TY - JOUR
T1 - Epstein-Barr Virus-associated Mucocutaneous Ulcer in a Patient with T-Cell Acute Lymphoblastic Leukemia
T2 - Importance of Accurate Diagnosis and Conservative Management
AU - Vatsayan, Anant
AU - Gupta, Ashish
AU - Ahuja, Sanjay
AU - Egler, Rachel
AU - Beck, Rose C.
AU - Matloub, Yousif
N1 - Publisher Copyright:
© 2016 Wolters Kluwer Health, Inc. All rights reserved.
Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2017
Y1 - 2017
N2 - Epstein-Barr virus-associated mucocutaneous ulcer (EBV-MCU) is a recently characterized entity that falls under the spectrum of EBV-lymphoproliferative disorders. First described in 2010 by Dojcinov et al, it is an EBV-driven localized proliferation of B cells, occurring in mucocutaneous tissues including the skin, the oropharynx, and the gastrointestinal tract of immunosuppressed patients in the absence of an intact T-cell repertoire. Typically, it has been described in elderly patients with age-related immunosenescence and patients who are on immunosuppressive therapy. However, only 2 cases have been reported in pediatric, adolescent, and young adult age groups, with all these patients manifesting after solid organ transplant. To the best of our knowledge there are no case reports of EBV-MCU occurring in association with hematologic malignancy. Here, we present a case of EBV-MCU in a young adult patient with T-cell acute lymphoblastic leukemia. Our report serves to promote awareness among clinicians regarding this newly described and extremely rare clinical entity in young immunosuppressed patients. In addition, we highlight the importance of accurate diagnosis to prevent overtreatment of this indolent, often self-resolving disease that has a significant clinicopathologic overlap with other aggressive forms of EBV-lymphoproliferative disorders that require more intensive therapy.
AB - Epstein-Barr virus-associated mucocutaneous ulcer (EBV-MCU) is a recently characterized entity that falls under the spectrum of EBV-lymphoproliferative disorders. First described in 2010 by Dojcinov et al, it is an EBV-driven localized proliferation of B cells, occurring in mucocutaneous tissues including the skin, the oropharynx, and the gastrointestinal tract of immunosuppressed patients in the absence of an intact T-cell repertoire. Typically, it has been described in elderly patients with age-related immunosenescence and patients who are on immunosuppressive therapy. However, only 2 cases have been reported in pediatric, adolescent, and young adult age groups, with all these patients manifesting after solid organ transplant. To the best of our knowledge there are no case reports of EBV-MCU occurring in association with hematologic malignancy. Here, we present a case of EBV-MCU in a young adult patient with T-cell acute lymphoblastic leukemia. Our report serves to promote awareness among clinicians regarding this newly described and extremely rare clinical entity in young immunosuppressed patients. In addition, we highlight the importance of accurate diagnosis to prevent overtreatment of this indolent, often self-resolving disease that has a significant clinicopathologic overlap with other aggressive forms of EBV-lymphoproliferative disorders that require more intensive therapy.
KW - Epstein-Barr virus
KW - Epstein-Barr virus-associated lymphoproliferative disorder
KW - Epstein-Barr virus-positive mucocutaneous ulcer
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U2 - 10.1097/MPH.0000000000000709
DO - 10.1097/MPH.0000000000000709
M3 - Article
C2 - 27879545
AN - SCOPUS:84996921054
SN - 1077-4114
VL - 39
SP - e338-e341
JO - Journal of pediatric hematology/oncology
JF - Journal of pediatric hematology/oncology
IS - 6
ER -