Case report: (non)chelated, poly-transfused patient with 5q minus syndrome

Czech version

Authors: E. Kadlčková ¹; R. Neuwirtová ²; P. Salajka ³; Červínek L.⁴; A. Jonášová ²
Authors‘ workplace: Hematologicko-transfuzní oddělení Krajské nemocnice T. Bati, Zlín ¹; I. interní klinika hematologie Všeobecné fakultní nemocnice Praha, 1. lékařská fakulta Univerzity Karlovy, Praha ²; Patologicko-anatomické oddělení Krajské nemocnice T. Bati, Zlín ⁴Interní hematoonkologická klinika Fakultní nemocnice, Brno-Bohunice ³
Published in: Transfuze Hematol. dnes,21, 2015, No. 3, p. 136-142.
Category: Comprehensive Reports, Original Papers, Case Reports

Overview

The authors present a case of a 60-year-old patient with incidentally uncovered significant macrocytic anaemia and thrombocytosis. Myelodysplastic syndrome, namely 5q- syndrome was diagnosed. From the beginning, the patient was dependent on red blood cell transfusions. Chelation was initially successful, but later it failed especially due to the patient’s non compliance. At the time when treatment with lenalidomide was clearly indicated, it was not possible to obtain this medication. At 53 months from diagnosis, the disease progressed to RAEB. The patient died of liver and renal failure without developing acute leukaemia. Severe hemosiderosis of the organs, especially the liver and heart, was one of the causes of death. The article includes the autopsy report and photographs of the relevant histological slides, showing hemosiderosis of the vital organs and pathological-anatomical changes that led to their failure. The paper shows severe organ damage that occurs in patients with untreated iron overload. However, haematologists can currently prevent fatal organ damage in long-term transfused patients suffering from myelodysplastic syndrome with the aid of timely and consistent iron chelator therapy and by attempting to elimi-nate the dependence on transfusions using erythropoietin or in case of its failure by administering lenalidomide.

Key words:
5q-syndrome, iron overload, iron chelation

Sources

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Labels

Haematology Internal medicine Clinical oncology

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New screening tests for syphilis in blood donors

Article was published in

Transfusion and Haematology Today

2015 Issue 3