open access

Vol 52, No 4 (2021)
Review article
Published online: 2021-08-31
Submitted: 2021-04-25
Accepted: 2021-05-12
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Calreticulin, a multi-faceted protein: thrombotic and bleeding risks in CALR mutation positive essential thrombocythemiaa

Krzysztof Lewandowski1
DOI: 10.5603/AHP.2021.0055
·
Acta Haematol Pol 2021;52(4):284-290.
Affiliations
  1. Department of Hematology and Bone Marrow Transplantation, Karol Marcinkowski University of Medical Sciences, Szamarzewskiego 84, 60-569 Poznań, Poland

open access

Vol 52, No 4 (2021)
REVIEW ARTICLE
Published online: 2021-08-31
Submitted: 2021-04-25
Accepted: 2021-05-12

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.

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.

Get Citation

Keywords

CALR, JAK2, MPL, acetylsalicylic acid, thrombosis, bleeding, ERp57, calnexin pathway, store-operated calcium entry, platelet function

About this article
Title

Calreticulin, a multi-faceted protein: thrombotic and bleeding risks in CALR mutation positive essential thrombocythemiaa

Journal

Acta Haematologica Polonica

Issue

Vol 52, No 4 (2021)

Article type

Review article

Pages

284-290

Published online

2021-08-31

DOI

10.5603/AHP.2021.0055

Bibliographic record

Acta Haematol Pol 2021;52(4):284-290.

Keywords

CALR
JAK2
MPL
acetylsalicylic acid
thrombosis
bleeding
ERp57
calnexin pathway
store-operated calcium entry
platelet function

Authors

Krzysztof Lewandowski

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