Lymphoma in Danon disease with chronic rhabdomyolysis treated with EPOCH-R: A case report

Rare disorders often represent a challenge for clinicians and require close collaboration of an interdisciplinary team.We present the complex case of a 22-year-old male with Danon disease and late-onset of posttransplant lymphoproliferative disorder after heart transplantation. The critical aspects...

Full description

Saved in:
Bibliographic Details
Published in:Medicine (Baltimore) 2016-07, Vol.95 (29), p.e4237
Main Authors: Porpaczy, Edit, Mayerhoefer, Marius, Salzer-Muhar, Ulrike, Jaeger, Ulrich
Format: Article
Language:English
Subjects:
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Clinical Case Report
Cyclophosphamide - therapeutic use
Doxorubicin - therapeutic use
Etoposide - therapeutic use
Glycogen Storage Disease Type IIb - complications
Heart Transplantation
Humans
Lymphoma, Large B-Cell, Diffuse - diagnosis
Lymphoma, Large B-Cell, Diffuse - drug therapy
Lymphoma, Large B-Cell, Diffuse - pathology
Male
Prednisone - therapeutic use
Rhabdomyolysis - complications
Rituximab - administration & dosage
Vincristine - therapeutic use
Young Adult
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Rare disorders often represent a challenge for clinicians and require close collaboration of an interdisciplinary team.We present the complex case of a 22-year-old male with Danon disease and late-onset of posttransplant lymphoproliferative disorder after heart transplantation. The critical aspects of his condition were: pre-existing rhabdomyolysis; infiltration of muscle and gut with lymphoma; advanced clinical stage with bulky disease; nonresponsiveness to the reduction of immunosuppression and rituximab monotherapy; expected cardiotoxicity of anthracyclines. Therefore, the patient was treated with the EPOCH-R protocol, which includes continuous administration of doxorubicin over 4 days, instead of R-CHOP, in which the anthracycline is given in a short single infusion. Complete remission was achieved after the third cycle; rhabdomyolysis did not increase and heart function was not affected. The patient received a total of 6 cycles and is still in metabolic complete remission.We conclude that patients with Danon disease can be treated with anthracycline-containing chemotherapy and that continuous infusion of EPOCH-R does not exacerbate pre-existing rhabdomyolysis.
ISSN:0025-7974
1536-5964
DOI:10.1097/MD.0000000000004237