地中海贫血患者的髓外造血：其患病率、临床特征和生存率的横断面描述

IF 1.8 Q3 HEMATOLOGY

Hematology, Transfusion and Cell Therapy Pub Date : 2024-11-01 DOI:10.1016/j.htct.2023.07.005

Eduardo Cerello Chapchap , Murilo Marques Almeida Silva , Ronaldo Hueb Baroni , Aderson da Silva Araujo , Reijane Alves de Assis , Sandra Regina Loggetto , Antonio Fabron Junior , Monica Pinheiro de Almeida Verissimo , Giorgio Roberto Baldanzi , Kleber Yotsumoto Fertrin , Fernando Tricta , Antonio Giulio Piga , Nelson Hamerschlak

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引用次数: 0

摘要

尽管对地中海贫血患者髓外造血（EMH）的了解有所进展，但其真实情况仍是一个悬而未决的问题。目的评估重度地中海贫血（TM）和中度地中海贫血（TI）患者EMH的患病率，描述磁共振成像（MRI）结果，并探讨临床危险因素。方法回顾性分析2004 ~ 2011年连续184例进行T2* MRI检查的地中海贫血患者的影像和临床资料。研究了EMH与患者生存率的关系。结果TM患者（19 ~ 49岁）中有16/168例（9.5%）检测到semh， TI患者（36 ~ 41岁）中有3/16例（18.8%）检测到semh。大多数（88%）有椎旁胸椎和/或腹部肿块。年龄与EMH风险显著相关(风险比，[HR] 1.10/年；置信区间[CI]: 1.03-1.18；假定值& lt;T2*MRI显示胰腺铁含量较低(HR: 0.94/ms；置信区间:0.89—-0.99;p值= 0.049)为保护因素。emh阳性患者（n = 19）的估计生存率优于emh阴性患者（n = 75）（p值= 0.013）。结论EMH患病率为10.3%(19/184)，以3 ~ 10 cm肿块为主。年龄是EMH发展的一个危险因素，而在这个队列中，较低的胰腺铁可能是一个保护因素。

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

Introduction

Despite knowledge advances on extramedullary haematopoiesis (EMH) in thalassemic patients, the real picture remains an open issue.

Objectives

To assess EMH prevalence in patients with thalassemia major (TM) and intermedia (TI), to describe magnetic resonance imaging (MRI) findings and to explore clinical risk factors.

Methods

In this cross-sectional study, images and clinical records of 184 consecutive patients with thalassemia who underwent T2* MRI between 2004 and 2011 were reviewed. Association of EMH with survival was investigated for patients with available follow-up charts.

Results

EMH was detected in 16/168 (9.5%) patients with TM (aged 19-49 years) and in 3/16 (18.8%) with TI (aged 36-41 years). Most (88%) had paravertebral thoracic and/or abdominal masses. Age was significantly associated with EMH risk (hazard ratio, [HR] 1.10/year; confidence interval [CI]: 1.03-1.18; p-value < 0.001), while lower pancreatic iron content by T2*MRI (HR: 0.94/ms; CI: 0.89-0.99; p-value = 0.049) was a protective factor. Estimated survival rate was superior for EMH-positive (n = 19) when compared to EMH-negative patients (n = 75) (p-value = 0.013).

Conclusions

The prevalence of EMH was 10.3% (19/184), presented mainly as tumoral masses of 3 to 10 cm. Age was a risk factor for EMH development, while lower pancreatic iron might be a protective factor in this cohort.

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