Hasselbalch H, Junker P, Lisse I, Bentsen K D, Risteli L, Risteli J
Am J Hematol. 1986 Oct;23(2):101-11. doi: 10.1002/ajh.2830230204.
Myelofibrosis is characterized by excessive deposition of interstitial and basement membrane collagens in the bone marrow. In this study, specific radioimmunoassays for the aminoterminal propeptide of type III procollagen and for the 7S collagen domain of type IV (basement membrane) collagen were used to determine how this accumulation is reflected in serum. Of the 41 patients with chronic myeloproliferative disorders studied, the highest levels of both parameters were found in idiopathic myelofibrosis and in chronic myelogenous leukaemia associated with bone marrow fibrosis. Increasing degrees of bone marrow fibrosis were accompanied by increasing serum concentrations of both markers, except for osteomyelosclerosis, where notably low values were seen. Pathologically high values of one or both parameters were also found in a few patients with polycythaemia vera or a transitional myeloproliferative disorder. The antigens related to type III procollagen and type IV collagen correlated significantly with each other and with the leucocyte count. These parameters should provide noninvasive means for following the accumulation of interstitial and basement membrane collagens in the bone marrow.
骨髓纤维化的特征是骨髓中细胞间质和基底膜胶原蛋白过度沉积。在本研究中，采用针对III型前胶原氨基端前肽和IV型（基底膜）胶原7S胶原结构域的特异性放射免疫分析法，以确定血清中如何反映这种蓄积情况。在所研究的41例慢性骨髓增殖性疾病患者中，这两个参数的最高水平见于特发性骨髓纤维化以及伴有骨髓纤维化的慢性粒细胞白血病。除骨硬化症（其值显著较低）外，骨髓纤维化程度的增加伴随着两种标志物血清浓度的升高。在少数真性红细胞增多症或过渡性骨髓增殖性疾病患者中也发现一个或两个参数的病理性高值。与III型前胶原和IV型胶原相关的抗原彼此之间以及与白细胞计数均显著相关。这些参数应为追踪骨髓中细胞间质和基底膜胶原蛋白的蓄积情况提供非侵入性手段。
