Evans' syndrome, myelofibrosis and systemic lupus erythematosus: role of procollagens in myelofibrosis

Pathology. 1995 Jul;27(3):255-9. doi: 10.1080/00313029500169073.


We describe an 18 yr old female with systemic lupus erythematosus presenting with Evans' syndrome (autoimmune hemolytic anemia and immune thrombocytopenia) and dense myelofibrosis. Her clinical course and response to treatment were monitored with regular blood counts, serial measurements of serum procollagen I and III and periodic bone marrow examinations. This report brings to 9 the number of well-documented cases of systemic lupus erythematosis and myelofibrosis in the literature; we discuss the pathogenesis and management of patients with this apparently rare disorder, with particular reference to the role of serum procollagen measurements as markers of myelofibrosis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Anemia, Hemolytic, Autoimmune / blood
  • Anemia, Hemolytic, Autoimmune / etiology*
  • Biomarkers / analysis
  • Female
  • Humans
  • Lupus Erythematosus, Systemic / complications*
  • Primary Myelofibrosis / blood
  • Primary Myelofibrosis / etiology*
  • Procollagen / blood
  • Procollagen / physiology*
  • Thrombocytopenia / blood
  • Thrombocytopenia / etiology*


  • Biomarkers
  • Procollagen