학술논문
Acute myeloid leukemia with paraneoplastic pemphigus successfully treated with a personalized antileukemic and immunosuppressive strategy.
Document Type
Academic Journal
Author
Iovene FR; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy. francesca.iovene@unicampus.it.; Research Unit of Hematology and Stem Cell Transplantation, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Roma, Italy. francesca.iovene@unicampus.it.; Santinelli E; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy.; Department of Biomedicine and Prevention, PhD program in Immunology, Molecular Medicine and Applied Biotechnologies, Tor Vergata University, Rome, Italy.; Armiento D; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy.; Sarlo C; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy.; Bancone C; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy.; Research Unit of Hematology and Stem Cell Transplantation, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Roma, Italy.; Silvestri L; IRCC-IDI Istituto Dermopatico dell'Immacolata, Roma, Italy.; Erculei S; IRCC-IDI Istituto Dermopatico dell'Immacolata, Roma, Italy.; Sanhust MG; IRCC-IDI Istituto Dermopatico dell'Immacolata, Roma, Italy.; Cristiano A; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy.; Department of Biomedicine and Prevention, PhD program in Immunology, Molecular Medicine and Applied Biotechnologies, Tor Vergata University, Rome, Italy.; Fabiani E; Department of Biomedicine and Prevention, Tor Vergata University, Rome, Italy.; Divona M; Department of Biomedicine and Prevention, Tor Vergata University, Rome, Italy.; Page C; Department of Biomedicine and Prevention, Tor Vergata University, Rome, Italy.; Di Zenzo G; IRCC-IDI Istituto Dermopatico dell'Immacolata, Roma, Italy.; Cantonetti M; IRCC-IDI Istituto Dermopatico dell'Immacolata, Roma, Italy.; Rigacci L; Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, Roma, 200 - 00128, Italy.; Research Unit of Hematology and Stem Cell Transplantation, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Roma, Italy.
Source
Publisher: Springer Verlag Country of Publication: Germany NLM ID: 9107334 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-0584 (Electronic) Linking ISSN: 09395555 NLM ISO Abbreviation: Ann Hematol Subsets: MEDLINE
Subject
Language
English
Abstract
Bullous pemphigoid (BP) is a rare blistering disease often considered a primary sign of a paraneoplastic syndrome. Retrospective studies have established its link with hematological malignancies, particularly lymphoproliferative disorders. Here, we present what we believe to be the inaugural case of successful simultaneous management of BP and de novo acute myeloid leukemia (AML) in a 28-year-old male patient. Given the rarity and severity of both conditions, our treatment strategy aimed to maximize efficacy by combining immunosuppressive therapy (initially plasmapheresis with high-dose corticosteroids, followed by anti-CD20 monoclonal antibody and intravenous immunoglobulins 2 g/m 2 ) with lymphodepleting antileukemic chemotherapy utilizing Fludarabine (FLAG-IDA induction regimen). Following diagnosis, considering the patient's youth and the concurrent presence of two rare and potentially life-threatening diseases, we opted for an aggressive treatment. Upon achieving complete morphological remission of AML with measurable residual disease (MRD) negativity, despite incomplete resolution of BP, we proceeded with high-dose cytarabine consolidation followed by peripheral stem cell harvest and autologous stem cell transplantation (ASCT). Our conditioning regimen for ASCT involved Bu-Cy with the addition of anti-thymocyte globulins. At day + 100 post-ASCT, bone marrow evaluation confirmed morphological remission and MRD negativity. Meanwhile, BP had completely resolved with normalization of BP180 antibody levels.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)