Mellibovsky L, Mariñoso M L, Cervantes F, Besses C, Nacher M, Nogués X, Florensa L, Munné A, Diez-Perez A, Serrano S
Department of Internal Medicine and Metabolic Unit, Hospital Universitari del Mar, Autonomous University of Barcelona, Barcelona, Spain.
Bone. 2004 Feb;34(2):330-5. doi: 10.1016/j.bone.2003.10.010.
Idiopathic myelofibrosis (IMF) induces dramatic changes in bone. Bone remodeling and densitometric alterations in a series of nine patients with IMF and their relationship with the histologic stage of the disease were assessed. Patients were included at diagnosis and a bone marrow biopsy, dual-energy X-ray absorptiometry, and transiliac bone biopsy for histomorphometric analysis were performed. Five cases were classified as IMF histologic stage 1, one as stage 2, and three as stage 3. Compared with 40 age- and sex-matched controls, the following histomorphometric parameters were significantly higher in our patients: bone volume (BV/TV), osteoblast surface (Ob.S/BS), eroded surface (ES/BS), osteoclast surface (Oc.S/BS), osteoclast number (N.Oc/TA), mineralizing surface (MS/BS), reversal period (Rv.P), and remodeling period (Rm.P). Mineral apposition rate (MAR) and erosion depth (E.Depth) were significantly decreased (P < 0.05 for all comparisons). Bone mineral density (BMD) measurements showed high values for patient age and sex both at femur neck (Z score range +0.19 to +7) and total femur (Z score range -0.09 to +6.48). When densitometric values were analyzed according to IMF histologic stage, patients in stages 1 and 2 had significantly lower BMD values than to those in stage 3 (P = 0.024). In conclusion, patients with IMF present a characteristic bone histomorphometric pattern with increased bone volume and bone cells but low apposition and decreased erosion depth, suggesting a positive balance in bone remodeling units. This balance would produce the increase in bone mass observed in this disease. Given the increase in BMD observed with more advanced stages of IMF, this noninvasive method could be useful tool for assessing IMF progression.
原发性骨髓纤维化（IMF）可引起骨骼的显著变化。我们评估了9例IMF患者的骨重塑和骨密度改变及其与疾病组织学分期的关系。患者在诊断时纳入研究，并进行了骨髓活检、双能X线吸收法以及用于组织形态计量学分析的髂骨活检。5例被分类为IMF组织学1期，1例为2期，3例为3期。与40名年龄和性别匹配的对照组相比，我们的患者以下组织形态计量学参数显著更高：骨体积（BV/TV）、成骨细胞表面（Ob.S/BS）、侵蚀表面（ES/BS）、破骨细胞表面（Oc.S/BS）、破骨细胞数量（N.Oc/TA）、矿化表面（MS/BS）、静止期（Rv.P）和重塑期（Rm.P）。矿化沉积率（MAR）和侵蚀深度（E.Depth）显著降低（所有比较P < 0.05）。骨密度（BMD）测量显示，患者在股骨颈（Z值范围为+0.19至+7）和全股骨（Z值范围为-0.09至+6.48）的BMD值均高于相应年龄和性别的正常范围。当根据IMF组织学分期分析骨密度值时，1期和2期患者的BMD值显著低于3期患者（P = 0.024）。总之，IMF患者呈现出特征性的骨组织形态计量学模式，骨体积和骨细胞增加，但矿化沉积减少且侵蚀深度降低，提示骨重塑单位呈正平衡。这种平衡会导致该疾病中观察到的骨量增加。鉴于在IMF更晚期观察到BMD增加，这种非侵入性方法可能是评估IMF进展的有用工具。
