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Title in Portuguese: Severely impaired terminal erythroid differentiation as an independent prognostic marker in myelodysplastic syndromes
Author: Ali, Abdullah Mahmood
Huang, Yumin
Pinheiro, Ronald Feitosa
Xue, Fumin
Hu, Jingping
Iverson, Nicholas
Hoehn, Daniela
Coutinho, Diego
Kayani, Jehanzeb
Chernak, Brian
Lane, Joseph
Hillyer, Christopher
Galili, Naomi
Jurcic, Joseph
Mohandas, Narla
An, Xiuli
Raza, Azra
Keywords: Anemia
Medula Óssea
Bone Marrow
Issue Date: Jun-2018
Publisher: Blood Advances
Citation: ALI, A. M. et al. Severely impaired terminal erythroid differentiation as an independent prognostic marker in myelodysplastic syndromes. Blood Advances, v. 2, n. 12, p. 1393-1402, jun. 2018.
Abstract: Anemia is the defining feature in most patients with myelodysplastic syndromes (MDS), yet defects in erythropoiesis have not been well characterized. We examined freshly obtained bone marrow (BM) samples for stage-specific abnormalities during terminal erythroid differentiation (TED) from 221 samples (MDS, n = 205 from 113 unique patients; normal, n = 16) by measuring the surface expression of glycophorin A, band 3, and integrin α-4. Clinical and biologic associations were sought with presence or absence of TED and the specific stage of erythroid arrest. In 27% of MDS samples (56/205), there was no quantifiable TED documented by surface expression of integrin α-4 and band 3 by terminally differentiating erythroblasts. Absence of quantifiable TED was associated with a significantly worse overall survival (56 vs 103 months, P = .0001) and SRSF2 mutations (7/23, P < .05). In a multivariable Cox proportional hazards regression analysis, absence of TED remained independently significant across International Prognostic Scoring System-Revised (IPSS-R) categories, myeloid/erythroid ratio, and mutations in several genes. In 149/205 MDS samples, the proportion of cells undergoing TED did not follow the expected 1:2:4:8:16 doubling pattern in successive stages. Absence of TED emerged as a powerful independent prognostic marker of poor overall survival across all IPSS-R categories in MDS, and SRSF2 mutations were more frequently associated with absence of TED.
metadata.dc.type: Artigo
ISSN: 2473-9529 (Online)
Appears in Collections:DPML - Artigos publicados em revista científica

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