Increased Erythrocyte Count on Top of Bone Marrow Histology but not Serum EPO Level or JAK2 Mutation Load Discriminates between JAK2V617F Mutated Essential Thrombocythemia and Polycythemia Vera

Michiels, Jan; Medinger, Michael

doi:10.4172/2329-8790.1000s1-001

Cited by 3 publications

(5 citation statements)

References 22 publications

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“…[15] The bone marrow pathological findings in the present study of CALR mutated thrombocythemia and MF in Figures 7-10 show dense clusters of large immature megakaryocytes with immature cloud-like nuclei, which clearly differ from the pleomorphic megakaryocytes in JAK2 V617F mutated ET and PV, which indeed are clearly distinct from the giant megakaryocytes with hyperlobulated staghorn-like nuclei in MPL 515 mutated ET [Figures 1-4]. [6] Since 2014 Michiels et al demonstrated that cases of hypercellular ET associated with PMGM belong to the third MPN of CALR-mutated thrombocythemia and MF without features of PV at time and Calreticulin mutated clonal myeloproliferative neoplasms (MPNs), JAK2 exon 12 mutated erythrocytosis and polycythemia vera and triple negative MPN [6,[14][15][16][17][18][19][20] a c d b of diagnosis and during follow-up. [6,[14][15][16][17][18][19][20] The updated 2018 WHO -Clinical Laboratory Molecular and Pathological (CLMP) criteria define five clonal mutually exclusive MPNs and transitional states at the laboratory and bone marrow level [Figure 11]: JAK2 V617F mutated ET, prodromal PV, classical and advanced PV; JAK2 exon 12 mutated idiopathic erythrocytosis and PV; MPL 515 mutated thrombocythemia and MF; and CALR mutated thrombocythemia and MF and triple negative ET and MF.…”

Section: Discussionmentioning

confidence: 99%

“…[6] Since 2014 Michiels et al demonstrated that cases of hypercellular ET associated with PMGM belong to the third MPN of CALR-mutated thrombocythemia and MF without features of PV at time and Calreticulin mutated clonal myeloproliferative neoplasms (MPNs), JAK2 exon 12 mutated erythrocytosis and polycythemia vera and triple negative MPN [6,[14][15][16][17][18][19][20] a c d b of diagnosis and during follow-up. [6,[14][15][16][17][18][19][20] The updated 2018 WHO -Clinical Laboratory Molecular and Pathological (CLMP) criteria define five clonal mutually exclusive MPNs and transitional states at the laboratory and bone marrow level [Figure 11]: JAK2 V617F mutated ET, prodromal PV, classical and advanced PV; JAK2 exon 12 mutated idiopathic erythrocytosis and PV; MPL 515 mutated thrombocythemia and MF; and CALR mutated thrombocythemia and MF and triple negative ET and MF. [6,[14][15][16][17][18][19][20] The JAK2 V617F positive trilinear MPDs, MPL 515 normocellular thrombocythemia, and CALR mutated thrombocythemia and MF mutually exclude each other [Table 1].…”

Section: Discussionmentioning

confidence: 99%

“…[6,[14][15][16][17][18][19][20] The updated 2018 WHO -Clinical Laboratory Molecular and Pathological (CLMP) criteria define five clonal mutually exclusive MPNs and transitional states at the laboratory and bone marrow level [Figure 11]: JAK2 V617F mutated ET, prodromal PV, classical and advanced PV; JAK2 exon 12 mutated idiopathic erythrocytosis and PV; MPL 515 mutated thrombocythemia and MF; and CALR mutated thrombocythemia and MF and triple negative ET and MF. [6,[14][15][16][17][18][19][20] The JAK2 V617F positive trilinear MPDs, MPL 515 normocellular thrombocythemia, and CALR mutated thrombocythemia and MF mutually exclude each other [Table 1]. The natural history and MPN disease burden of each of the clonal MPNs is best reflected by the degree of anemia and splenomegaly on top of mutation allele burden, bone marrow cellularity and an increase of RF.…”

Section: Discussionmentioning

confidence: 99%

“…The natural history and MPN disease burden of each of the clonal MPNs is best reflected by the degree of anemia and splenomegaly on top of mutation allele burden, bone marrow cellularity and an increase of RF. [6,[15][16][17][18][19][20] Prospective validation studies are warranted to confirm and amend the proposed WHO-CLMP classification.…”

Section: Discussionmentioning

confidence: 99%

“…Figure 11: 2018 update of the World Health Organization and Clinical Laboratory Molecular and Pathological Classification and translational states of three distinct JAK2 V617F , MPL, 515and Calreticulin mutated clonal myeloproliferative neoplasms (MPNs), JAK2 exon 12 mutated erythrocytosis and polycythemia vera and triple negative MPN[6,[14][15][16][17][18][19][20] …”

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Bone Marrow Histology is a Pathognomonic Clue to Each of the JAK2V617F, MPL,515 and Calreticulin Mutated Thrombocythemia in Myeloproliferative Neoplasms

