A rare case of catastrophic antiphospholipid syndrome triggered by estrogen‐containing oral contraceptives in a patient with double heterozygous factor V Leiden and prothrombin <scp>G20210A</scp> mutations

McRae, Hannah L; Yang, Annie; Kruzer, Karen; Scott, Glynis; Refaai, Majed A.

doi:10.1002/ajh.26138

Cited by 4 publications

(3 citation statements)

References 12 publications

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“…Oral contraceptives assumption could also be an additive risk factor for CAPS occurrence ( 9 ), as presented by McRae and colleagues in their recent paper.…”

Section: Discussionmentioning

confidence: 93%

Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Pasqui

Camarri

Donato

et al. 2021

Front. Cardiovasc. Med.

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Introduction: Asherson's Syndrome, also defined as Catastrophic Antiphospholipid Syndrome (CAPS), represents the most severe manifestation of Antiphospholipid Antibody Syndrome. Rarely, the first CAPS diagnosis is based on macro-thrombotic event as acute limb ischemia.Case Presentation: We present a case of a 65-year-old woman admitted with an acute lower limb arterial ischemia with a complete occlusion of all the three tibial vessels. Three endovascular recanalization procedures were performed contemporary to 48 h intraarterial thrombolysis administration. The patency of tibial arteries was restored with a near-complete absence of digital arteries and microvessel perfusion of the foot. In the following days, an aggressive foot gangrene was established, leading to a major lower-limb amputation. Due to the general clinical status worsening and aggressiveness of ischemic condition, further investigations were performed leading to the diagnosis of an aggressive Asherson's Syndrome that was also complicated by a severe heparin-induced thrombocytopenia. Medical management with a high dose of intravenous steroids and nine sessions of plasma exchange led to a clinical condition stabilization.Conclusion: In our case, the presence of a “sine causa” acute arterial occlusion of a large vessel represented the first manifestation of an aggressive form of Asherson's Syndrome that could represent a fatal disease. Due to the extreme variety of manifestations, early clinical suspicion, diagnosis, and multidisciplinary management are essential to limit the life-threatening consequences of patients.

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“…Oral contraceptives assumption could also be an additive risk factor for CAPS occurrence ( 9 ), as presented by McRae and colleagues in their recent paper.…”

Section: Discussionmentioning

confidence: 93%

Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Pasqui

Camarri

Donato

et al. 2021

Front. Cardiovasc. Med.

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“…Patients with genetic thrombophilia may develop more severe forms of CAPS. McRae et al [ 47 ] described a case of CAPS in a 25-year-old female patient with the prothrombin gene mutation G20210A and heterozygous Leiden. She took combined oral contraceptives for 6 months.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Catastrophic Antiphospholipid Syndrome

Bitsadze,

Yakubova,

Khizroeva

et al. 2024

IJMS

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Unlike classic APS, CAPS causes multiple microthrombosis due to an increased inflammatory response, known as a “thrombotic storm”. CAPS typically develops after infection, trauma, or surgery and begins with the following symptoms: fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. Although the presence of antiphospholipid antibodies in the blood is one of the diagnostic criteria, the level of these antibodies can fluctuate significantly, which complicates the diagnostic process and can lead to erroneous interpretation of rapidly developing symptoms. Triple therapy is often used to treat CAPS, which includes the use of anticoagulants, plasmapheresis, and high doses of glucocorticosteroids and, in some cases, additional intravenous immunoglobulins. The use of LMWH is recommended as the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease that requires not only an interdisciplinary approach but also highly qualified medical care, adequate and timely diagnosis, and appropriate prevention in the context of relapse or occurrence of the disease. Improved new clinical protocols and education of medical personnel regarding CAPS can significantly improve the therapeutic approach and reduce mortality rates.

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“…6 Our patient has the additional risk factor of APS, a rare autoimmune disorder, which with concurrent double heterozygosity of FVL/PT-G20210A has been described in at least one case of catastrophic antiphospholipid syndrome as a contributor to disease. 7 For APS alone, there is conflicting evidence for the use of low-dose aspirin for primary prevention of arterial and venous thromboembolism. 2,8 Regarding secondary prevention of venous thromboembolism in patients with APS, as would apply to our patient, warfarin monotherapy is routinely utilized with a target international normalized ratio of 2.0-3.0.…”

Section: No Procedural Interventions Were Indicatedmentioning

confidence: 99%

Provoked deep vein thrombosis and saddle pulmonary embolism in a pediatric patient with multiple genetic risk factors for venous thromboembolism

Hofstee

Sartain

Shapiro

2022

Pediatric Blood & Cancer

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A rare case of catastrophic antiphospholipid syndrome triggered by estrogen‐containing oral contraceptives in a patient with double heterozygous factor V Leiden and prothrombin G20210A mutations

Cited by 4 publications

References 12 publications

Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Catastrophic Antiphospholipid Syndrome

Provoked deep vein thrombosis and saddle pulmonary embolism in a pediatric patient with multiple genetic risk factors for venous thromboembolism

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