Dr Jayana Devathasan1, Dr Tze Liang Goh2
1Waikato Hospital, New Zealand, 2Auckland City Hospital, New Zealand
Biography:
Dr Jayana Devathasan is an early career nephrologist who has worked in a number of hospitals across Aotearoa New Zealand and recently completed a nephrology fellowship in Vancouver, British Columbia. She currently works at Waikato Hospital and was the chair of the organising committee for the Aotearoa ANZSN Chapter Meeting in 2024. She has interests in haemodialysis, glomerulonephritis and medical education, particularly in the rise of FOAMed.
Background:
Patients with chronic kidney disease (CKD) have anaemia due to abnormal iron metabolism and reduced endogenous erythropoietin. Pure Red Cell Aplasia (PRCA) is a condition that has been rarely associated with erythropoietin products, commonly prescribed in patients with CKD related anaemia.
Case Report:
We describe three cases of Binocrit (epoeitin-alfa) associated PRCA which has resulted in significant morbidity. Each presented with profound anaemia and consistent bone marrow biopsy findings approximately 8-12 months after commencing subcutaneous Binocrit. We were able to confirm positive erythropoietin antibodies in two of the cases , with testing availability limiting confirmation in the third.
Case 1: 51F, European, presents with nadir haemoglobin of 51 g/L. She was treated with prednisone, ciclosporin and tacrolimus.
Case 2: 61M, NZ Maori, presents with nadir haemoglobin of 44 g/L. He was treated with ciclosporin and prednisone however his course was complicated by myositis, temporary deactivation from renal transplant listing, and commencement of dialysis, likely accelerated by profound anaemia.
Case 3: 69M, NZ European, presents with nadir haemoglobin of 67 g/L. He was treated with ciclosporin.
All of the above patients required over 20 red cell units throughout their clinical course. Each was subsequently commenced on a HIF (hypoxia inducible factor) Inhibitor to maintain their haemoglobin levels without need for further transfusion or immunosuppression.
Conclusions:
Our cluster of cases highlights the need for strong suspicion of acquired PRCA in patients treated with erythropoietin products who present with refractory anaemia. HIF-inhibitors such as Roxadustat , which have otherwise not been licensed in New Zealand, seem to be beneficial in these cases, possibly minimising the need for such heavy immunosuppression.
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