De Raeve,

Fostier,

Valster

et al. 2018

Clin Res Hematol

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According to the World Health Organization and Clinical Laboratory Molecular and Pathological criteria bone marrow pathology in JAK2 V617F mutated trilinear myeloproliferative neoplasm (MPN) patients essential thrombocythemia (ET) and polycythemia vera are indistinguishably featured by clustered medium to large pleomorphic megakaryocytes and increased cellularity (60-90%) due to increased erythropoiesis and megakaryopoiesis. MPL 515 mutated ET is the second distinct clonal MPN characterized by thrombocythemia in a normocellular bone marrow showing clustered increased large to giant mature megakaryocytes with staghorn-like hyperlobulated nuclei. Calreticulin (CALR) mutated hypercellular thrombocythemia associated with prefibrotic megakaryocytic, granulocytic myeloproliferation (MGM) recently became the third distinct MPN featured by dense clusters of immature megakaryocytes with cloud-like nuclei. Bone marrow pathology in newly diagnosed MPN patients appears to be a pathognomonic clue for diagnostic differentiation between JAK2 V617F mutated trilinear MPN, MPL 515 normocellular thrombocythemia, and CALR thrombocythemia with MGM characteristics followed by secondary reticulin fibrosis. Their natural histories clearly differ featured by an increase of erythro/granulopoiesis and cellularity in JAK2 V617F , decrease of erythropoiesis and cellularity in MPL 515 and increase of dual megakaryo/granulopoiesis and cellularity in CALR mutated MPN.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Bone Marrow Histology is a Pathognomonic Clue to Each of the JAK2V617F, MPL,515 and Calreticulin Mutated Thrombocythemia in Myeloproliferative Neoplasms

De Raeve,

Fostier,

Valster

et al. 2018

Clin Res Hematol

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Bone marrow histology in CALR mutated thrombocythemia and myelofibrosis: results from two cross sectional studies in 70 newly diagnosed JAK2/MPL wild type thrombocythemia patients

Michiels¹,

Kim²,

Kim³

et al. 2019

Int J Bone Marrow Res

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The clinical phenotypes in 268 JAK2 V617F mutated MPN patients in the Seoul study were PV in 101, ET in 95 and MF in 78 and 56 CALR mutated MPN consisted of PV in none, ET in 40 and MF in 16 cases. CALR mutated MPN patients were younger than JAK2V 617F mutated MPN patients (mean ages 57.5 and 66 years), had lower values for values for leukocytes (8.6 vs 11.9x10 9 /L) and higher values for platelets (898 vs 643x10 9 /L respectively). Bone marrow histopathology in 268 JAK2 V617F mutated MPN patients in the Seoul study was featured by an increased erythropoiesis and megakaryopoiesis (EM) in 13.5%, an increased erythropoiesis, megakaryopoiesis and granulopoiesis (EMG) in 31.3%, a normocellular megakaryocytic (M) proliferation in 29,1%, a megakaryocytic and granulocytic (MG) proliferation with a relative reduction of erythropoiesis in post-ET and Post-PV myelofi brosis in 26.2%. The bone marrow histology in 56 cases of CALR mutated MPN show a predominantly increased megakaryopoiesis (M) in two thirds and an increased megakaryopoiesis and granulopoiesis (MG) with a decreased erythropoiesis in one third.Thirteen consecutive CALR MPN patients in the Belgian & Dutch cross sectional study presented with thrombocythemia associated with a typical PMGM bone marrow histology in 11 and myelofi brosis in 2 cases. All 11 thrombocythemia and 2 myelofi brosis CALR mutated MPN patients did not have constitutional symptoms and did not suffer from microvascular erythromelalgic disturbances, major thrombosis at platelet counts between 400 and 1000x10 9 /L. There was an occurrence of hemorrhages at platelet counts above 1000x10 9 /L in two CALR thrombocythemia cases.Bone marrow histology of CALR mutated thrombocythemia in the Seoul and Belgian/Dutch study showed loose clusters of large megakaryocytes (M) with bulky, cloud-like nuclei with a normal or a minor reduction of erythropoiesis and no increase in reticulin fi bers grade 0 or 1 (RF 0 or 1). CALR thrombocythemia patients show various degrees of increased bone marrow cellularity due to dual megakaryocytic and granulocytic (MG) proliferation featured by large megakaryocytes with roundish bulky nuclear forms and cloud-like clumsy nuclei, which are almost never seen in JAK2 V617F ET and PV. Assessment of allele burden is an independent and most important factor for all molecular variants MPN disease burden. Overt myelofi brosis with advanced post PV and or ET myelofi brosis at the bone marrow level occurred in one third (30%) of 208 evaluable JAK2 MPN patients and in 8 (14%) of 56 CALR MPN patients in the Seoul study. Research Article Bone marrow histology in CALR mutated thrombocythemia and myelofi brosis: results from two cross sectional studies in 70 newly diagnosed JAK2/MPL wild type thrombocythemia patients How to cite this article: Michiels JJ, Kim Y, Kim M, Valster F, Potters V, et al. Bone marrow histology in CALR mutated thrombocythemia and myelofi brosis: results from two cross sectional studies in 70 newly diagnosed JAK2/MPL wild type thrombocythemia patients . Int J Bone M...

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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

Michiels¹,

Lam²,

Kate³

et al. 2020

Int J Bone Marrow Res

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Increased Erythrocyte Count on Top of Bone Marrow Histology but not Serum EPO Level or JAK2 Mutation Load Discriminates between JAK2V617F Mutated Essential Thrombocythemia and Polycythemia Vera

Cited by 3 publications

References 22 publications

Bone Marrow Histology is a Pathognomonic Clue to Each of the JAK2V617F, MPL,515 and Calreticulin Mutated Thrombocythemia in Myeloproliferative Neoplasms

Bone Marrow Histology is a Pathognomonic Clue to Each of the JAK2V617F, MPL,515 and Calreticulin Mutated Thrombocythemia in Myeloproliferative Neoplasms

Bone marrow histology in CALR mutated thrombocythemia and myelofibrosis: results from two cross sectional studies in 70 newly diagnosed JAK2/MPL wild type thrombocythemia patients

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

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