» Articles » PMID: 30834265

Erythropoiesis and Iron Homeostasis in Non-Transfusion-Dependent Thalassemia Patients with Extramedullary Hematopoiesis

Overview
Journal Biomed Res Int
Publisher Wiley
Date 2019 Mar 6
PMID 30834265
Citations 8
Authors
Affiliations
Soon will be listed here.
Abstract

Background: Extramedullary hematopoiesis (EMH) is common in non-transfusion-dependent thalassemia (NTDT) patients. Clinical presentations of EMH vary as MRI screening is not feasible. Hence, serum biomarkers are used to predict the risk of EMH.

Materials And Methods: 52 NTDT patients, including 26 EMH (+) and 26 EMH (-), together with 26 healthy controls, were enrolled in this case-control study from 2013 to 2016. EMH was confirmed by computed tomography or MRI. Demographic, transfusion, genetic, laboratory, and liver iron concentration (LIC) data, as well as clinical complications, were analyzed.

Results: EMH (+) patients had significantly higher serum ferritin (SF), growth differentiation factor 15 (GDF15), and erythropoietin (EPO) levels compared with EMH (-) patients and controls. The levels of erythroferrone (ERFE), hepcidin, and sTfR did not differ significantly between EMH (+) and EMH (-) patients (p>0.05). In NTDT patients, serum ERFE was not related to SF, LIC, hepcidin, sTfR, EPO, GDF15, and Hb levels. GDF15, EPO concentrations, and GDF15 to sTfR and GDF15 to EPO ratios are able to determine the presence of EMH with considerable sensitivity and specificity.

Conclusions: GDF15, EPO, and GDF15 to EPO and GDF15 to sTfR ratios are potential biomarkers for the early prediction of NTDT in patients who are at risk for EMH.

Citing Articles

A case of intrathoracic extramedullary hematopoiesis-associated pleural effusion in a beta-thalassemia intermedia patient and a short literature review.

Barakos G, Papazafiropoulou A, Mihos K, Eminidou V, Andronikou S, Ainalidou L SAGE Open Med Case Rep. 2024; 12:2050313X241305169.

PMID: 39634073 PMC: 11615976. DOI: 10.1177/2050313X241305169.


A randomized placebo-controlled clinical trial of oral green tea epigallocatechin 3-gallate on erythropoiesis and oxidative stress in transfusion-dependent β-thalassemia patients.

Settakorn K, Hantrakool S, Petiwathayakorn T, Hutachok N, Tantiworawit A, Charoenkwan P Front Mol Biosci. 2024; 10:1248742.

PMID: 38328786 PMC: 10848917. DOI: 10.3389/fmolb.2023.1248742.


Increased iron uptake by splenic hematopoietic stem cells promotes TET2-dependent erythroid regeneration.

Tseng Y, Kageyama Y, Murdaugh R, Kitano A, Kim J, Hoegenauer K Nat Commun. 2024; 15(1):538.

PMID: 38225226 PMC: 10789814. DOI: 10.1038/s41467-024-44718-0.


Extramedullary haematopoiesis in patients with thalassemia: a cross-sectional description of its prevalence, clinical features and survival.

Chapchap E, Silva M, Baroni R, Araujo A, de Assis R, Loggetto S Hematol Transfus Cell Ther. 2023; 46 Suppl 5:S143-S151.

PMID: 37690980 PMC: 11670632. DOI: 10.1016/j.htct.2023.07.005.


Phosphatidylserine-exposed red blood cells and ineffective erythropoiesis biomarkers in patients with thalassemia.

Chansai S, Yamsri S, Fucharoen S, Fucharoen G, Teawtrakul N Am J Transl Res. 2022; 14(7):4743-4756.

PMID: 35958503 PMC: 9360869.


References
1.
Taher A, Ismaeel H, Cappellini M . Thalassemia intermedia: revisited. Blood Cells Mol Dis. 2006; 37(1):12-20. DOI: 10.1016/j.bcmd.2006.04.005. View

2.
Tanno T, Bhanu N, Oneal P, Goh S, Staker P, Lee Y . High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin. Nat Med. 2007; 13(9):1096-101. DOI: 10.1038/nm1629. View

3.
Tanno T, Porayette P, Sripichai O, Noh S, Byrnes C, Bhupatiraju A . Identification of TWSG1 as a second novel erythroid regulator of hepcidin expression in murine and human cells. Blood. 2009; 114(1):181-6. PMC: 2710947. DOI: 10.1182/blood-2008-12-195503. View

4.
Theurl I, Finkenstedt A, Schroll A, Nairz M, Sonnweber T, Bellmann-Weiler R . Growth differentiation factor 15 in anaemia of chronic disease, iron deficiency anaemia and mixed type anaemia. Br J Haematol. 2009; 148(3):449-55. DOI: 10.1111/j.1365-2141.2009.07961.x. View

5.
Taher A, Musallam K, Karimi M, El-Beshlawy A, Belhoul K, Daar S . Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood. 2009; 115(10):1886-92. DOI: 10.1182/blood-2009-09-243154. View