TY - JOUR
T1 - Refractory Hydroa Vacciniforme-Like Lymphoma
T2 - Biological Insights from Morphoproteomic Analysis
AU - Goswamy, Rohit
AU - Ajufo, Helen
AU - Maiti, Abhishek
AU - Brown, Robert
AU - Juneja, Harinder
AU - Apostolidou, Effrosyni
N1 - Publisher Copyright:
© 2022 Tehran University of Medical Sciences.
PY - 2022
Y1 - 2022
N2 - T-cell/natural killer cell lymphoproliferative disorders are rare, associated with poor overall survival, and have limited treatment options. We report a case of a patient who developed hydroa vacciniforme-like lymphoma (HVLL, an EBV-peripheral T-cell lymphoma), refractory to multiple lines of systemic therapy including methotrexate, mycophenolate mofetil, dapsone, thalidomide, prednisone, and romidepsin. We conducted morphoproteomic analysis of the patient’s tumor which provided important biological insights. Histopathology showed primarily lymphohistiocytic infiltrates strongly positive EBV expression with a Ki-67 of >50% in the pretreatment biopsy and approximately 90% in the post-treatment biopsy, strong expression of Enhancer of Zester Homolog 2 (EZH2), a constitutively active mTOR pathway, 50% cytoplasmic BCL-2 expression; largely negative PD-1 positive CD8 T-cells. Based on this morphoproteomic analysis and published literature, we postulated that novel agents, including venetoclax, tazemetostat, and other agents may provide a targeted approach for treating HVLL. This case illustrates the use of morphoproteomic analysis to better understand the biology of tumors.
AB - T-cell/natural killer cell lymphoproliferative disorders are rare, associated with poor overall survival, and have limited treatment options. We report a case of a patient who developed hydroa vacciniforme-like lymphoma (HVLL, an EBV-peripheral T-cell lymphoma), refractory to multiple lines of systemic therapy including methotrexate, mycophenolate mofetil, dapsone, thalidomide, prednisone, and romidepsin. We conducted morphoproteomic analysis of the patient’s tumor which provided important biological insights. Histopathology showed primarily lymphohistiocytic infiltrates strongly positive EBV expression with a Ki-67 of >50% in the pretreatment biopsy and approximately 90% in the post-treatment biopsy, strong expression of Enhancer of Zester Homolog 2 (EZH2), a constitutively active mTOR pathway, 50% cytoplasmic BCL-2 expression; largely negative PD-1 positive CD8 T-cells. Based on this morphoproteomic analysis and published literature, we postulated that novel agents, including venetoclax, tazemetostat, and other agents may provide a targeted approach for treating HVLL. This case illustrates the use of morphoproteomic analysis to better understand the biology of tumors.
KW - Epstein-Barr virus
KW - Hydroa vacciniforme-like lymphoma (HVLL)
KW - Morphoproteomics
KW - Resistance mechanisms
KW - T-cell lymphoproliferative disorders
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M3 - Article
AN - SCOPUS:85134010314
SN - 1735-1243
VL - 16
SP - 174
EP - 180
JO - International Journal of Hematology-Oncology and Stem Cell Research
JF - International Journal of Hematology-Oncology and Stem Cell Research
IS - 3
ER -