Autoimmune myelofibrosis (AIMF) is considered as an infrequent cause of bone marrow fibrosis (BMF) and a rare complication of systemic lupus erythematosus (SLE). Due to its rarity, it is mistakenly diagnosed as primary myelofibrosis (MF).
We describe the clinicopathologic features of a secondary form of AIMF in a 33- year- old female patient with an undiagnosed SLE which presented with acute bicytopenia. Absence of splenomegaly, leukopenia, anemia, BMF (grade MF-1), and presence of autoantibodies were some of noticeable features. Treatment with corticosteroid led to complete regeneration of the bone marrow and subsequently to an improved hematological status. Six- month follow-up showed that the patient was in good clinical condition.
Identification of AIMF is a diagnostic challenge and pitfall and it is actually a diagnosis of exclusion. It could be the first and only presenting feature of SLE and results in hematologic disturbances. So, we should consider SLE-associated AIMF in the differential diagnosis of pancytopenia.