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Pure Red Cell Aplasia and Anti-Erythropoietin Antibodies In

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Pure Red Cell Aplasia and Anti-Erythropoietin Antibodies In CASE REPORTS | RELATOS DE CASO Pure red cell aplasia and anti-erythropoietin antibodies in patients on hemodialysis: a report of two cases and a literature review Aplasia pura de células vermelhas e anticorpo antieritropoietina em pacientes de hemodiálise: relato de dois casos e revisão da literatura Authors ABSTraCT RESUMO 1,2 Gabriela Lacreta Introduction: Anemia is a frequent multi- Introdução: Anemia é complicação fre- Sérgio Gardano Elias factorial complication of CKD seen in pa- quente da Doença Renal Crônica (DRC) em Bucharles1,2,3 tients on dialysis derived mainly from im- pacientes dialíticos. Apresenta caráter mul- 4 Gabriela Sevignani paired erythropoietin (EPO) production. A tifatorial principalmente pela insuficiente 2 Miguel Carlos Riella less common cause of anemia in individuals produção de eritropoietina (EPO). Situação 1,3 Marcelo Mazza do Nascimento with CKD is pure red cell aplasia (PRCA) rara causadora de anemia na DRC é Apla- secondary to the production of anti-EPO sia Pura de Células Vermelhas (APCV), em antibodies. Objective: This paper aimed decorrência da produção de anticorpos anti- 1 Universidade Federal do Paraná, two describe two cases of PRCA secondary EPO. Objetivo: Descrever 2 casos de APCV Hospital de Clínicas, Serviço de Nefrologia, Curitiba, PR, Brasil. to the production of anti-EPO antibodies com formação de anticorpos anti-EPO, 2 Fundação Pró Renal, Curitiba, including choice of treatment, patient pro- sua abordagem clínica, evolução e revisão PR, Brasil. gression, and a literature review. Materials: de literatura. Métodos: Dois pacientes em 3 Clínica Evangélico de This study included the cases of two patients hemodiálise que desenvolveram anemia Hemodiálise, Curitiba, PR, Brasil. with CKD on hemodialysis with severe ane- grave, necessitando investigação e manejo 4 Universidade Federal do Paraná, Hospital de Clínicas, Curitiba, PR, mia in need of specific investigation and específico. Resultados: Paciente nº 1: femi- Brasil. management. Results: Patient 1 with CKD nina, 75 anos, DRC secundária à hiperten- secondary to hypertension treated with EPO são arterial. Após 7 meses com EPO de- for 7 months showed persistent decreases in senvolveu queda persistente em valores de hemoglobin (Hb) levels despite the subcuta- hemoglobina (Hb) mesmo com incremento neous administration of increasing doses of em doses EPO SC, necessitando transfusões EPO; the patient required recurring blood de sangue recorrentes. Extensa investigação transfusions. Workup and imaging tests laboratorial e de imagem resultou negativa were negative for the main causes of anemia para principais causas de anemia. Paciente in individuals with CKD on dialysis. Patient nº 2: masculino, 66 anos, DRC secundária 2 with CKD secondary to adult polycystic à DRPA, há 2 anos em uso de EPO. No kidney disease had been taking EPO for 2 mês de entrada em HD desenvolveu ane- years. The patient developed severe abrupt mia severa, também exigindo transfusões anemia the month he was started on HD, recorrentes para tratamento da anemia sin- and required recurring transfusions to treat tomática. Extensa investigação laboratorial the symptoms of anemia. Workup and im- e por imagem, sem chegar a uma conclusão aging findings were inconclusive. Specific definitiva. Em ambos os casos a presença laboratory tests confirmed the patients had de anticorpos anti-EPO foi confirmada por anti-EPO antibodies. After six months of im- exames laboratoriais específicos. Terapia munosuppressant therapy (corticosteroids + imunossupressora resultou em estabilização cyclosporine) the patients were stable with do quadro e Hb > 9,0 g/dl em ambos os pa- Hb > 9.0 g/dl. Conclusion: PRCA is a rare cientes, 6 meses após início do tratamento. condition among patients on dialysis treated Conclusão: APCV é condição rara entre with rhEPO and should be considered as a pacientes dialíticos que recebem EPOHuR Submitted on: 03/02/2018. possible cause of refractory anemia. Treat- e deve ser lembrada como causa de anemia Approved on: 06/18/2018. ing patients with PRCA may be challenging, refratária. Seu manejo específico e diag- since the specific management and diagnos- nóstico laboratorial nem sempre acessível, Correspondence to: tic procedures needed in this condition are tornando desafiadora a condução dos casos Sérgio Gardano Elias Bucharles. not always readily available. para o nefrologista. E-mail: sergio_bucharles@hotmail. com Keywords: Anemia; Renal Insufficiency, Palavras-chave: Anemia; Insuficiência Re- DOI: 10.1590/2175-8239-JBN-2018-0054 Chronic; Renal Dialysis. nal Crônica; Diálise Renal. 