The significance of the aminoterminal propeptide of type III procollagen in paroxysmal nocturnal haemoglobinuria and myelofibrosis

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Abstract
The level of the aminoterminal propeptide Col 1–3 of type III procollagen (PC-III) was determined in patients with paroxysmal nocturnal haemoglobinuria (PNH) and primary myelofibrosis (PMF), to study whether PC-III can be used as a parameter for the rate and/or degree of bone marrow replacement with collagen. Normal PC-III levels were found in PNH (6.6±1.1 μg/l; N: 8.6±1.8 μg/l), while significantly increased levels were found in PMF (24.8±2.2 μg/l). During a follow-up of 1 year, a slight increase of 2 μg/l occurred in three patients with a stable fibrosis, while one patient with more active disease demonstrated an increase of 25 μg/l. Treatment with acetylsalicylic acid led to a decline of PC-III as well as β-thromboglobulin level, although normalization did not occur. It was demonstrated by means of gel filtration that the antigens related to the PC-III peptide were heterogenous, and that in PMF at least two main peaks were present, with molecular masses equal to and smaller than PC-III peptide. These data demonstrate that the radioimmunoassay cannot be used for the quantitative determination of PC-III; nevertheless it gives some insight in the process of bone marrow fibrosis.