Factors related to the development of acquired von Willebrand syndrome in patients with essential thrombocythemia and polycythemia vera

2017 ◽  
Vol 41 ◽  
pp. 49-54 ◽  
Author(s):  
A. Rottenstreich ◽  
G. Kleinstern ◽  
S. Krichevsky ◽  
D. Varon ◽  
D. Lavie ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

scholarly journals Factors Related to the Development of Acquired Von Willebrand Syndrome in Patients with Essential Thrombocythemia and Polycythemia Vera

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5466-5466
Author(s):  
Amihai Rottenstreich ◽  
Geffen Kleinstern ◽  
Svetlana Krichevsky ◽  
David Varon ◽  
David Lavie ◽  
...  
Keyword(s):  
Platelet Count ◽  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Multivariable Analysis ◽  
Jak2 V617f ◽  
Jak2 V617f Mutation ◽  
Acquired Von Willebrand Syndrome ◽  
Platelet Counts ◽  
V617f Mutation ◽  
Von Willebrand

Abstract Objective: We characterized acquired von Willebrand syndrome (AVWS) among essential thrombocythemia (ET) and polycythemia vera (PV) patients. Methods: A review of patients with ET or PV evaluated for AVWS. Results: Of 116 patients with ET, 64 (55%) developed AVWS; of 57 with PV, 28 (49%) developed AVWS. Median platelet counts of ET and PV patients who developed AVWS were 920 X 109/L and 679 X 109/L, respectively (P=0.01). Of patients who developed AVWS, 69.5% had platelet counts below 1000 X 109/L. Bleeding was more common in patients with AVWS, among both ET and PV patients (P<0.001). VWF:RCo levels and VWF:RCo/VWF:Ag ratio were lower among JAK2 V617F positive- vs. JAK2 V617F negative- ET patients (P=0.02 and P=0.002, respectively); whereas VWF:Ag levels were comparable (P=0.96). ET patients harboring the JAK2 V617F mutation were more likely to develop AVWS than were calreticulin-positive patients (70.3% vs. 45.7%, P=0.02), despite lower platelet counts (median 773 vs. 920 X 109/l, P=0.05). In multivariable analysis, age (β=0.26, P=0.002), platelet count (β=-0.38, P<0.001), hemoglobin level (β=-0.22, P=0.01) and JAK2 V617F mutation (β=-0.23, P=0.01) independently predicted VWF:RCo, among ET patients; whereas only platelet count predicted VWF:RCo among PV patients (β=-0.49, P<0.001). Conclusion: Among ET and PV patients, AVWS was common and associated with higher bleeding rates and higher platelet count; nonetheless, most AVWS patients had platelet counts under 1000 X 109/L. Thus, AVWS screening should be included in routine assessment of ET and PV patients. Among ET patients, JAK2 V617F was a main driver for the development of AVWS. Disclosures No relevant conflicts of interest to declare.

Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS)

2014 ◽  
Vol 101 (4) ◽  
pp. 405-410 ◽  
Author(s):  
Joanna Rupa-Matysek ◽  
Krzysztof Lewandowski ◽  
Maria Lewandowska ◽  
Ewelina Wojtasińska ◽  
Marzena Liliana Wojtaszewska ◽  
...  
Keyword(s):  
Essential Thrombocythemia ◽  
Positive Patient ◽  
Bleeding Complications ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

scholarly journals Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding

Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1746 ◽  
Author(s):  
Hassan Awada ◽  
Maria Voso ◽  
Paola Guglielmelli ◽  
Carmelo Gurnari
Keyword(s):  
Essential Thrombocythemia ◽  
Clinical Evidence ◽  
Antiplatelet Agents ◽  
Bleeding Risk ◽  
Clinical Approach ◽  
Evidence Based ◽  
Vascular Events ◽  
Acquired Von Willebrand Syndrome ◽  
The Past ◽  
Von Willebrand

Over the past decade, new insights have emerged on the pathophysiology of essential thrombocythemia (ET), its clinical management, and associated thrombohemostatic disturbances. Here, we review the latest diagnostic and risk stratification modalities of ET and its therapeutics. Moreover, we discuss the clinical evidence-based benefits, deriving from major clinical trials, of using cytoreductive therapy and antiplatelet agents to lower the risk of fatal vascular events. Also, we focus on the condition of extreme thrombocytosis (>1000 × 109/L) and bleeding risk, the development and pathogenesis of acquired von Willebrand syndrome, and the clinical approach to this paradoxical scenario in ET.

scholarly journals Increased von Willebrand factor levels in polycythemia vera and phenotypic differences with essential thrombocythemia

2020 ◽  
Vol 4 (3) ◽  
pp. 413-421
Author(s):  
Monica Sacco ◽  
Paola Ranalli ◽  
Stefano Lancellotti ◽  
Giovanna Petrucci ◽  
Alfredo Dragani ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Von Willebrand Factor ◽  
Essential Thrombocythemia ◽  
Phenotypic Differences ◽  
Von Willebrand ◽  
Willebrand Factor

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.

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