慢性特发性骨髓纤维化患者血清五项指标和骨髓显像类型与疾病分期的关系

Study on thd Relationship Between Serum Fibrosis Markers Levels,Bone Marrow Scintigraphy Patterns and Clinical Staging in Patients with Chronic Idiopathic Myelofibrosis(CIMF)

目的:探讨慢性特发性骨髓纤维化(CIMF)患者血清中转化生长因子β1(TGF-β1)、Ⅲ型前胶原(PCⅢ)、层黏蛋白(LN)、Ⅳ型胶原(C-Ⅳ)、血小板源生长因子(PDGF)和放射性核素骨髓显像类型与疾病分期的关系。方法:采用放射免疫分析及ELISA测定46例CIMF患者血清TGF-β1、PCⅢ、C-Ⅳ、LN、PDGF;46例CIMF用99mTc-硫胶体骨髓显像。同时取34例正常健康人作对照。结果:CIMF患者细胞期,胶原形成期,硬化期血清五项指标相比较均有统计学意义(P<0.05)。细胞期(14例)骨髓显像类型多表现为增强型(共13例,占92.9%),胶原形成期(19例)骨髓显像类型多表现为抑制扩张型(共17例,占89.5%),硬化期(13例)骨髓显像类型表现为抑制型(共13例,占100%)。结论:血清五项指标TGF-β1、PCⅢ、C-Ⅳ、LN、PDGF在CIMF患者明显升高,与疾病发生、发展有一定关系,骨髓显像的不同类型与疾病分期有一定关系,血清五项指标和骨髓显像有助于CIMF的诊断、了解临床状态和预后。

Objective To study the changes of serum fibrosis markers levels and the pattern of bone marrow scintigraphy and their relationship with clinical stages in patients with chronic idiopathic myelofibrosis（CIMF）.Methods Transforming growth factor-β1（TGF-β1） and platelet derived growth factor（PDGF）（with ELISA）,serum typeⅢ procollagen（PCⅢ）,type Ⅳ collagen（C-Ⅳ）,and laminin（LN）（with RIA） were detected in 46 with patients with chronic idiopathic myelofibrosis and 34 controls.Bone marrow scintigraphy 99mTc-sulfur colloid was performed in the 46 CIMF patients.Results Five fibrosis markers in patients with CIMF were significantly higher than those in controls.There were significantly differences among the levels in different stages,especially with sclerotic stage in CIMF patients.The imaging patterns were classified into three types according to the radio-distribution and activity of bone marrow： 13 cases of increased imaging in 14 patients of cellular stage,17 cases with creased expansion imaging in 19 patients of collagen fiber stage and 13 cases（100%） with totally depressed imaging in patients of sclerotic stage.Conclusion As the disease progressed,the serum fibrosis markers levels gradually incroased and the scintigraphy patterns corresponded well to the clinical staging.