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特发性骨髓纤维化及相关疾病中的血清层粘连蛋白P1

Serum laminin P1 in idiopathic myelofibrosis and related diseases.

作者信息

Hasselbalch H, Junker P

机构信息

Department of Medicine and Haematology L, Herlev University Hospital, Denmark.

出版信息

Leuk Res. 1994 Aug;18(8):623-8. doi: 10.1016/0145-2126(94)90044-2.

Abstract

The serum concentration of laminin P1 antigen was determined in 32 patients with various myeloproliferative disorders, including 19 patients with idiopathic myelofibrosis. The serum concentration of the aminoterminal propeptide of type III procollagen (PIIINP) was measured concomitantly in 27 patients. Serial laminin measurements were carried out in 25 patients. The median serum laminin concentration in patients with acute disease, i.e. acute myelofibrosis and patients in a transforming disease phase was significantly higher (1.58 U/ml; range 1.15-2.07) as compared with patients with chronic disease (1.02 U/ml; range 0.75-1.76; p = 0.012) and healthy control subjects (1.13 U/ml; range 0.75-1.67; p = 0.00015). In individual patients serum laminin covariated closely with serum PIIINP, the leucocyte count and LDH. The median serum laminin concentration in patients with a huge spleen was significantly lower than in the patient group with a normal spleen size/previous splenectomy. Pronounced splenomegaly was particularly prevalent in patients with chronic disease implicating splenic enlargement as a significant extrahepatic site of laminin uptake/degradation in myeloproliferative disorders.

摘要

对32例各种骨髓增殖性疾病患者测定了层粘连蛋白P1抗原的血清浓度,其中包括19例特发性骨髓纤维化患者。同时对27例患者测定了III型前胶原氨基端前肽(PIIINP)的血清浓度。对25例患者进行了层粘连蛋白的系列测定。与慢性病患者(1.02 U/ml;范围0.75 - 1.76;p = 0.012)和健康对照者(1.13 U/ml;范围0.75 - 1.67;p = 0.00015)相比,急性病患者即急性骨髓纤维化患者和处于疾病转化期的患者血清层粘连蛋白浓度中位数显著更高(1.58 U/ml;范围1.15 - 2.07)。在个体患者中,血清层粘连蛋白与血清PIIINP、白细胞计数和乳酸脱氢酶密切相关。脾肿大患者的血清层粘连蛋白浓度中位数显著低于脾大小正常/既往行脾切除术的患者组。明显的脾肿大在慢性病患者中尤为普遍,这意味着脾肿大是骨髓增殖性疾病中层粘连蛋白摄取/降解的一个重要肝外部位。

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