Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus

Stufano, Francesca; Baronciani, Luciano; Biguzzi, Eugenia; Cozzi, Giovanna; Colpani, Paola; Chisini, Marta; Peyvandi, Flora

doi:10.1111/hae.13626

Cited by 5 publications

(4 citation statements)

References 11 publications

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“…In AVWS secondary to SLE, the autoantibody against the FVIII/VWF complex is present, causing FVIII and VWF deficiency. 8 In our research, the 2 patients with AVWS accordingly exhibited reduced FVIII activity. However, the activity of FVIII in AHA patients was lower than that in AVWS patients.…”

Section: Discussionsupporting

confidence: 48%

“…The VWF is an adhesive protein that participates in hemostasis 7 and acts as the carrier protein of FVIII. In AVWS secondary to SLE, the autoantibody against the FVIII/VWF complex is present, causing FVIII and VWF deficiency 8 . In our research, the 2 patients with AVWS accordingly exhibited reduced FVIII activity.…”

Section: Discussionmentioning

confidence: 47%

“…In systemic lupus erythematosus (SLE)-related AH patients, the presence of inhibitory antibodies against coagulation factor VIII (FVIII) or von Willebrand factor (VWF) is involved in the pathogenesis. 7,8 The AHA, caused by antibodies against FVIII, is the most common pattern. The SLE accounts for 8.6% of autoimmune diseases-related AH.…”

mentioning

confidence: 99%

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Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus

Cao

Zhang

et al. 2019

J Clin Rheumatol

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ObjectiveBecause acquired hemophilia (AH) is a rare entity in systemic lupus erythematosus (SLE), we aimed to investigate the clinical features of SLE-related AH in Chinese patients.MethodsThis is a medical records review study carried out at a large tertiary care hospital in China from years 1986 to 2018. We searched the case database in Peking Union Medical College Hospital using the International Classification of Diseases. The clinical data on SLE-related AH patients were collected.ResultsA total of 9282 SLE patients had been hospitalized. Six female SLE-related AH patients were identified. Four patients had acquired hemophilia A (AHA), and 2 patients had acquired von Willebrand syndrome. Their mean age was 33.67 ± 13.77 years. Five patients had active disease. The mean SLE disease activity index measured at the time of diagnosis of AH was 10.50 ± 5.28. The average level of activated partial thromboplastin time was 86.5 seconds. Coexistence of secondary antiphospholipid syndrome and AHA was found in one case, and pulmonary embolism was observed 3 years later. After immunosuppressive therapy and symptomatic treatment, an overall remission rate of 83.3% was achieved.ConclusionsThe frequency of SLE-related AH was low. The development of AH in SLE patients frequently occurs with active disease. The AH could be the first clinical presentation of SLE. Secondary antiphospholipid syndrome and AHA could appear in the same SLE patient. Early and aggressive treatment contributes to a favorable prognosis.

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Section: Discussionsupporting

confidence: 48%

Section: Discussionmentioning

confidence: 47%

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Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus

Cao

Zhang

et al. 2019

J Clin Rheumatol

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“…Additionally, AVWS is associated with many other underlying disorders, such as solid tumors, hematological cancers, and autoimmune diseases. More than 20 patients with AVWS and SLE have been reported (7)(8)(9)(10).…”

Section: Discussionmentioning

confidence: 99%

Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage

Wang

Hu²,

Chen³

et al. 2022

Front. Pediatr.

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BackgroundAcquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary von Willebrand disease (VWD). AVWS is secondary to other diseases, and systemic lupus erythematosus (SLE) is a relatively rare cause.Case presentationWe report a case of AVWS as onset clinical presentation of SLE manifested as epistaxis and pulmonary hemorrhage. A 13-year-old male child presented to the hospital with a six-month history of recurrent epistaxis and a one-month history of anemia. Routine blood tests demonstrated severe normocytic anemia and normal platelet count. Von Willebrand test revealed a significantly lower level. High-resolution chest computed tomography (CT) showed patchy ground glass opacities consistent with hemorrhagic changes. After ruling out the family history, the patient was diagnosed with AVWS. Additional tests confirmed positive antinuclear and anti-Sm antibodies. The underlying SLE was diagnosed and treated with methylprednisolone with disease recovery.ConclusionWe recommend screening for bleeding disorders in patients with recurrent epistaxis. AVWS should be considered when laboratory findings suggest hereditary von Willebrand disease without a personal or familial history of bleeding. In addition, the underlying disease should be explored.

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Bleeding and Clotting Disorders

Napeñas¹,

Patton²

2021

Burket's Oral Medicine

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Dental health care workers are increasingly called upon to provide quality dental care to individuals whose bleeding and clotting mechanisms have been altered by inherited or acquired diseases. This provides an opportunity for the dentist who is trained in the recognition of oral and systemic signs of altered hemostasis to assist in the diagnosis of the underlying condition. A number of dental procedures result in the risk of bleeding that can have serious consequences, such as severe hemorrhage or possibly death, for the patient with a bleeding disorder. Safe dental care may require consultation with the patient's physician, systemic management, and dental treatment modifications.Of the inherited coagulopathies, von Willebrand's disease (vWD) is the most common. It results from deficiency of von Willebrand's factor (vWF) and affects about 0.8 to 1% of the population. 1 Hemophilia A, caused by coagulation factor (F) VIII deficiency, is the next most common, followed by hemophilia B, a F IX deficiency. The age-adjusted prevalence of hemophilia in six surveillance states in 1994 was 13.4 cases in 100,000 males (10.5 for hemophilia A and 2.9 for hemophilia B). 2 Application to the US population resulted in an estimated national prevalence of 13,320 cases of hemophilia A and 3,640 cases of hemophilia B, with an incidence rate of 1 per 5,032 live male births. Hemophilia A was predominant, accounting for 79% of all hemophiliacs, and prevalence of disease severity was 43% severe (< 1% F VIII), 26% moderate (1-5% F VIII), and 31% with mild (6-30% F VIII) disease. 2 Acquired coagulation disorders can result from drug actions or side effects, or underlying systemic disease. A stratified household sample of 4,163 community residents aged 65 years or older living in a five-county area of North Carolina revealed 51.7% to be taking one or more medications (aspirin, warfarin, dipyridamole, nonsteroidal anti-inflammatory drugs

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Severe acquired von Willebrand syndrome secondary to systemic lupus erythematosus

Cited by 5 publications

References 11 publications

Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus

Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus

Case report: A case of acquired von Willebrand syndrome as onset clinical presentation of systemic lupus erythematosus manifested as epistaxis and pulmonary hemorrhage

Bleeding and Clotting Disorders

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