TY - JOUR
T1 - Case Report
T2 - Very Late, Atypical Extra-Medullary Relapse in a Patient With Acute Promyelocytic Leukemia (APL) Rescued With a Transplant-Free Approach
AU - Molica, Matteo
AU - Mazzone, Carla
AU - Ottone, Tiziana
AU - Niscola, Pasquale
AU - Abruzzese, Elisabetta
AU - Fratoni, Stefano
AU - Voso, Maria Teresa
AU - de Fabritiis, Paolo
N1 - Funding Information:
The authors thank Prof. Nathan Tublitz for help in revising the manuscript.
Publisher Copyright:
© Copyright © 2021 Molica, Mazzone, Ottone, Niscola, Abruzzese, Fratoni, Voso and de Fabritiis.
PY - 2021/6/29
Y1 - 2021/6/29
N2 - Relapses of acute promyelocytic leukemia (APL) beyond 7 years from the first molecular remission are exceptional, and it is unclear whether these relapses represent a new, therapy-related leukemia rather than a delayed relapse of the original leukemic clone. The increase extra-medullary relapses (ER) in the era of all-trans retinoic acid (ATRA) therapy suggests a potential correlation between ATRA therapy and ER, and several potential explanations have been proposed. The gold standard post-remission approach, particularly for patients in late relapse, has not yet been established. The benefit of a transplant approach has been questioned in this setting because continuing ATRA-arsenic trioxide (ATO) might be curative. Here we report on the case of an APL patient who relapsed 9 years after achieving her first molecular complete remission (mCR) and who showed an atypical isolated localization at nodal sites, including the into- and peri-parotid glands. Genomic PML/RARa breakpoint analysis detected the same bcr3 PML/RARa hybrid gene in DNA purified from bone marrow and lymph nodes, suggesting that the relapse was because of the reemergence of the initial clone. This case shows that APL, treated with ATRA and cytotoxic drugs, may still emerge in extra-medullary sites even after a very prolonged mCR and could be salvaged with an ATO-based protocol, not including a transplant approach.
AB - Relapses of acute promyelocytic leukemia (APL) beyond 7 years from the first molecular remission are exceptional, and it is unclear whether these relapses represent a new, therapy-related leukemia rather than a delayed relapse of the original leukemic clone. The increase extra-medullary relapses (ER) in the era of all-trans retinoic acid (ATRA) therapy suggests a potential correlation between ATRA therapy and ER, and several potential explanations have been proposed. The gold standard post-remission approach, particularly for patients in late relapse, has not yet been established. The benefit of a transplant approach has been questioned in this setting because continuing ATRA-arsenic trioxide (ATO) might be curative. Here we report on the case of an APL patient who relapsed 9 years after achieving her first molecular complete remission (mCR) and who showed an atypical isolated localization at nodal sites, including the into- and peri-parotid glands. Genomic PML/RARa breakpoint analysis detected the same bcr3 PML/RARa hybrid gene in DNA purified from bone marrow and lymph nodes, suggesting that the relapse was because of the reemergence of the initial clone. This case shows that APL, treated with ATRA and cytotoxic drugs, may still emerge in extra-medullary sites even after a very prolonged mCR and could be salvaged with an ATO-based protocol, not including a transplant approach.
KW - acute promyelocitic leukemia
KW - all-trans retinoic acid and arsenic trioxide combination treatment
KW - bcr3 variant
KW - transplant free approach
KW - very late relapse
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U2 - 10.3389/fonc.2021.699886
DO - 10.3389/fonc.2021.699886
M3 - Article
AN - SCOPUS:85110229881
VL - 11
JO - Frontiers in Oncology
JF - Frontiers in Oncology
SN - 2234-943X
M1 - 699886
ER -