Background: A 50-year-old man who had received a simultaneous pancreas and kidney transplant 9 years earlier developed pancytopenia 3 weeks after starting azathioprine therapy to treat worsening proteinuria suspected to be caused by sirolimus.
Investigations: Laboratory tests, including complete blood counts, measurement of serum levels of vitamin B(12) and folate, liver function tests, virological assays, and thiopurine S-methyltransferase (TPMT) genotyping.
Diagnosis: Severe myelosuppression as a consequence of azathioprine therapy in a patient homozygous for the TPMT*3A allele.
Management: Discontinuation of azathioprine, treatment with an erythropoiesis-stimulating agent, red blood cell transfusions, filgrastim (a granulocyte colony-stimulating factor analogue) and folic acid.