A Teenage Boy with Systemic Lupus Erythematosus Complicated with Acquired von Willebrand Syndrome: A Rare Case and Challenging in Making Diagnosis
https://doi.org/10.47836/mjmhs.19.3.50
- Author:
Noor Hayati Sabtu
1
,
2
;
Faridah Idris
2
;
Eusni Rahayu Tohit
2
;
Azlinda Abu Bakar
1
;
Wan Aswani Wan Yusof
1
;
Raudhawati Osman
1
Author Information
1. Haematology Unit, Department of Pathology, Hospital Melaka, Jalan Mufti Haji Khalil, 75400, Melaka&
2. Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, 43400, UPM Serdang, Selangor
- Publication Type:Case Reports
- Keywords:
Systemic lupus erythematosus (SLE), Acquired von Willebrand syndrome (AVWS), von Willebrand factor (VWF)
- From:Malaysian Journal of Medicine and Health Sciences
2023;19(No.3):386-389
- CountryMalaysia
- Language:English
-
Abstract:
In systemic lupus erythematosus (SLE), haematological abnormalities are frequent, although they are an uncommon
cause of acquired von Willebrand syndrome (AVWS). AVWS is a rare condition that can cause a bleeding disorder.
We presented a case of AVWS in the early diagnosis of SLE. One month before admission, the patient had a history of recurrent epistaxis. He presented to the hospital with symptomatic anaemia and was noted to have severe
anaemia with iron deficiency. During hospitalisation, recurrent epistaxis recurred and was found to have prolonged
activated partial thromboplastin time (aPTT), presence of lupus anticoagulant (LA), and lower von Willebrand factor
(VWF), and factor 8 (VIII) levels. Simultaneously, he was diagnosed with SLE based on Systemic Lupus International
Collaborating Clinics (SLICC) criteria. He underwent blood transfusions and was treated with immunosuppressive
drugs such as steroids, mycophenolate mofetil, and an anti-fibrinolytic agent; he subsequently stopped bleeding and
showed clinical improvement.
- Full text:11.2023my1550.pdf
