Hematologic involvement is a common manifestation during systemic lupus erythematosus (SLE). Pancytopenia represents an infrequent mode of revelation, most often of peripheral origin, exceptionally secondary to a bone marrow disorder and particularly to an autoimmune myelofibrosis (AIMF). This entity, distinct from a primary myelofibrosis (MFP), is characterized by reticulin fibrosis of the bone marrow lack of atypical bone marrow cells, the presence of auto-antibodies and absence of classical signs of myeloproliferation. Generally the AIMF associated to the SLE had a favorable evolution and appears to often respond to corticosteroids and/or immunosuppressive treatments. This case illustrates the original association of an SLE revealed by a pancytopenic MFAI in a male patient with a dramatic improvement under corticosteroids.
Keywords: autoimmune myelofibrosis; pancytopeny; systemic lupus erythematosus.