MO062A RARE COMBINATION OF POLYCYTHEMIA VERA WITH A THROMBOPHILIA FACTOR V MUTATION IN A HEMODIALYSIS PATIENT :CASE REPORT
| Autor: | Soumaya Yaich, Asma Amouri, Hanen Chaker, Faten Ghabi, Khawla Kammoun, Ikram Agerbi, Toumi Salma, Mohamed Benhamida, Najla Damak |
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| Rok vydání: | 2021 |
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| Zdroj: | Nephrology Dialysis Transplantation. 36 |
| ISSN: | 1460-2385 0931-0509 |
| Popis: | Introduction Anemia is an almost constant complication in patients with end stage renal disease. In contrast, primary polycythemia is extremely rare, with only a few cases that have been reported in the literature. We report in this work a rare association of thrombophilia and acquired polycythemia in a chronic hemodialysis patient. Observation This is a 47-year-old man, non-smoking, hypertensive, with CKD in hemodialysis since 2015 secondary to undetermined nephropathy. The change in the patient's condition on hemodialysis was marked by the occurrence of repeated thrombosis of these arteriovenous fistulas(AVFs), including exploration that revealed a coagulation factor V mutation, hence his initiation of antivitamin K. The patient initially presented with severe anemia at the start of the extra renal purification , the average hemoglobin (Hb) level between 2015 and 2018 was 7.4 g / dl thus requiring the use of iron supplementation (IV iron) and stimulating agents erythropoiesis (ESA). In february 2019, erythropoietin requirements were reduced due to the increase in Hb levels and then stopped in April 2019. The Hb level continued to increase to reach 16.8 g / dl in february 2020 without receiving ESAs or iron supplements. From where an etiological investigation was initiated to search for the origin of this polycythemia going up to the genetic study for the JAK2V617F mutation which came back positive. Furthermore, gas scans, chest x-rays and abdominal ultrasounds are normal, hence polycythemia vera was retained in this patient. For fear of losing his AVF, the patient underwent intermittent phlebotomies during dialysis associated with the prescription of IEC and his Hb level rose to 14.5g / dl. Conclusion Primary erythrosis remains rare in dialysis, the pathophysiological mechanism of which remains poorly understood. Iterative phlebotomies remain the solution sometimes coupled with the prescription of IEC. |
| Databáze: | OpenAIRE |
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