Systemic Lupus Erythematosus-Associated Autoimmune Myelofibrosis Mimicking TAFRO Syndrome: A Case Report and Literature Review

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder with diverse hematologic manifestations, rarely complicated by autoimmune myelofibrosis (AIMF), a non-clonal and potentially reversible form of marrow fibrosis.Differentiating SLE-associated AIMF from TAFRO syndrome can be difficult because both conditions share overlapping features, including cytopenias, effusions, renal dysfunction, systemic inflammation, and reticulin fibrosis.We describe a 33-year-old woman who presented with recurrent low-grade fever, pedal edema, ascites, and dyspnea.Laboratory evaluation revealed pancytopenia, Coombs-positive hemolytic anemia, proteinuric renal disease, elevated inflammatory markers, antinuclear antibodies (ANA) positivity (1:2560, speckled pattern), high anti-dsDNA titers, and low complement C3 levels.Bone marrow biopsy demonstrated grade III reticulin fibrosis with increased megakaryocytes without significant atypia, while testing for myeloproliferative driver mutations, including JAK2, CALR, and MPL, was negative.Although the patient fulfilled several clinical criteria suggestive of TAFRO syndrome, lymph node biopsy showed only reactive hyperplasia without features of idiopathic multicentric Castleman disease, supporting a diagnosis of SLE-associated AIMF.She was treated with intravenous methylprednisolone pulses followed by oral prednisolone, hydroxychloroquine, intravenous immunoglobulin, and rituximab, resulting in marked clinical and hematologic improvement, with remission achieved within six weeks.This case highlights the importance of careful serologic, histopathologic, and clinicopathologic correlation in distinguishing AIMF from TAFRO-like syndromes, as prompt recognition and immunosuppressive therapy can lead to reversal of marrow fibrosis and favorable outcomes.