Composite and sequential lymphoma between classical Hodgkin lymphoma and primary mediastinal lymphoma/diffuse large B‐cell lymphoma, a clinico‐pathological series of 25 cases

Abstract

Composite and sequential lymphomas involving both classical Hodgkin lymphoma (CHL) and primary mediastinal B-cell lymphoma (PMBCL) are rare phenomena. Beyond the relevant biological interest raised by these cases, treatments and outcome data are poorly covered in the recent literature. This retrospective analysis describes the pathological and clinical characteristics of 10 composite and 15 sequential cases included after a central pathological review. At diagnosis, 70% of the composite lymphomas presented a disseminated and extranodal disease. Among the 15 sequential lymphomas, 12 were CHL at first occurrence and three were PMBCL. Based on their clinical evolution, these sequential lymphomas could be divided into early (i.e., diagnosis of second lymphoma within a year) and late [(i.e., a second lymphoma occurrence occurring after a long period of complete remission]). All composite cases were alive in complete remission after a median follow-up of 34 months. If the early sequential lymphoma presented a particularly poor outcome with a median overall survival shorter than one year, the late cases were efficiently salvaged. Further molecular studies are needed to describe the underlying biology of these rare diseases, possibly representing the extreme of tumour cell plasticity found in grey-zone lymphoma.

Hodgkin Reed–Sternberg-Like Cells in Non-Hodgkin Lymphoma

“…Another CL type is PMBL combined with cHL [86]. The existence of this form as well as GZL suggests lineage plasticity between the two molecularly related entities, cHL and PMBL [87], driven by abnormalities involving the JAK/STAT [88] and/or other molecular pathways [89].…”

Incidence Patterns of Sequential or Composite Lymphoma: A Population-Based Cancer Registry Study

“…than that in the US (4.7%) (Kim 1993), Italy (13%) (Tucci et al 2005), Germany (0.9%) (Khristi et al 2013), and France (12%) (Aussedat et al 2020). The reasons for the lower frequency of multiple histological types of lymphoma in a patient in the present study are unknown, but a possible reason might be related to diff erence in study design, disease definition, and ethnicity of the study subjects.…”

Clinical outcomes of composite and sequential B‐cell lymphomas with features intermediate between DLBCL/PMBCL and classical Hodgkin Lymphoma from the SEER database

“…In conclusion, the existence of a detectable number of cases of CL and SL in a population‐based cancer registry justifies the recent efforts for precise histological definition of these entities such as the work of Aussedat et al 1 as well as the elucidation of their underlying molecular features with the hope for improvement of their clinical management and outcome.…”

“…[5][6][7] Of particular interest is the observation that a second large B-cell lymphoma (Figure S4) can develop in CHL survivors after a long latency and this does worsen the prognosis, making the overall survival of those patients similar to that of those with CL at initial diagnosis. In conclusion, the existence of a detectable number of cases of CL and SL in a population-based cancer registry justifies the recent efforts for precise histological definition of these entities such as the work of Aussedat et al 1 as well as the elucidation of their underlying molecular features with the hope for improvement of their clinical management and outcome.…”

“…Recently, Aussedat et al published a retrospective report on the clinical and pathological features of composite (CL) and sequential lymphomas (SL) with features intermediate between diffuse large B-cell lymphoma (DLBCL) or primary mediastinal B-cell lymphoma (PMBCL) and classical Hodgkin lymphoma (CHL). 1 Their report was based on the pathological reassessment of cases from the LYSA (LYmphoma Study Association) centres. This report emphasises the role of careful pathological assessment as well as of the close follow-up and registration.…”