Mahdieh Rajabi-Moghaddam - Dept. of Pathology, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran
Gholamali Sarparast - Dept. of Hematology and Oncology, Iranmehr Hospital, Birjand University of Medical Sciences, Birjand, Iran
Hamid Abbaszadeh - Dept. of Oral and Maxillofacial Pathology, School of Dentistry, Birjand University of Medical Sciences, Birjand, Iran

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 ۳۳- year- old female patient with an undiagnosed SLE which presented with acute bicytopenia. Absence of splenomegaly, leukopenia, anemia, BMF (grade MF-۱), 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.

Autoimmune diseases, Primary Myelofibrosis, Fibrosis, Lupus Erythematosus, Systemic