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

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 micrograms/l; N: 8.6 +/- 1.8 micrograms/l), while significantly increased levels were found in PMF (24.8 +/- 2.2 micrograms/l). During a follow-up of 1 year, a slight increase of 2 micrograms/l occurred in three patients with a stable fibrosis, while one patient with more active disease demonstrated an increase of 25 micrograms/l. Treatment with acetylsalicylic acid led to a decline of PC-III as well as beta-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.

Serum markers for type IV collagen and type III procollagen in the myelofibrosis‐osteomyelosclerosis syndrome and other chronic myeloproliferative disorders

“…Previous studies of patients with MF and related disorders have suggested serum P-III-P to be a useful marker of the fibroproliferative activity in the bone marrow [12][13][14][15].…”

“…During synthesis of type III collagen fibers, the aminoterminal propeptide of type 111 procollagen (P-III-P) is removed enzymatically from the procollagen molecules and subsequently released into the circulation [26,27]. Previous studies of patients with MF and related disorders have suggested serum P-III-P to be a useful marker of the fibroproliferative activity in the bone marrow [12][13][14][15].…”

Circulating hyaluronan in the myelofibrosis/osteomyelosclerosis syndrome and other myeloproliferative disorders

“…Of the test sample, 500 pl was incubated with the following reagents for 20 hr at 4°C during slow rotation: 1) 400 pl of I . 5 patients, HYA in serum was determined by a modification of the original radioassay (Pharmacia HYA assay; denoted assay €3). Among these, serial measurement of HYA was done in 11 patients (total number of analyses 78).…”

Procollagen type III aminoterminal peptide in serum in idiopathic myelofibrosis and allied conditions: Relation to disease activity and effect of chemotherapy

“…This assay system does not detect the small degradation products of the aminoterminal propeptide, but only serum antigens of a size similar to the intact propeptide or larger [9]. The relative contribution of these antigens to the serum level of the aminoterminal propeptide of type I11 procollagen in myeloproliferative disorders has only been reported in a single patient, in whom the disease activity was not characterized [2]. The purposes of the present investigation were-1 .…”