There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.
Abstract
Hodgkin/Reed-Sternberg (HRS) cells in the setting of chronic lymphocytic leukemia
(CLL) exist in 2 forms: type I with isolated HRS cells in a CLL background (Hodgkin-like
lesion) and type II with typical classic Hodgkin lymphoma, a variant of Richter transformation
(CHL-RT). The clinical significance of the 2 morphological patterns is unclear, and
their biological features have not been compared. We retrospectively reviewed 77 cases:
26 of type I and 51 of type II CHL-RT; 3 cases progressed from type I to type II.
We examined clinical features, Epstein-Barr virus (EBV) status, and clonal relatedness
after microdissection. Median age for type I was 62 years versus 73 years for type
II (P=.01); 27% (type I) versus 73% (type II) had a history of CLL. HRS cells were
positive for EBV in 71% (55/77), similar in types I and II. Clonality analysis was
performed in 33 cases (type I and type II combined): HRS cells were clonally related
to the underlying CLL in 14 and unrelated in 19. ZAP-70 expression of the CLL cells
but not EBV status or morphological pattern was correlated with clonal relatedness:
all 14 clonally related cases were ZAP-70 negative, whereas 74% (14/19) of clonally
unrelated cases were ZAP-70 positive. Overall median survival (types I and II) after
diagnosis was 44 months. Advanced age was an adverse risk factor for survival, but
not histologic pattern, type I versus type II. HRS-like cells in a background of CLL
carries a similar clinical risk to that of CHL-RT and may progress to classic Hodgkin
lymphoma in some cases.