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Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma
المؤلفون المشاركون
Maas, Dominique
Laros-van Gorkom, Britta
Gianotten, Sanne
Cruijsen, Marjan
van Heerde, Waander
Nijziel, Marten
المصدر
العدد
المجلد 2018، العدد 2018 (31 ديسمبر/كانون الأول 2018)، ص ص. 1-3، 3ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2018-01-30
دولة النشر
مصر
عدد الصفحات
3
التخصصات الرئيسية
الملخص EN
We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a mantle cell lymphoma.
A 61-year-old male suffered from recurrent bleeding symptoms since a few months.
Initially, physical examination was normal.
von Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were low (0.31 IU/ml and 0.43 IU/ml, resp.).
Ristocetin cofactor activity (VWF:RCo) was 0.09 IU/ml, and collagen binding activity (VWF:CB) was 0.10 IU/ml.
VWF:RCo/VWF:Ag ratio was 0.29, and RIPA value was normal.
Highest molecular weight VWF multimers were absent.
A diagnosis of von Willebrand Disease (VWD) type 2A was made.
However, no genetic mutation was found.
No inhibitory antibodies against VWF or factor VIII were detected.
A few months later, cervical, axillary, and inguinal lymphadenopathy was found on physical examination.
A CT scan confirmed multiple enlarged lymph nodes, and a clonal B-cell population matching a mantle cell lymphoma was detected in the bone marrow.
Chemoimmunotherapy resulted in a very good partial remission and concomitantly in a rapid decrease of bleeding problems and complete normalization of FVIII:C and VWF:Ag.
The diagnosis of AVWS cannot be rejected by negative mixing studies due to difficulties in the detection of autoantibodies and because of a highly heterogeneous pathogenesis of AVWS.
When the suspicion of AVWS is high, an extensive investigation should be performed to find the underlying cause.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Maas, Dominique& Laros-van Gorkom, Britta& Gianotten, Sanne& Cruijsen, Marjan& van Heerde, Waander& Nijziel, Marten. 2018. Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. Case Reports in Hematology،Vol. 2018, no. 2018, pp.1-3.
https://search.emarefa.net/detail/BIM-1144041
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Maas, Dominique…[et al.]. Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. Case Reports in Hematology No. 2018 (2018), pp.1-3.
https://search.emarefa.net/detail/BIM-1144041
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Maas, Dominique& Laros-van Gorkom, Britta& Gianotten, Sanne& Cruijsen, Marjan& van Heerde, Waander& Nijziel, Marten. Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. Case Reports in Hematology. 2018. Vol. 2018, no. 2018, pp.1-3.
https://search.emarefa.net/detail/BIM-1144041
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1144041
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
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