scholarly journals Glanzmann thrombasthenia complicated by frequent myeloproliferative neoplasm‐related thromboembolism: thrombosis occurring regardless of αIIbβIII integrin deficiency

2021 ◽  
Vol 9 (9) ◽  
Author(s):  
Roger E. G. Schutgens ◽  
Rolf T. Urbanus
Author(s):  
Roger Schutgens ◽  
Rolf Urbanus

We present a patient with congenital Glanzmann Thrombasthenia, who developed recurrent venous thrombosis. Over time, she developed the clinical picture of a myeloproliferative neoplasm, being JAK2 positive. This case clearly indicates that the platelet aIIbβIII integrin (lacking in Glanzmann thrombasthenia) does not have a role in thrombosis in MPN


2000 ◽  
Vol 10 (4) ◽  
pp. 326-327
Author(s):  
C. Kaplan ◽  
F. Bianchi ◽  
V. Jallu

2019 ◽  
Author(s):  
K. von Bargen ◽  
M.-J. Kraus ◽  
E.F. Strasser ◽  
G. Kappert ◽  
S. Halimeh ◽  
...  

2019 ◽  
Vol 70 (8) ◽  
pp. 2822-2825 ◽  
Author(s):  
Cornel Moisa ◽  
Mihnea Alexandru Gaman ◽  
Camelia Cristina Diaconu ◽  
Amelia Maria Gaman

Essential thrombocythemia (ET) is a BCR-ABL1-negative myeloproliferative neoplasm associated with thrombotic and haemorrhagic complications. Reactive oxygen species (ROS) overexpression induces a growth advantage to JAK2V617F-positive clones and, in association with a higher number of immature platelets, leukocytosis, and additional cardiovascular risk factors, leads to an increased risk for thrombotic events. We evaluated oxidative stress by measuring ROS levels and the total antioxidant capacity (TAC) in 62 ET patients and investigated the relationship between oxidative stress, JAK2V617F mutational status and the development of thrombotic events. We found higher oxidative stress levels in JAK2V617F-positive vs. JAK2V617F-negative ET cases with no significant differences between homozygous and heterozygous genotypes. Increased ROS levels and thrombotic events were more frequent in ET patients with old age at diagnosis, higher haematocrit levels or leukocytosis.


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