Vol 52, No 4 (2021)
Review article
Published online: 2021-08-31
Calreticulin, a multi-faceted protein: thrombotic and bleeding risks in CALR mutation positive essential thrombocythemiaa
1
DOI: 10.5603/AHP.2021.0055
Acta Haematol Pol 2021;52(4):284-290.
Abstract
Essential thrombocythemia (ET) is a clonal disorder of a multipotent hematopoietic progenitor cell. In most patients, a driving mutation of Janus kinase 2 gene, calreticulin gene or myeloproliferative leukemia virus oncogene is detected. The occurrence of thrombotic and/or bleeding complications is very typical in manifestations of ET, with many cases of both occurring in the same patient. The thrombotic or bleeding phenotype can be a consequence of the coexistence of driving and non-driving molecular mutations and polymorphisms, affecting the platelet number and function. This paper discusses the nature of this disease, paying special attention to calreticulin gene function.
Keywords: CALRJAK2MPLacetylsalicylic acidthrombosisbleedingERp57calnexin pathwaystore-operated calcium entryplatelet function
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