145 Pure red cell aplasia in hemodialysis INTRODUCTION One of the less frequent causes of anemia in indi- viduals with CKD, pure red cell aplasia (PRCA) as- Anemia is a frequently observed complication in pa- sociated with EPO therapy was first described in the tients with chronic kidney disease (CKD) and indi- medical literature in the 1980s and 1990s. PRCA has viduals on chronic dialysis in particular.1,2 Anemia is attracted the interest of nephrologists on account of defined as the presence of hemoglobin levels below its challenging clinical characteristics and diagnostic 12 g/dl and 13 g/dl in females and males, respectively. and therapeutic peculiarities.8 PRCA is a rare hemato- Prevalence grows throughout the various stages of logic condition characterized by sudden onset severe CKD to reach values above 50% among patients with progressive normocytic normochromic anemia, low glomerular filtration rates < 15 ml/minute/1.73m2.3 reticulocyte count (< 10.000/mm3), and nearly com- The presence of anemia in CKD contributes to de- plete absence of erythroid precursor cells in the bone creased quality of life, increased risk of hospital- marrow.9 Patients on chronic dialysis and erythropoi- ization, and cognitive impairment, not to mention esis-stimulating agents may have PRCA secondary to associations with severe complications such as car- the formation of neutralizing anti-EPO antibodies, diovascular disease and increased mortality.4 which block the interaction of EPO and its receptors10 Although anemia in CKD is primarily caused by by neutralizing all exogenous EPO and cross-reacting impaired production of erythropoietin (EPO), various with endogenous EPO. other clinical conditions and concurrent diseases may This paper aimed to describe two cases of PRCA contribute to the onset of anemia in individuals with induced by the use of recombinant human erythropoi- advanced CKD, such as functional or absolute iron etin (rhEPO) infused subcutaneously in patients with deficiency, chronic infection, systemic inflammation, chronic kidney disease on hemodialysis at a renal re- inadequate dialysis, digestive blood loss, specific vita- placement therapy center in Curitiba, Brazil; the pa- min deficiency (vitamin B12 and folate), in addition per also aimed to perform a review on PRCA to help to osteitis fibrosa cystica and hemoglobinopathy con- nephrologists better recognize this condition and shed comitant to lack of response to stimulation with EPO. light on possible therapeutic approaches. EPO is normally produced in the interstitial fibro- blasts of the renal cortex, tubular epithelial cells, and CLINICAL CASES peritubular capillaries.5 In structural terms, EPO is a glycoprotein hormone composed by a chain of 165 CASE 1 amino acids and carbohydrates - an essential feature A.G., a retired 75-year-old Caucasian female resid- for the in vivo biological function of EPO, since par- ing in Curitiba with a history of CKD secondary to tially or completely deglycosylated EPO is rapidly de- hypertensive nephrosclerosis, had been managed con- graded in the body.5 EPO stimulates the production of servatively for nearly two years. Two months prior to red cells by binding to homodimer receptors located the start of renal replacement therapy (January 2015), in primitive erythroid progenitors and colony form- she was started on an erythropoiesis-stimulating agent ing units - erythroid, preventing the apoptosis of these (epoetin alfa); at the time, her hemoglobin (Hb) level cell types and of the initially formed erythroblasts and was below 10 g/dl. allowing cell division and the maturation of red blood At the start of hemodialysis (March 2015, Figure 1), cells.5 the patient was anemic (Hb = 8.5 g/dl) and with abso- Anemia in CKD is typically normocytic and nor- lute iron deficiency (serum ferritin: 10.1 ng/ml; serum mochromic, without leukopenia or thrombocytope- iron: 20,6 µg/dl; transferrin saturation: 4.3%). She nia. Mean survival and the production of red cells are was started on intravenous iron hydroxide and the decreased in CKD settings, although the latter is more dose of subcutaneous EPO was escalated to a weekly important.2 Serum EPO levels are in general normal 12,000 IU. Her workup improved, with hemoglobin or discretely elevated in patients with CKD and ane- reaching 12,0 g/dl in May 2015 and iron stores nor- mia, but are deemed exceedingly low in relation to malizing (serum iron: 156.1 µg/dl; ferritin: 409 ng/ the degree of anemia, since anemic patients with pre- ml; and transferrin saturation: 45.3%), while dialy- served renal function have EPO levels 10 to 100 times sis adequacy was satisfactory (KT/V > 1.2). Between higher.6,7 July and October 2015, while on EPO therapy, her Braz. J. Nephrol. (J. Bras. Nefrol.) 2019;41(1):145-151 146 Pure red cell aplasia in hemodialysis Figure 1. Timeline showing hemoglobin levels (g/dl) and main clinical events - Case 1. hemoglobin dropped significantly and gradually to she was positive for neutralizing anti-EPO antibodies. below 8,0 g/dl despite changes in EPO dosage, which Treatment with EPO was discontinued and the patient prompted the need for blood transfusions on account was started on immunosuppressant therapy (prednisone of symptomatic anemia and hospitalization to further 1 mg/kg/day with further de-escalation; and cyclosporine investigate the causes of anemia unresponsive to EPO. 4 mg/kg/day, divided in two doses). On the second semester of 2015 and on the first Hemoglobin levels improved gradually after semester of 2016 the patient underwent examination the introduction of immunosuppressant therapy.
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