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.
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Barosi G, Viarengo G, Pecci A et al (2001) Diagnostic and clinical relevance of the number of circulating CD34 (+) cells in myelofibrosis with myeloid metaplasia. Blood 98:3249–3255
Andréasson B, Swolin B, Kutti J (2002) Patients with idiopathic myelofibrosis show increased CD34+ cell concentrations in peripheral blood compared to patients with polycythaemia vera and essential thrombocytopenia. Eur J Haematol 68:189–193
Passamonti F, Vanelli L, Malabarba L et al (2003) Clinical utility of the absolute number of circulating CD34-positive cells in patients with chronic myeloproliferative disorders. Haematologica 88:1123–1129
Arora B, Sirhan S, Hoyer JD, Mesa RA, Tefferi A (2005) Peripheral blood CD34 count in myelofibrosis with myeloid metaplasia: a prospective evaluation of prognostic value in 94 patients. Br J Haematol 128:42–48
Arber DA, Orazi A, Hasserjian R et al (2016) The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 127:2391–2405
Klampfl T, Gisslinger H, Harutyunyan AS et al (2013) Somatic mutations of calreticulin in myeloproliferative neoplasms. N Engl J Med 369:2379–2390
Nangalia J, Massie CE, Baxter EJ et al (2013) Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2. N Engl J Med 369:2391–2405
Tefferi A, Thiele J, Vannucchi AM, Barbui T (2014) An overview on CALR and CSF3R mutations and a proposal for revision of the WHO diagnostic criteria for myeloproliferative neoplasms. Leukemia 28:1407–1413
Dobo I, Boiret N, Lippert E et al (2004) A standardized endogenous megakaryocytic erythroid colony assay for the diagnosis of essential thrombocythemia. Haematologica 89:1207–1212
Rumi E, Pietra D, Pascutto C et al (2014) Clinical effect of driver mutations of JAK2, CALR, or MPL in primary myelofibrosis. Blood 124:1062–1069
Tefferi A, Guglielmelli P, Larson DR et al (2014) Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood 124:2507–2513
Massa M, Rosti V, Ramajoli I et al (2005) Circulating CD34+, CD133+, and vascular endothelial growth factor receptor 2-positive endothelial progenitor cells in myelofibrosis with myeloid metaplasia. J Clin Oncol 23:5688–5695
Pietra D, Rumi E, Ferretti VV et al (2016) Differential clinical effects of different mutation subtypes in CALR-mutant myeloproliferative neoplasms. Leukemia 30:431–438
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We thank the technicians from both centers for their help.
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The study was performed in accordance with institutional ethical guidelines and the Declaration of Helsinki.
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The authors declare that they have no conflict of interest.
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C. Orvain and D. Luque Paz contributed equally to this work.
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Orvain, C., Luque Paz, D., Dobo, I. et al. Circulating Cd34+ cell count differentiates primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms: a pragmatic study. Ann Hematol 95, 1819–1823 (2016). https://doi.org/10.1007/s00277-016-2784-x
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DOI: https://doi.org/10.1007/s00277-016-2784-x