Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma

Autor: Britta A P Laros-van Gorkom, M.R. Nijziel, Dominique P M S M Maas, Waander L. van Heerde, Marjan Cruijsen, Sanne Gianotten
Rok vydání: 2018
Předmět:
Pathology
medicine.medical_specialty
congenital
hereditary
and neonatal diseases and abnormalities
Population
Vascular damage Radboud Institute for Health Sciences [Radboudumc 16]
Case Report
030204 cardiovascular system & hematology
Pathogenesis
03 medical and health sciences
0302 clinical medicine
All institutes and research themes of the Radboud University Medical Center
hemic and lymphatic diseases
Von Willebrand disease
Medicine
education
GeneralLiterature_REFERENCE(e.g.
dictionaries
encyclopedias
glossaries)
education.field_of_study
business.industry
lcsh:RC633-647.5
Other Research Radboud Institute for Health Sciences [Radboudumc 0]
Autoantibody
General Medicine
Inguinal lymphadenopathy
lcsh:Diseases of the blood and blood-forming organs
Mixing study
medicine.disease
medicine.anatomical_structure
Mantle cell lymphoma
Bone marrow
medicine.symptom
business
030215 immunology
circulatory and respiratory physiology
Rare cancers Radboud Institute for Health Sciences [Radboudumc 9]
Zdroj: Case Reports in Hematology, 2018, pp.
Case Reports in Hematology
Case Reports in Hematology, 2018,
Case Reports in Hematology, Vol 2018 (2018)
ISSN: 2090-6560
Popis: 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.
Databáze: OpenAIRE
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