Analysis of determinant factors of liver fibrosis progression in ex-thalassemic patients

• Published in: International journal of hematology Vol. 113; no. 1; pp. 145 - 157
• Main Authors: Rostami, Tahereh, Monzavi, Seyed Mostafa, Poustchi, Hossein, Khoshdel, Ali Reza, Behfar, Maryam, Hamidieh, Amir Ali
• Format: Journal Article
• Language: English
• Published: Singapore Springer Singapore 2021; Springer Nature B.V
• Subjects: Adolescent; Allografts; beta-Thalassemia - complications; beta-Thalassemia - therapy; Bone marrow; Bone marrow transplantation; Bone Marrow Transplantation - adverse effects; Child; Child, Preschool; Complications; Depletion; Disease Progression; Elasticity Imaging Techniques; Female; Ferritin; Fibrosis; Graft vs Host Disease - etiology; Graft-versus-host reaction; Hematology; Hematopoiesis; Hematopoietic Stem Cell Transplantation - adverse effects; Hematopoietic stem cells; Humans; Iron; Liver; Liver Cirrhosis - diagnosis; Liver Cirrhosis - etiology; Liver diseases; Liver transplantation; Male; Medicine; Medicine & Public Health; Oncology; Original Article; Prospective Studies; Stem cell transplantation; Stem cells; Thalassemia; Transfusion; Transplantation; Transplantation, Homologous; Hepatic fibrosis; Beta-thalassemia; Transient elastography; Hematopoietic stem cell transplantation
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-020-02998-4

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) potentially renders thalassemia patients disease-free with presumably cessation of associated complications. This study analyzes the liver fibrosis status and the determinants of its progression in ex-thalassemic patients. The liver fibrosis status of 108 pediatric transfusion-dependent β-thalassemia major patients was evaluated before and one year after allo-HSCT using transient elastography (TE). All patients achieved normal hematopoiesis. In univariate analyses, not in all, but in patients developing significant post-HSCT iron overload or hepatic graft-versus-host disease (GvHD), as well as recipients of bone marrow stem cells (BMSC), significant TE increment occurred. In multivariable analyses, through a model with large effect size (Adj.
R
2
 = 26%,
F
(3,104)
 = 13.53,
P
 < 0.001), post-HSCT serum ferritin and hepatic GvHD were ascertained as independent determinants of significant TE increase, and the effect of stem cell graft source approached the level of significance. Excluding the patients with intermediate/high Lucarelli risk classes, the TE increase was significantly greater only in BMSC recipients (
P
 = 0.033). Although the risk impact of allograft source on liver fibrosis progression requires further evaluation; hepatic status of ex-thalassemic patients can be preserved after HSCT, if hepatic GvHD is controlled and adequate post-transplantation iron depletion is ensured.