Figure 2

Novel European Asiatic Clinical, Laboratory, Molecular and Pathobiological (2015-2020 CLMP) criteria for JAK2V617F trilinear polycythemia vera (PV), JAK2exon12 PV and JAK2V617F, CALR and MPL515 thrombocythemias: From Dameshek to Constantinescu-Vainchenker, Kralovics and Michiels

Jan Jacques Michiels*, King H Lam, Fibo Ten Kate, Dong-Wook Kim, Myungshin Kim, Vasily Shuvaev, Francisca Valster, Vincent Potters, Wilfried Schroyens, Mihaela Andreescu, Adrian Trifa, Achille Pich and Hendrik De Raeve

Published: 03 April, 2020 | Volume 3 - Issue 1 | Pages: 001-020


Figure 2:

The heterozygous JAK2V617F mutated acquired thrombocythemia and germline TPO and JAK2V671I or JAK2Q534R mutated hereditary thrombocythemia as well as acquired MPL515 and congenital hereditary MPL505 mutated thrombocythemia (ET) are driven by indirect cytokine activation heterozygous JAK2V617F→STAT5, germline JAK2 or TPO→TpoR = MPL. Direct binding of TPO to the D3D4 domain of TpoRor direct cytokine activation (MPL515 and MPL505) cytokine receptor activation induce an ET phenotype of MPN without features of PV (EEC negative) Michiels, et al. 2014 [27]. CALR mutants Type 1 and 2, but not wild type CALR, did induce STAT5 activation via TpoR (MPL) and GCSFR, but not via EpoR. The STAT5 activation via GSCFR was much weak erthan via TpoR (MPL). The extracelllar domain of TpoR (MPL), but not of EpoR, was indispensible for CALR mutant induced activity and the D1D2 distal part of the extracelluar TpoR domain and its associated N-glycosylation sites but not the TPO binding site in the D3D4 domain Araki, et al. Vainchenker and Kralovics 2017) [65].

Read Full Article HTML DOI: 10.29328/journal.ijbmr.1001011 Cite this Article Read Full Article PDF

More Images

Similar Articles

Recently Viewed

Read More

Most Viewed

Read More

Help ?