Circulating Cd34+ cell count differentiates primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms: a pragmatic study

Abstract

A high number of circulating CD34+ cells has been advocated to distinguish primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms. We re-evaluated the diagnostic interest of measuring circulating CD34+ cells in 26 healthy volunteers and 256 consecutive patients at diagnosis for whom a myeloproliferative neoplasm was suspected. The ROC curve analysis showed that a number of CD34+ <10/μl excludes the diagnosis of primary myelofibrosis with a sensitivity of 97 % and a specificity of 90 % (area under the curve: 0.93 [0.89-0.98]; p < 0.001). Patients with PMF harboring a CALR mutation had more circulating CD34+ cells than patients with either a JAK 2 or MPL mutation (p = 0.02 and p < 0.01, respectively). These results suggest that this fast, simple, non-invasive, and standardized test is of particular interest to exclude the diagnosis of primary myelofibrosis.

Increased Plasma Levels of lncRNAs LINC01268, GAS5 and MALAT1 Correlate with Negative Prognostic Factors in Myelofibrosis

“…The fibrotic conversion of bone marrow occurring in MF induces a release into circulation of CD34+ cells normally residing in bone marrow [ 41 ]. Circulating neoplastic cells derived from neoplastic clones might thus discharge an abnormal number of secreted molecules into plasma.…”

Bioinformatics Analysis of Key Genes and Pathways Associated with Thrombosis in Essential Thrombocythemia

“…Also, the expression of CD11b mRNA in CD34+ cells was significantly upregulated and enriched in secretory vesicles (by CC analysis). Considering the increased number of CD34+ cells in peripheral blood of patients with ET compared with healthy controls [25], this may facilitate the interaction between peripheral CD34+ cells and neutrophils. It was assumed that CD11b were overexpressed in CD34+ cells and that the gene products were transported to neutrophils in a secretory vesicle-dependent manner, leading to the extrinsic gain of CD11b protein in neutrophils.…”

Different number of circulating CD34 + cells in essential thrombocythemia, prefibrotic/early primary myelofibrosis, and overt primary myelofibrosis

“…In our hands, a number of CD34 + < 10/μl excludes the diagnosis of primary myelofibrosis (PMF) with a sensitivity of 97% and a specificity of 90%. [6] The diagnostic criteria for MPN are constantly evolving. In particular in the 2016 WHO classification, the morphological major criteria, combined with clinico-biological minor criteria, have been better defined to establish the diagnosis of prefibrotic/early PMF (prePMF) disease [5,7].…”

“…It has been previously shown (also by our team) that this non-invasive test could easily distinguish ET and overt PMF cases. [1][2][3]6] However, data regarding circulating CD34 + cells in prePMF are still scarce. [9,11] We show that the median number of circulating CD34 + cells in patients with prePMF is intermediate between ET and overt PMF.…”