Abstract
The distinction between chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with isolated Hodgkin/Reed–Sternberg cells (CLL-HRS; background milieu with a paucity of inflammatory cells) and overt transformation to classic Hodgkin lymphoma (CLL-HL; mixed inflammatory background) is incompletely understood. This retrospective study examined the clinicopathologic features of CLL-HRS (n = 15) and CLL-HL (n = 31) patients seen over the past three decades from a single institution. The phenotypic features of Reed–Sternberg cells in both groups were similar, including expression of CD30, CD15, and PAX5, as well as EBV status. However, a spectrum of background CLL/SLL infiltration amongst the HRS cells was noted on pathologic review, and four patients had both diagnoses, either concurrently or in succession. The median overall survival (OS) of patients with CLL-HRS was 17.5 months compared to 33.5 months for patients with CLL-HL (P = 0.24). Among patients with CLL-HRS, those who received Hodgkin-directed therapy had a significantly longer median OS (57 months) compared to those who received CLL-directed therapy (8.4 months, P = 0.02). Our clinical and pathologic findings suggest a biologic continuum between CLL-HRS and CLL-HL and indicate that CLL-HRS patients may benefit from Hodgkin-directed therapy.
Highlights
Progression of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) to classic Hodgkin lymphoma (CHL) as a form of Richter transformation is an uncommon but well-documented event and occurs in >1% of CLL/SLL patients [1]
The World Health Organization (WHO) 2017 classification of lymphoid neoplasms emphasizes that the distinction between overt CHL transformation (CLL-HL) and CLL-HRS lies in the presence or absence of the mixed inflammatory background [8]
Patient cohort A total of 51 patients with CLL/SLL and either CLL-HRS or CLL-HL were identified, of whom five patients were excluded
Summary
Progression of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) to classic Hodgkin lymphoma (CHL) as a form of Richter transformation is an uncommon but well-documented event and occurs in >1% of CLL/SLL patients [1]. In spite of the pathologic emphasis on distinguishing the two, whether CLL-HRS clinically represents a spectrum of low-grade CLL/SLL, a transformation event similar to CLL-HL, or something in between, remains an unanswered question. The goal of this retrospective study was to compare the follows: CLL-HRS (eight excisional, three core needles, four bone clinicopathologic features of a cohort of CLL-HRS and CLL-HL marrow); CLL-HL (ten excisional, nine core needles, ten bone patients with an emphasis on comparing the clinical outcomes of marrow); CLL-RS and CLL-HL (one excisional one core needle). The median time from CLL to CLL-HL transformation was 6.6 years and CLL to
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