Heart transplantation and follow-up treatment with AL-amyloidosis in 5 patients


Authors: Zdeněk Adam 1;  Eva Ozábalová 2;  Petr Němec 3;  Helena Bedáňová 3;  Milan Kuman 3;  Jan Krejčí 2;  Lenka Špinarová 2;  Víta Žampachová 4;  Zdeňka Čermáková 5;  Luděk Pour 1;  Marta Krejčí 1;  Viera Sanecká 1;  Martin Štork 1;  Tomáš Pika 6;  Jan Straub 7;  Dagmar Adamová 8;  Yvetta Stavařová 9;  Zdeněk Král 1;  Jiří Mayer 1
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice 1;  I. interní kardio-angiologická klinika LF MU a FN u sv. Anny v Brně 2;  Centrum kardiovaskulární a transplantační chirurgie, Brno 3;  I. patologicko-anatomický ústav LF MU a FN u sv. Anny v Brně 4;  Oddělení klinické biochemie FN Brno, pracoviště Bohunice 5;  Hemato-onkologická klinika LF UP a FN Olomouc 6;  I. interní klinika – klinika hematologie 1. LF UK a VFN v Praze 7;  Hematologicko-transfúzní oddělení Slezské nemocnici v Opavě, p. o. 8;  Hematologicko-transfuzní oddělení Krajské nemocnice T. Bati, a. s., Zlín 9
Published in: Vnitř Lék 2018; 64(4): 441-449
Category: Case Reports

Overview

The prognosis for patients with cardiac impairment due to AL-amyloid deposition and severe cardiac insufficiency is poor, with a survival median in the order of months. The classical treatment of AL-amyloidosis in combination with cardiac insufficiency is very poorly tolerated and the treatment of such patients is associated with considerably higher mortality than among other patients with AL-amyloidosis. If, however, patients with an isolated or another dominating cardiac impairment, without severe damage to other organs and tissues, have a heart transplant performed, their cardiovascular condition will significantly improve as a result, along with their ability to tolerate any kind of treatment for AL-amyloidosis including that using high-dose chemotherapy with a transplant of autologous hematopoietic stem cells. The achievement of complete remission of AL-amyloidosis is a precondition for long-term survival, since when not achieved, amyloid deposition also arises in the transplanted heart. At the Centre for Cardiovascular and Transplantation Surgery, Brno, the first heart transplant due to its impairment by AL-amyloidosis was performed in 2010. By the year 2017 the number of patients with AL-amyloidosis, who had first undergone a heart transplant with subsequent treatment for AL-amyloidosis, increased to 5. The median age at which a heart transplant was performed is 60 (48–65) years. Four patients were men, one was a woman. The median monitoring equals 65 (88–15) months. Complete remission of AL-amyloidosis was achieved in all the patients. There were 5 lines of treatment needed for the first patient to attain it, of that twice high-dose melphalan with autologous stem cell transplantation, for the second patient a second-line treatment, high-dose melphalan and bortezomib-based therapy. No specific therapy was needed for the third patient, as immunosuppressive therapy following the heart transplant containing prednison led to complete remission of AL-amyloidosis. In the fourth case, sustainable complete remission was reached by high-dose melphalan and in the fifth case by one line of bortezomib-based therapy. The aforementioned data illustrate that a heart transplant is the first step which makes the patients with a severe heart failure, not tolerating any efficient therapy of AL-amyloidosis, capable of undergoing intense treatment of AL-amyloidosis. Sometimes one high-dose chemotherapy is sufficient, while at other times multiple treatment lines are needed to reach complete remission of AL-amyloidosis.

Key words:
AL-amyloidosis – autologous hematopoietic stem cells transplantation – bortezomib – cardiomyopathy – lenalidomide – thalidomide – heart transplantation


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