 18.0% (range, 0.1–69%). 64.2% of LAHPS patients experienced hemorrhage, with 29.4% having multisite hemorrhage and 20.6% experiencing both nonsevere and severe hemorrhage. Most of the reported cases were secondary to autoimmune diseases (60.6%), followed by infections (33.3%). Corticosteroids were administered to 79.3% of patients with hemorrhage, and 90.6% of patients with LAHPS showed improvement. In conclusion, LAHPS is most commonly observed in female patients, particularly those under 18 years of age. LAHPS is characterized by hemorrhage, occurring at various sites and with varying degrees of severity, but the majority of patients improve with appropriate treatment and management.

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Section: Literature Search Strategymentioning

confidence: 99%

Section: Antiphospholipid Antibodymentioning

confidence: 99%

Section: Bleeding Manifestations and Thrombosismentioning

confidence: 99%

Section: Treatment and Outcomementioning

confidence: 99%

Section: Treatment and Outcomementioning

confidence: 99%

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

Wang,

Tang,

Zhang

2024

Semin Thromb Hemost

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In vitro differential inhibition of the factor XI activity assay in the setting of a lupus anticoagulant

Rabichow,

Dinh,

Nguyen

et al. 2024

Blood Coagulation &Amp; Fibrinolysis

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Acquired factor XI deficiencies due to factor-specific inhibitors are rare and may be associated with lupus anticoagulant. We report a 63-year-old male with suspected postsurgical bleeding, prior surgical site infection, an isolated prolonged activated partial thromboplastin time, and a positive lupus anticoagulant. Although the factor II assay was normal, factor VIII and IX assays initially demonstrated nonparallelism with factor activity that consistently increased to normal reference ranges with serial dilutions. A discrepancy in factor XI activity results was discovered when the in-house method demonstrated undetectable activity (<3%); send-out testing using different instrument/reagent combinations revealed the presence of factor XI activity between 70% and 76%. The patient received surgical follow-up and was subsequently discharged home. Given the differential in vitro inhibition of factor XI activity on our initial in-house testing, this case highlights the importance of recognizing factor assay interference in the presence of a known lupus anticoagulant inhibitor, with strategies to mitigate potentially erroneous results.

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Spontaneous Retroperitoneal Bleeding in a Patient with Systemic Lupus Erythematosus

Chen,

Yang

2023

Medicina

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Background and Objectives: Systemic lupus erythematosus (SLE) is a disease with multiple organ involvement, and spontaneous hemorrhage, especially perirenal hemorrhage, is rare. Case Presentation: We report the case of a 19-year-old teenager with SLE who experienced left flank pain and hypovolemic shock. Abdominal computed tomography revealed a large left retroperitoneal hematoma. Recurrent hypovolemic shock occurred despite the transcatheter arterial embolization of the left renal artery. Repetitive abdominal computed tomography results showed active hemorrhage. Result: An exploratory laparotomy was used to confirm descending colonic mesenteric artery bleeding, which was resolved. The patient needed temporary regular kidney replacement therapy for active lupus nephritis, which terminated one month after discharge. Conclusions: When patients with SLE experience acute abdominal pain, flank pain, or back pain combined with hypovolemia, there is a higher risk of bleeding due to spontaneous hemorrhage, which should be included in the differential diagnosis. Therefore, early diagnosis and adequate emergency intervention are necessary.

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Lupus anticoagulant-hypoprothrombinemia syndrome: A cerebral bleeding case report as systemic lupus erythematosus debut

Cited by 3 publications

References 13 publications

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

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

In vitro differential inhibition of the factor XI activity assay in the setting of a lupus anticoagulant

Spontaneous Retroperitoneal Bleeding in a Patient with Systemic Lupus Erythematosus

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