β地中海贫血中的髓外造血——不寻常表现：病例报告

Extramedullary haematopoiesis in beta thalassaemia--unusual presentations: case reports.

作者信息

Galati Maria Concetta, Raiola Giuseppe, De Sanctis Vincenzo, Arcuri Vincenzo, Arcuri Pier Paolo, Brachi Sara, Borsari Gloria

机构信息

Paediatric Haematoncology Unit, Pugliese, Ciaccio Hospital Catanzaro, Catanzaro, Italy.

出版信息

Pediatr Endocrinol Rev. 2008 Oct;6 Suppl 1:140-3.

PMID:19337168

Abstract

Extramedullary haematopoiesis (EH) refers to the production of blood cells outside the bone marrow as a compensatory mechanism for bone marrow dysfunction. It occurs in conditions with hyperactive, depleted or infiltrated marrow. The most frequent cause of EH is thalassaemia intermedia, due to increased demand on the hematopoietic system from anemia not reduced by transfusion therapy. The usual localizations are adjacent to bone. We report three unusual cases and discuss the current treatment.

摘要

髓外造血（EH）是指在骨髓外产生血细胞，作为骨髓功能障碍的一种代偿机制。它发生在骨髓功能亢进、耗竭或浸润的情况下。EH最常见的原因是中间型地中海贫血，这是由于输血治疗无法缓解的贫血对造血系统的需求增加所致。其常见的定位是在骨骼附近。我们报告三例不寻常的病例并讨论当前的治疗方法。