Acquired von Willebrand syndrome in myeloproliferative neoplasms with extreme thrombocytosis

2021 ◽  
Author(s):  
Chiara Trotti ◽  
Emanuela Sant’Antonio ◽  
Daniele Vanni ◽  
Ilaria Carola Casetti ◽  
Oscar Borsani ◽  
...  
Keyword(s):  
Myeloproliferative Neoplasms ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

scholarly journals From Budd-Chiari syndrome to acquired von Willebrand syndrome: thrombosis and bleeding complications in the myeloproliferative neoplasms

Blood ◽  
2019 ◽  
Vol 134 (22) ◽  
pp. 1902-1911 ◽  
Author(s):  
Brady L. Stein ◽  
Karlyn Martin
Keyword(s):  
Myeloproliferative Neoplasms ◽  
Bleeding Complications ◽  
Budd Chiari Syndrome ◽  
Acquired Von Willebrand Syndrome ◽  
Chiari Syndrome ◽  
Appropriate Therapy ◽  
Von Willebrand

Stein and Martin provide a review of the thrombotic and bleeding complications of myeloproliferative neoplasms and provide a roadmap for appropriate therapy.

scholarly journals Fulminant Essential Thrombocythemia Associated with Acquired Von Willebrand Syndrome and Bleeding Episodes in a 14-year-old Girl

2019 ◽  
Vol 39 (04) ◽  
pp. 404-408 ◽  
Author(s):  
C. Schneider ◽  
E. Stutz-Grunder ◽  
S. Lüer ◽  
P. Keller ◽  
J. A. Kremer Hovinga ◽  
...  
Keyword(s):  
Essential Thrombocythemia ◽  
Clinical Symptoms ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Neoplasm ◽  
Driver Mutations ◽  
Acute Therapy ◽  
Acquired Von Willebrand Syndrome ◽  
Platelet Counts ◽  
Bleeding Episodes ◽  
Von Willebrand

Background Essential thrombocythemia is a chronic myeloproliferative neoplasm. It is extremely rare in children below 15 years of age with an estimated annual incidence of only 0.09 per million. Usually, clinical symptoms associated with essential thrombocythemia are mild or absent. Case Here, we present the case of a 14-year-old female patient fulminantly presenting with acute symptoms comprising visual impairment, palmar and plantar stabbing pain. Blood count revealed massive thrombocytosis of 2373 × 109/L. Bone marrow morphology showed elevated numbers of mature megakaryocytes. Von Willebrand factor activity/antigen ratio was significantly reduced compatible with an acquired Von Willebrand syndrome associated with high platelet counts. Molecular analyses for driver mutations of myeloproliferative neoplasms including JAK2V617F, CALR and MPL were negative. Acute therapy comprising hyperhydration and oxygen supply complemented by acetylsalicylic acid led to amelioration of symptoms. Medication with hydroxycarbamide maintained a significant reduction of platelet counts but had to be reduced or withheld several times due to neutropenia. Repeated bleeding episodes observed in the course were clearly associated with increases in platelet counts above 1200 × 109/L explained by acquired von Willebrand syndrome. Sixteen months after diagnosis, therapy was switched to pegylated interferon and platelet counts could be stabilized without significant side effects.

Pathogenesis of antibody-induced acquired von willebrand syndrome

1980 ◽  
Vol 9 (4) ◽  
pp. 363-371 ◽  
Author(s):  
Tong Eng Gan ◽  
Ronald J. Sawers ◽  
Jerry Koutts
Keyword(s):  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

Acquired von Willebrand Syndrome in Systemic Lupus Erythematodes

2001 ◽  
Vol 7 (2) ◽  
pp. 106-112 ◽  
Author(s):  
Jan Jacques Michiels ◽  
Wilfried Schroyens ◽  
Marc van der Planken ◽  
Zwi Bememan
Keyword(s):  
Lupus Erythematodes ◽  
Systemic Lupus ◽  
Acquired Von Willebrand Syndrome ◽  
Systemic Lupus Erythematodes ◽  
Von Willebrand

Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia

2015 ◽  
Vol 56 (12) ◽  
pp. 3452-3454 ◽  
Author(s):  
Mona Lisa Alattar ◽  
Maria Ciccone ◽  
Mahmoud R. Gaballa ◽  
Candida Vitale ◽  
Xavier C. Badoux ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Lymphocytic Leukemia ◽  
Bleeding Diathesis ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand
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