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

ijbmr-aid1011-g002

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

  • Transumbilical Single-incision Hiatal Hernia Repair and Nissen Fundoplication in situs Inversus Totalis: A Rare Case Report
    Qing Cao, Chen Kang, Kang Gu, Yin Peng, Yang Lv, Xu-Zhong Ding and Peng Li* Qing Cao,Chen Kang,Kang Gu,Yin Peng,Yang Lv,Xu-Zhong Ding,Peng Li*. Transumbilical Single-incision Hiatal Hernia Repair and Nissen Fundoplication in situs Inversus Totalis: A Rare Case Report. Adv Treat ENT Disord. 2026: doi: 10.29328/journal.ated.1001017; 10: 001-003
  • Intravenous Leiomyomatosis of the Uterus with Intracardiac Extension
    Tomas Reyes-del Castillo*, Minerva I Hernandez-Rejon, Jose L Ruiz-Pier, Mario Peñaloza-Guadarrama, Carlos E Merinos-Avila, Cristina Juarez-Cabrera, Pedro A del Valle-Maldonado, Sofia Ley-Tapia and Valentín Gonzalez-Flores Tomas Reyes-del Castillo*,Minerva I Hernandez-Rejon,Jose L Ruiz-Pier,Mario Peñaloza-Guadarrama,Carlos E Merinos-Avila,Cristina Juarez-Cabrera,Pedro A del Valle-Maldonado,Sofia Ley-Tapia,Valentín Gonzalez-Flores. Intravenous Leiomyomatosis of the Uterus with Intracardiac Extension. Arch Vas Med. 2025: doi: 10.29328/journal.avm.1001021; 9: 003-007
  • Identification, Molecular Confirmation, and Antibiotic Sensitivity of Bacteria Isolated from Repeat Breeder Cows in Rangpur Division
    Mst. Mousumi Afroj, Md Faruk Islam, Muhammad Mamunur Roshid, Md Shanto Hossain, Md Samiul Tousif, Omiaya Azam Oishi, Subroto Sarma and Begum Fatema Zohara* Mst. Mousumi Afroj,Md Faruk Islam,Muhammad Mamunur Roshid,Md Shanto Hossain,Md Samiul Tousif,Omiaya Azam Oishi,Subroto Sarma,Begum Fatema Zohara*. Identification, Molecular Confirmation, and Antibiotic Sensitivity of Bacteria Isolated from Repeat Breeder Cows in Rangpur Division. Insights Vet Sci. 2025: doi: 10.29328/journal.ivs.1001047; 9: 008-17
  • Adalimumab in the Treatment of Complex Sarcoidosis-related Inflammatory Eye Disease: A Case Series
    Mina Al-Awqati, Supritha Prasad*, Valeria Esparza, Jacqueline Jansz, Wuily Carpio, Christian Ascoli, Huan Chang, Pooja Bhat, Ann-Marie Lobo-Chan and Nadera Sweiss Mina Al-Awqati, Supritha Prasad*, Valeria Esparza, Jacqueline Jansz, Wuily Carpio, Christian Ascoli, Huan Chang, Pooja Bhat, Ann-Marie Lobo-Chan, Nadera Sweiss. Adalimumab in the Treatment of Complex Sarcoidosis-related Inflammatory Eye Disease: A Case Series. Arch Vas Med. 2024: doi: 10.29328/journal.avm.1001018; 8: 001-003
  • Male in Early Adolescence Presenting with Guillain-Barré Syndrome Following BECOV2D Vaccine
    Vinit Suri*, Kanika Suri, Kunal Suri and Priyal Vinit Suri*, Kanika Suri, Kunal Suri, Priyal. Male in Early Adolescence Presenting with Guillain-Barré Syndrome Following BECOV2D Vaccine. J Child Adult Vaccines Immunol. 2023: doi: 10.29328/journal.jcavi.1001010; 7: 001-004

Read More

Most Viewed

Read More

Help ?