Therapeutic implications of collagen metabolism in myelofibrosis.
Myelofibrosis is an idiopathic condition of man associated with increased deposition of bone marrow collagen. This is directly seen on bone marrow reticulin or collagen staining, and reflected by increased levels of serum procollagen III aminoterminal peptide (28). It is but one of a number of diseases in which collagen, and an aberration of the connective tissue matrix, influence normal anatomy and physiology. Animal experiments have shown that the therapeutic manipulation of the intra- and extracellular processing of collagen with various agents can influence its deposition and the degree of end-organ damage. Several of these drugs have been used therapeutically for various clinical conditions and deserve clinical trials to evaluate their effectiveness in myelofibrosis. Bone marrow fibrosis, and peripheral serum markers which reflect collagen metabolism could be followed as experimental parameters. Development of an effective and safe antifibroblastic therapy is awaited eagerly. An attempt to prevent the fibrosis found in myelofibrosis, as opposed to managing its complications, would revolutinize our approach to managing these patients.
D000629:Aminopropionitrile / Q000627:therapeutic use; D000818:Animals; D003094:Collagen / Q000378:metabolism*; D006801:Humans; D006900:Hydroxylation; D006909:Hydroxyproline / Q000378:metabolism; D010396:Penicillamine / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000188:drug therapy* / Q000378:metabolism; D051381:Rats
D000629:Aminopropionitrile; D003094:Collagen; D010396:Penicillamine; D006909:Hydroxyproline