Type III aminoterminal propeptide of procollagen in some haematological malignancies

Abstract

Marrow fibrosis is involved in some haematological malignancies. Either because of sampling errors, variations of focal distribution of fibrosis or the discomfort for patients of bone biopsies, conventional histology appears to be unsuitable for the follow‐up of myelofibrosis. During collagen synthesis by marrow fibroblasts, the aminoterminal propeptide is removed from procollagen III and released in the serum. Thus, a sensitive radioimmunoassay of type III aminoterminal propeptide of procollagen (PC III) has been tested in myeloproliferative and lymphoproliferative disorders with a marked bone marrow fibrosis. In polycythaemia vera, PC III level was significantly increased as compared to controls and was related to marrow fibrosis of grade I. The more increased PC III values were observed in spent polycythaemia cases initially treated by phlebotomy alone. Follow‐up showed a transformation into myeloid metaplasia. In contrast, PC III remained stable in patients treated with radiophosphorus 32P or hydroxyurea who did not transform. In myeloid metaplasia, results of PC III were significantly higher than in controls or polycythaemia vera cases. Myelofibrosis of recent onset (< 2 years) gave higher values than chronic myelofibrosis. Increased PC III values were also emphasized in chronic myelocytic leukaemia, and in a few cases of refractory anaemia with excess of blasts, hairy cell leukaemia and chronic lymphocytic leukaemia.

Acute Lymphoblastic Leukemia in the Course of Polycythemia Vera: A Case Report and Review of Literature

“…In general, the extent of splenomegaly is in accordance with the extensive degree of MF, and microscopic observation did not reveal any positive relationship of the splenomegaly with MF. MF may be focal or unevenly distributed [27]. We further detected the level of serum markers that are indicative of MF: elevated expression of collagen IV, hyaluronic acid and procollagen III were revealed.…”

Revisiting Circulating Extracellular Matrix Fragments as Disease Markers in Myelofibrosis and Related Neoplasms

“…In addition, fibronectin contributes to the reticulin matrix [179]. The type III collagen fibers have been demonstrated by the application of antibodies against type III procollagen (pN collagen) [86,95], indicating that the propeptide is partly retained on the collagen fibers in the bone marrow.) Particularly increased collagen content, including both newly deposited type III procollagen (pN collagen) and mature collagen fibers, was found in patients with osteomyelosclerosis, which was also associated with the most pronounced neovascularization [95].…”

“…Besides fibroblast and endothelial proliferation, another important change in bone marrow in PMF includes the formation of continuous sheets of basement membranes beneath endothelial cells [86,95,255]. This process appears to be very similar to the "capillarization" of liver sinusoids during the development of liver cirrhosis [257].…”

“…Previous studies on the potential of circulating extracellular matrix (ECM) fragments released to the circulation consequent to the altered MPN-related connective tissue turnover as markers of current early bone marrow inflammation and late fibrogenesis, respectively, have demonstrated highly pertinent associations between circulating levels of several ECM proteins and disease activity and drug responses in MPNs, particularly regarding the procollagen III-N-terminal propeptide, type IV collagen, laminin and hyaluronan [80][81][82][83][84][85][86][87][88][89][90][91][92][93][94][95][96][97][98]. Within recent years, the research field on ECM and circulating ECM fragments has expanded immensely in several diseases, not least within cardiovascular diseases and cancer .…”

Serum Procollagen‐iii‐peptide as a Marker for Primary Myelofibrosis: Effect of Anthracyclin

“…It has recently been suggested that the serum procollagen-111-peptide level in patients with myeoproliferative disorders could be a marker of progressive myelofibrosis (1). These diseases are considered as clonal proliferations of the multipotent stem cell (2); hence, as in an ongoing study (3), an aggressive chemotherapy was used.…”