Composite Classic Hodgkin Lymphoma and Follicular Lymphoma

Abstract

Composite classic Hodgkin lymphoma and follicular lymphoma (CHLFL) is a rare and poorly characterized entity. Herein, we report the clinicopathologic features of 22 cases of CHLFL from 3 institutions and we assess 27 additional cases reported in the literature. In our cohort (n = 22), patients with CHLFL had a median age of 61 years and an equal male to female incidence. Most cases (95%) arose de novo with the remaining patients having a history of non-Hodgkin lymphoma. CHLFL always involved lymph nodes (100%) and most cases (95%) revealed 2 distinct areas separately diagnostic for CHL and FL. The CHL component represented a variable proportion of the overall neoplasm (5% to 90%) and was either mixed cellularity (82%) or nodular sclerosis (18%) type. The Hodgkin/ Reed-Sternberg cells expressed CD30 (100%), PAX5 (100%), CD15 (62%), BCL6 (47%), BCL2 (29%), and EBER (25%), in a polymorphous inflammatory background typical of CHL. The FL component was low-grade in 55%, grade 3A in 36%, and grade 3B in 9% of cases. All 3 cases investigated by cytogenetic methods for a clonal relationship between the CHL and FL components were clonally related. These clinicopathologic features of our cohort are similar to those of cases reported in the literature. The 5-year overall survival in combined patients with CHLFL (n = 49) was 48%, comparable to CHL but worse than FL in the elderly. In summary, CHLFL is a rare entity that most often occurs in older adults, involves lymph nodes, and most commonly presents de novo. In the small number of cases assessed, the CHL and FL components are usually clonally related suggesting that the CHL and FL components may share a common progenitor B-cell, likely a mutated germinal center B-cell.

Case report: Anterior mediastinal mass in a patient with pleural effusion and dyspnea

“…Cutting-edge research suggests that diverse mechanisms may underlie the coexistence of two lineages in a single patient. For example, a possible reason is a bidirectional differentiation after malignant transformation at the hematopoietic stem or progenitor cell level ( 8 , 9 ). It has been well documented since the early 1980s that these pluripotent cells with the capacity to differentiate into a B- or T-cell lineage underwent TCR and IGH gene rearrangements.…”

Histologic transformation of follicular lymphoma: pathologists’ viewpoint

“… 24 Majority of CHL cases are a mixed cellularity type, and minority of the cases are a nodular sclerosis type. 15 , 42 EBER1 is positive in 25% of CHL cases. 42 An FL component is sometimes detected in the bone marrow or duodenum during the staging procedure of CHL.…”

“… 15 , 42 EBER1 is positive in 25% of CHL cases. 42 An FL component is sometimes detected in the bone marrow or duodenum during the staging procedure of CHL. Using fluorescence in situ hybridization (FISH) analysis or Fluorescence Immunophenotyping and Interphase Cytogenetics as a Toll for the Investigation Of Neoplasm, namely, combination of FISH and immunohistochemistry for CD30, 43 the composite lymphomas of CHL and FL are cytogenetically related.…”

“…Using fluorescence in situ hybridization (FISH) analysis or Fluorescence Immunophenotyping and Interphase Cytogenetics as a Toll for the Investigation Of Neoplasm, namely, combination of FISH and immunohistochemistry for CD30, 43 the composite lymphomas of CHL and FL are cytogenetically related. 24 , 42 , 44 …”

Patients with Classic Hodgkin Lymphoma and Follicular Lymphoma Compared to Single Malignancy Controls

“…[13][14][15] The association between FL and CHL is not as thoroughly studied as that between CHL and other low-grade lymphomas. There is a paucity of well-documented cases, and only 2 studies include a relevant number of cases: 1 in which the 2 neoplasms were diagnosed simultaneously 16 and 1 with a mixture of simultaneous and sequential lesions. 17 Regardless of the temporal relationship between FL and CHL, there is a clonal relationship between FL and Reed-Sternberg (RS) cells, the neoplastic component of CHL.…”