Shapiro F, Key L L, Anast C
Department of Orthopaedic Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115.
Calcif Tissue Int. 1988 Aug;43(2):67-76. doi: 10.1007/BF02555149.
A light and transmission electron microscopic (TEM) study of iliac crest metaphyseal bone from nine patients with infantile osteopetrosis demonstrates a variable spectrum of osteoclast abnormalities. All bone was obtained at biopsy before treatment. The average age at biopsy was 6 months with a range from 1 to 12 months. Osteoclast number was always increased and the cells were always appropriately positioned in relation to bone and cartilage. Osteoclast number, size, and nucleation varied from midly to markedly increased. In those with only a mild-to-moderate osteoclast increase, the marrow had an otherwise near-normal appearance with a good complement of hematopoietic cells. In those with markedly increased osteoclasts (hyperosteoclastic state) there were only scanty collections of hematopoietic cells. Light microscopic histomorphometry documented the percentage of bone and cartilage surfaces covered by osteoclasts. Controls from areas of greatest osteoclast presence documented a 5% coverage. One osteopetrotic patient registered a 4.8% value with all others elevated from 7.6 to 27.9%. Quantitative electron microscopy showed the ruffled border-clear zone complex to be absent or markedly diminished in seven of the nine patients. In two, however, osteoclast profiles had abundant ruffled border-clear zone complexes. Patients with the hyperosteoclastic bone marrow were more severely affected clinically. Light and TEM studies of marrow biopsies in initial assessment of osteopetrosis establish a baseline profile, may provide prognostic information, and allow for more meaningful treatment follow-up.
对9例婴儿骨硬化症患者的髂嵴干骺端骨进行光镜和透射电子显微镜（TEM）研究，结果显示破骨细胞异常情况多样。所有骨组织均在治疗前通过活检获取。活检时的平均年龄为6个月，范围为1至12个月。破骨细胞数量总是增加，且细胞相对于骨和软骨的位置总是恰当的。破骨细胞数量、大小和成核情况从轻度增加到显著增加不等。在那些破骨细胞仅轻度至中度增加的患者中，骨髓在其他方面外观接近正常，造血细胞数量充足。在破骨细胞显著增加（破骨细胞增多状态）的患者中，造血细胞仅少量聚集。光镜组织形态计量学记录了破骨细胞覆盖的骨和软骨表面的百分比。破骨细胞存在最多区域的对照显示覆盖率为5%。1例骨硬化症患者的该值为4.8%，其他所有患者的值从7.6%升高至27.9%。定量电子显微镜显示，9例患者中有7例的皱襞缘-清晰区复合体缺失或显著减少。然而，有2例患者的破骨细胞轮廓有丰富的皱襞缘-清晰区复合体。破骨细胞增多性骨髓的患者临床症状更严重。对骨硬化症进行初步评估时，对骨髓活检进行光镜和TEM研究可建立基线特征，可能提供预后信息，并有助于进行更有意义的治疗随访。
