Yokus Osman, Yigit Betul, Goze Hasan, Serin Istemi
Department of Hematology, University of Health Sciences, Istanbul Training and Research Hospital Istanbul, Turkey.
Department of Internal Medicine, University of Health Sciences, Istanbul Training and Research Hospital Istanbul, Turkey.
Am J Blood Res. 2020 Dec 15;10(6):345-350. eCollection 2020.
Myelofibrosis (MF) is a disease in which the grade of bone marrow fibrosis increases in proportion to the degree of extramedullary hematopoiesis and splenomegaly. Associated with increased cytokines and inflammation, anemia deepens and an increase in serum ferritin levels is also expected. There are no studies addressing the relationship between ferritin and splenomegaly or fibrosis. In this study, the relationship between serum ferritin level and splenomegaly and bone marrow fibrosis was examined.
The study was performed retrospectively in 46 MF cases diagnosed between 2012 and 2020. MF was divided into 3 separate subgroups: Primary myelofibrosis, secondary myelofibrosis and myeloproliferative neoplasms (MDS/MPN) with myelodsplastic syndrome.
Thirty (28.3%) of cases were PMF, 26 (56.5%) were SMF and 7 (15.2%) were MDS/MPN. There was no relation found between serum ferritin and splenomegaly in none of the cases or subgroup analysis (for PMF p: 0.564, for SMF p: 0.192, for MDS/MPN p: 0.364). There was a statistically significant relationship between serum ferritin and marrow fibrosis within the group of ages 60 years and older (p: 0.016).
Disruption of hematopoiesis and progressive splenomegaly causes an increase in iron stores associated with an increased need for transfusion. This causes iron-related organ toxicity and bone marrow hematopoiesis disruption, leading to an increase in morbidity. We see that a significant relationship between ferritin and fibrosis has been revealed in the group aged 60 years and older. It is an unprecedented study in the literature in terms of both examining the relationship ferritin and fibrosis or splenomegaly and its results.
骨髓纤维化（MF）是一种骨髓纤维化程度与髓外造血及脾肿大程度成正比增加的疾病。与细胞因子增加和炎症相关，贫血会加重，血清铁蛋白水平也预计会升高。目前尚无关于铁蛋白与脾肿大或纤维化之间关系的研究。在本研究中，对血清铁蛋白水平与脾肿大及骨髓纤维化之间的关系进行了研究。
本研究对2012年至2020年期间诊断的46例MF病例进行回顾性分析。MF分为3个独立亚组：原发性骨髓纤维化、继发性骨髓纤维化和伴有骨髓增生异常综合征的骨髓增殖性肿瘤（MDS/MPN）。
30例（28.3%）为原发性骨髓纤维化，26例（56.5%）为继发性骨髓纤维化，7例（15.2%）为MDS/MPN。在所有病例或亚组分析中，均未发现血清铁蛋白与脾肿大之间存在关联（原发性骨髓纤维化p：0.564，继发性骨髓纤维化p：0.192，MDS/MPN p：0.364）。在60岁及以上年龄组中，血清铁蛋白与骨髓纤维化之间存在统计学显著关联（p：0.016）。
造血功能破坏和进行性脾肿大导致铁储存增加，与输血需求增加相关。这会导致与铁相关的器官毒性和骨髓造血功能破坏，从而导致发病率增加。我们发现，在60岁及以上年龄组中，铁蛋白与纤维化之间存在显著关联。就研究铁蛋白与纤维化或脾肿大之间的关系及其结果而言，这是文献中一项前所未有的研究。
