Autoimmune pancytopenia occurring late after simultaneous pancreas and kidney transplantation

Abstract

A 35-year-old woman presented with a widespread petechial rash and pancytopenia. She underwent simultaneous pancreas and kidney transplantation for type 1 diabetes 8 years previously followed by a renal transplant 1 year prior to presentation, and was taking tacrolimus as long-term immunosuppression. The full blood count showed haemoglobin 97 g/L, platelet count 2×10⁹/L and neutrophil count 0.22×10⁹/L. Peripheral blood film examination confirmed genuine thrombocytopenia in the absence of any haemolytic or malignant features. Serological testing identified autoantibodies against all three blood lineages, consistent with a diagnosis of autoimmune pancytopenia. Treatment with steroids, intravenous immunoglobulins, romiplostim and mycophenolate mofetil achieved only fleeting remissions. Blood counts eventually normalised following the administration of rituximab and a change from tacrolimus to ciclosporin immunosuppression. Cytopenias are a well-recognised complication of post-transplantation care but we believe this to be the first reported case of autoimmune pancytopenia following solid organ transplantation. In this case report, we discuss the approach to investigation of haematological abnormalities post-transplant and the rationale for, and outcome of, the management of this rare case.

Keywords: haematology (drugs and medicines); haematology (incl blood transfusion); pathology; renal transplantation; unwanted effects / adverse reactions.

Figure 1

Bone marrow examination at diagnosis. (A) Aspirate prepared with modified Wright's stain, demonstrates granulocyte maturation arrest, predominantly at the promyelocyte stage with absence of mature neutrophils. (B) Trephine prepared with H&E (×20) demonstrates marrow hypercellularity. (C) Trephine with CD68P immunostaining highlights macrophage activation with haemophagocytosis. (D) Trephine with glycophorin A immunostaining demonstrates severe erythroid hypoplasia.

Figure 2

Weekly blood counts since presentation accompanied by key interventions. IVIg, intravenous immunoglobulin; MMF, mycophenolate mofetil.