Abstract
Most reports of post-transplant erythrocytosis have involved kidney recipients and, so far, there have been no large studies of onset of erythrocytosis after orthotopic liver transplantation (OLT) in children. We present a long-term survey of pediatric liver recipients, evaluating prevalence, outcome and the main potential causes of erythrocytosis, including a comprehensive mutational analysis of commonly related genes (mutations of HBB and HBA, JAK2, EPOR, VHL, EPAS1 and EGLN1). Between 2000 and 2015, 90 pediatric OLT recipients were observed for a median period of 8.7 years (range 1–20.4 [IQR 4.9–13.6] years). Five percent of the study population (4 males and 1 female) developed erythrocytosis at 8.5 years post OLT (range 4.1–14.9 [IQR 4.7–14.7]) at a median age of 16.6 years (range 8.2–18.8 [IQR 11.7–17.7]). Erythrocytosis-free survival after OLT was 98.6% at 5 years, 95% at 10 years, and 85% at 15 years, with an incidence rate of 6/1000 person-years. No cardiovascular events or thrombosis were reported. No germinal mutation could be clearly related to the development of erythrocytosis. One patient, with high erythropoietin levels and acquired multiple bilateral renal cysts, developed clinical hyper-viscosity symptoms, and was treated with serial phlebotomies. In conclusion, this prospective longitudinal study showed that erythrocytosis is a rare complication occurring several years after OLT, typically during adolescence. Erythrocytosis was non-progressive and manageable. Its pathogenesis is still not completely understood, although male gender, pubertal age, and renal cysts probably play a role.
Highlights
Most reports of post-transplant erythrocytosis have involved kidney recipients and, so far, there have been no large studies of onset of erythrocytosis after orthotopic liver transplantation (OLT) in children
Erythropoietin (EPO), renin-angiotensin system, insulin-like growth factor, male gender and renal cysts are some of the factors that seem to play a role in post-renal transplant erythrocytosis[5,7,8,9,10]
Post-transplant erythrocytosis has usually been described in isolated renal graft and simultaneous kidney-pancreas recipients, and mainly involves adults[5,6,7,8,11]
Summary
Most reports of post-transplant erythrocytosis have involved kidney recipients and, so far, there have been no large studies of onset of erythrocytosis after orthotopic liver transplantation (OLT) in children. With high erythropoietin levels and acquired multiple bilateral renal cysts, developed clinical hyper-viscosity symptoms, and was treated with serial phlebotomies. This prospective longitudinal study showed that erythrocytosis is a rare complication occurring several years after OLT, typically during adolescence. The main concern related to erythrocytosis in the general population, and especially in patients undergoing organ transplantation, is the risk for cardiovascular events and death, as erythrocytosis can affect transplant outcome and lead to long-term complications[5]. Prevalence and causes of erythrocytosis were studied in a cohort of 96 adults in follow up for at least one year post-orthotopic liver transplantation (OLT)[13]. The Authors reported cardiovascular events in two patients several years after OLT
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