Azathioprine‐induced pancytopenia: A case report

Abstract

Azathioprine (AZA) is commonly used as immunosuppressive therapy for autoimmune diseases, including systemic lupus erythematosus (SLE). Myelosuppression is a common side effect of AZA. Here we report a case of severe myelosuppression following AZA therapy in a 15-year-old girl despite a normal thiopurine methyltransferase (TPMT) level. She had been receiving AZA for SLE and presented with neutropenic fever and pancytopenia. AZA was stopped. After stopping AZA, her blood counts steadily improved. When TPMT genotyping results were normal, AZA was reintroduced. Pancytopenia reappeared after starting AZA, despite normal TPMT genotype. AZA was replaced with mycophenolate mofetil which consequently resulted in improvement of blood counts. It is essential to understand the temporal relationship between AZA use and pancytopenia onset in patients with normal TPMT activity. This case illustrates that regular monitoring of blood cell counts should be routine practice after starting AZA regardless of TPMT activity.azathioprine, systemic lupus erythematosus, thiopurine methyltransferase Key points• Azathioprine-induced myelosuppression can occur in patients with normal thiopurine methyltransferase (TPMT) activity. • Total cell counts should be evaluated on a frequent basis after initiating azathioprine even in patients with normal TMPT activity.

Targeting mechanistic target of rapamycin complex 2 attenuates immunopathology in Systemic Lupus Erythematosus

“…It presents various clinical manifestations, from mild skin rashes to life-threatening multi-organ damages such as nephritis, cerebritis, and myelofibrosis (2). Broad immunosuppressive agents such as azathioprine are commonly used for SLE management which may not lead to optimal disease control and can lead to undesired side effects (3). Unlike rheumatoid arthritis with numerous disease-specific biologics, only two biological agents, belimumab and anifrolumab, are approved for SLE treatment (4).…”