MO Popova1, IV Tsygankov1, YaV Gudozhnikova1, YuA Rogacheva1, NP Volkov1, KV Lepik1, MV Demchenkova2, MV Grigoreva2, AYu Efirkina2, TV Shneider3, YuV Kopeikina3, SA Stepanova3, VG Potapenko4, AV Klimovich4, NV Medvedeva4, MA Kolesnikova5, TI Pospelova5, NB Mikhailova1, VV Baikov1, AD Kulagin1
1 RM Gorbacheva Scientific Research Institute of Pediatric Oncology, Hematology and Transplantation; IP Pavlov First Saint Petersburg State Medical University, 6/8 L’va Tolstogo str., Saint Petersburg, Russian Federation, 197022
2 Irkutsk Regional Cancer Center, 32 Frunze str., Irkutsk, Russian Federation, 664035
3 Leningrad Regional Clinical Hospital, 45 bld. 2A Lunacharskogo pr-t, Saint Petersburg, Russian Federation, 194291
4 Municipal Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
5 Municipal Center for Hematology, 21 Polzunova str., Novosibirsk, Russian Federation, 630051
For correspondence: Marina Olegovna Popova, MD, PhD, 6/8 L’va Tolstogo str., Saint Petersburg, Russian Federation, 197022; Tel.: +7(911)711-39-77; e-mail: marina.popova.spb@gmail.com
For citation: Popova MO, Tsygankov IV, Gudozhnikova YaV, et al. Plasmablastic Lymphoma in HIV-Positive Patients: A Literature Review and Results of a Russian Multi-Center Retrospective Study. Clinical oncohematology. 2022;15(1):28–41. (In Russ).
DOI: 10.21320/2500-2139-2022-15-1-28-41
ABSTRACT
Background. Plasmablastic lymphoma (PBL) is a rare lymphoproliferative disease which is almost exclusively associated with immunodeficiency. Most ample experience of chemotherapy and hematopoietic stem cells transplantation (HSCT) in this lymphoma variant has been accumulated in HIV-positive patients.
Aim. To describe the current approaches to PBL diagnosis and treatment in HIV-positive patients as well as to provide the results of the first multi-center retrospective study on PBL epidemiology and therapy efficacy in HIV-positive patients in the Russian Federation.
Materials & Methods. The study included 26 HIV-positive patients with PBL who were treated and followed-up at 5 Russian centers during 2012–2019. The present study is a part of multi-center retrospective study on lymphoma epidemiology in HIV-positive patients in Russia.
Results. PBL accounted for 9.5 % of all lymphomas in HIV-positive patients enrolled in multi-center retrospective study on lymphoma epidemiology in HIV-positive patients in Russia. Epidemiological characteristics of these patients corresponded to those described in previously published literature: the disease being diagnosed mainly at late stages (88 %), oral and nasal mucosa lesions with a common involvement of facial bones (65 %), and lack of optimal HIV-infection control (66.7 %). Most commonly, the patients received EPOCH-like treatment as first-line therapy (50 %). However, the efficacy of primary therapy appeared to be low. Overall survival (OS) and progression-free survival (PFS) during a year after first-line therapy onset was 57 % and 46 %, respectively. Bortezomib included in first-line therapy was associated with a trend to a more favorable prognosis. Half of patients showed a lymphoma relapse or progression after first-line therapy. Most used second-line regimen was DHAP. Overall response to second-line therapy was 38.5 %. After second-line therapy onset, 1-year OS and PFS were 26 % and 15 %, respectively.
Conclusion. HIV-positive patients with PBL have poor prognosis. Efforts to improve the prognosis for HIV-positive patients with PBL should be aimed at increasing the efficacy of first-line therapy and should involve the use of intensive chemotherapy regimens with bortezomib. The role of auto- and allo-HSCTs in the treatment of PBL has not been clearly determined, however, PBL patients, despite their HIV-infection, should be regarded as auto-HSCT-eligible in the first remission and allo-HSCT-eligible in case of relapse. Further prospective multi-center studies are needed to optimize the treatment of HIV-positive patients with PBL.
Keywords: plasmablastic lymphoma, HIV-infection, Epstein-Barr virus, MYC, PD-1/PD-L1/2, auto-HSCT, allo-HSCT, bortezomib, nivolumab, immunotherapy.
Received: July 20, 2021
Accepted: November 27, 2021
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