Treatment of mantle cell lymphoma in Asia: a consensus paper from the Asian Lymphoma Study Group

Abstract

Background: Mantle cell lymphoma (MCL) is a B cell malignancy that can be aggressive and with a poor prognosis; the clinical course is heterogeneous. The epidemiology of MCL in Asia is not well documented but appears to comprise 2-6% of all lymphoma cases based on available data, with variation observed between countries. Although international guidelines are available for the treatment of MCL, there is a lack of published data or guidance on the clinical characteristics and management of MCL in patient populations from Asia. This paper aims to review the available treatment and, where clinical gaps exist, provide expert consensus from the Asian Lymphoma Study Group (ALSG) on appropriate MCL management in Asia. Body: Management strategies for MCL are patient-and disease stage-specific and aim to achieve balance between efficacy outcomes and toxicity. For asymptomatic patients with clearly indolent disease, observation may be an appropriate strategy. For stage I/II disease, following international guidelines is appropriate, which include either a short course of conventional chemotherapy followed by consolidated radiotherapy, less aggressive chemotherapy regimens, or a combination of these approaches. For advanced disease, the approach is based on the age and fitness of the patient. For young, fit patients, the current practice for induction therapy differs across Asia, with cytarabine having an important role in this setting. Hematopoietic stem cell transplantation (HSCT) may be justified in selected patients because of the high relapse risk. In elderly patients, specific chemoimmunotherapy regimens available in each country/region are a treatment option. For maintenance therapy after first-line treatment, the choice of approach should be individualized, with cost being an important consideration within Asia. For relapsed/ refractory disease, ibrutinib should be considered as well as other follow-on compounds, if available. Conclusion: Asian patient-specific data for the treatment of MCL are lacking, and the availability of treatment options differs between country/region within Asia. Therefore, there is no clear one-size-fits-all approach and further investigation on the most appropriate sequence of treatment that should be considered for this heterogeneous disease.

Real‐world outcomes of ibrutinib therapy in Korean patients with relapsed or refractory mantle cell lymphoma: a multicenter, retrospective analysis

“…Another explanation is that our patients tolerated better the ibrutinib treatment, which resulted in a longer duration of treatment (Table 1). Based on the favorable outcomes, a recent consensus guideline recommended the use of ibrutinib in the second-line rather than later-line setting [9]. Compared to previous studies [10], the post-ibrutinib OS was more favorable in the present study at 19.4 months (vs. 5.8 months).…”

Progress in molecular feature of smoldering mantle cell lymphoma

“…Variants with a low frequency of SOX11 negativity and IGHV-mutated genes are more indolent and associated with better prognosis. It is useful to confirm SOX11 negativity with hypermutated IGHV to identify a clearly indolent disease [ 54 , 55 ]. Sílvia et al demonstrated that SOX11 knockdown reduced engrafted tumor growth in vivo, which is consistent with the indolent clinical course of human SOX11-negative MCL [ 38 ].…”

“…In recent years, some cases of MCL have been characterized by IGHV mutations, and they are truly associated with an indolent disease course [ 57 , 59 ]. The typical clinical presentation of patients with IGHV mutations comprises leukemic non-nodal CLL-like, including splenomegaly, a Ki-67 proliferation fraction < 10%, a low tumor burden and noncomplex karyotypes [ 55 ]. The identified VH rearrangements in indolent MCL were VH1, VH2, VH3 and VH4 [ 3 ].…”

Treatment Outcomes and Survival Patterns of Asian Patients With Relapsed/Refractory Mantle Cell Lymphoma

“…Most recently, the advent of Bruton’s tyrosine kinase (BTK) inhibitors represented a breakthrough in the treatment of B-cell NHL and has proven beneficial in clinical trials on MCL [ 11 , 12 ]. A recent consensus paper by the Asian Lymphoma Study Group has also recommended the use of BTK inhibitors for R/R MCL [ 13 ]. However, treatment outcomes with each subsequent line of therapy, especially involving the use of novel-therapies, remain poorly characterized.…”