Autoimmune Myelofibrosis Associated With Systemic Lupus Erythematosus: Exceptionally Rare or Underrecognized?

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which may involve several organs or organ systems. SLE may also have hematological as well as joint, kidney, and central nervous system involvements. Hematological abnormalities such as anemia, leukopenia, thrombocytopenia, and autoimmune hemolytic anemia are the best-known and most common hematological findings detected in SLE patients which are included in the classification criteria for SLE. Autoimmune myelofibrosis (AIMF) refers to bone marrow (BM) fibrosis (myelofibrosis) that develops in an autoimmune setting. Myelofibrosis is not among the SLE classification criteria and it is also not well-known. Current reports in the literature on SLE-associated AIMF are mostly restricted to case reports or reviews of such case reports. The occurrence of BM fibrosis in SLE patients has been explored merely in few studies which concluded that myelofibrosis is a rare symptom of SLE. Herein, we propose the hypothesis that SLE-associated AIMF is not rare and, on the contrary, it can indeed be more frequent than what is known or expected.