title: Lymphoma in Danon disease with chronic rhabdomyolysis treated with EPOCH-R: A case report.
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.
authors.affiliation: Department of Internal Medicine I, Division of Hematology and Hemostaseology, Comprehensive Cancer Center Department of Biomedical Imaging and Image-Guided Therapy Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Pediatric Heart Center Vienna, Medical University of Vienna, Vienna, Austria.
authors.forename: Edit
authors.initials: E
authors.lastname: Porpaczy
authors.forename: Marius
authors.initials: M
authors.lastname: Mayerhoefer
authors.forename: Ulrike
authors.initials: U
authors.lastname: Salzer-Muhar
authors.forename: Ulrich
authors.initials: U
authors.lastname: Jaeger
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D052120:Glycogen Storage Disease Type IIb / Q000150:complications*; D016027:Heart Transplantation; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D012206:Rhabdomyolysis / Q000150:complications*; D000069283:Rituximab / Q000008:administration & dosage; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000069283:Rituximab; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone