Lymphoma in Danon disease with chronic rhabdomyolysis treated with EPOCH-R: A case report. Issue 29 (July 2016)

• Record Type: Journal Article
• Authors: Porpaczy, Edit; Mayerhoefer, Marius; Salzer-Muhar, Ulrike; Jaeger, Ulrich
• Other Names: Liu. Tao section editor.
• Is Part Of: Medicine. Volume 95:Issue 29(2016)
• Publication Date: 2016-07
• Subjects: Danon disease -- EPOCH-R -- glycogen storage disease -- LAMP2 -- posttransplant lymphoproliferative disorder -- PTLD -- rhabdomyolysis; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000004237
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract : Abstract: 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.