Autoimmune myelofibrosis secondary to systemic lupus erythematosus: a diagnostic challenge in a resource-limited setting.

Abstract

Autoimmune myelofibrosis (AIMF) is a rare, potentially reversible cause of bone marrow failure often linked to systemic autoimmune disease. We report a diagnostically challenging case of a 26-year-old woman from sub-Saharan Africa who presented with transfusion-dependent pancytopenia and an initial dry-tap marrow, with further testing limited by financial constraints. Seven months later, evolving clinical and immunologic features-including fever, leukopenia, thrombocytopenia, malar rash, positive ANA, and a sterile exudative pleural effusion-fulfilled the 2019 EULAR/ACR criteria for systemic lupus erythematosus (SLE). AIMF secondary to SLE was subsequently diagnosed, and she demonstrated a rapid hematologic response to glucocorticoids and hydroxychloroquine, remaining transfusion-independent for over five months. This case underscores the diagnostic complexity of AIMF in resource-limited settings and highlights the importance of sustained clinical suspicion and adaptable diagnostic strategies when evaluating unexplained pancytopenia.