Autoimmune myelofibrosis with pancytopenia as a presenting manifestation of systemic lupus erythematosus responsive to mycophenolate mofetil

Lupus. 2016 Apr;25(4):427-30. doi: 10.1177/0961203315615221. Epub 2015 Nov 3.

Abstract

Hematological abnormalities, such as anemia, leucopenia, and thrombocytopenia, secondary to peripheral destruction, are common in systemic lupus erythematosus (SLE). However, cytopenias from autoimmune myelofibrosis (AIMF) are extremely uncommon in SLE, with less than 40 reported cases in the literature. We report the case of a 33-year-old female who presented with bullous skin lesions and pancytopenia as the presenting manifestation of what was ultimately diagnosed as SLE with AIMF. She responded well to glucocorticoids and mycophenolate mofetil.

Keywords: Systemic lupus erythematosus; cutaneous lupus; hematologic changes.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy*
  • Biopsy
  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Erythematosus, Systemic / drug therapy*
  • Mycophenolic Acid / therapeutic use
  • Pancytopenia / diagnosis
  • Pancytopenia / drug therapy*
  • Pancytopenia / etiology
  • Prednisone / therapeutic use*
  • Primary Myelofibrosis / complications
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / drug therapy*
  • Treatment Outcome

Substances

  • Glucocorticoids
  • Immunosuppressive Agents
  • Mycophenolic Acid
  • Prednisone