TP53-independent apoptosis

High-Dose Chemotherapy for Grade 3B Relapsed Follicular Lymphoma with Deletions of Loci of BCL6 (3q27) and TP53 (17p13) Genes: Case Report and Literature Review

LYMPHOID TUMORS , ,

EE Zvonkov, NG Gabeeva, MV Firsova, EV Moiseeva, VV Troitskaya, LA Kuz’mina, TN Obukhova, AM Kovrigina, EN Parovichnikova, VG Savchenko

Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Nelli Georgievna Gabeeva, PhD, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167; Tel.: +7(495)612-48-10; e-mail: dr.gabeeva@gmail.com

For citation: Zvonkov EE, Gabeeva NG, Firsova MV, et al. High-Dose Chemotherapy for Relapsed Follicular Lymphoma Grade 3B with Deletions of Loci of BCL6 (3q27) and TP53 (17p13) Genes: Case Report and Literature Review. Clinical oncohematology. 2015;8(1):36–43 (In Russ).

ABSTRACT

Background. The article describes a rare case of grade 3В follicular lymphoma (FL) with a nodular-diffuse growth; it also presents a literature review. Grade 3В follicular lymphoma with a nodular-diffuse growth is a rare lymphoid neoplasm, and no optimal therapeutic strategies have been developed for it. In addition to the absence of the classic t(14;18), combined deletions of 17p13 (TР53 gene locus) and 3q27 (BCL6 gene locus) are observed in a very few cases. This combination may negatively affect the FL prognosis. The unique ability of bendamustine to induce ТР53-independent apoptosis offers new opportunities for the high-dose chemotherapy of lymphomas.

Objective. To evaluate efficacy and tolerance of the ВеЕАМ conditioning regimen using high-dose bendamustine followed by autologous HSCT in a patient with 3B FL relapse and combined 17p13 and 3q27 deletion.

Methods. A 58-year-old man was diagnosed with 3B grade FL. After 8 R-CHOP courses and a two-year maintenance therapy with Rituximab, he developed a generalized enlargement of lymph nodes, and a neoplasm in left liver lobe. A liver biopsy confirmed the relapse of grade 3B FL with a nodular-diffuse growth and a high Ki-67 level (60 %). Cytogenetic analysis identified 17p13 and 3q27 deletions.

Results. Four courses of anti-relapse therapy were performed (2 — DHAP and 2 — ICE). PET scanning showed a complete regression in lymph nodes, whereas the size of the focus in the liver and the rate of accumulation of radiopharmaceuticals in it remained unchanged. ВеЕАМ conditioning regimen (bendamustine 600 mg) followed by autologous HSCT were performed. 46 days after the autologous HSCT, the tumor focus in the liver was PET-negative. The remission retained for 10 months.

Conclusions. Further study of the efficacy of high-dose bendamustine in the therapy of aggressive lymphoid tumors with TP53 mutation seems promising.

Keywords: follicular lymphoma, TP53-independent apoptosis, bendamustine.

Received: October 24, 2014

Accepted: October 27, 2014

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