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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Hum+Pathol
2016 ; 55
(ä): 108-116
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English Wikipedia
Hodgkin lymphoma variant of Richter transformation: morphology, Epstein-Barr
virus status, clonality, and survival analysis-with comparison to Hodgkin-like
lesion
#MMPMID27184478
Xiao W
; Chen WW
; Sorbara L
; Davies-Hill T
; Pittaluga S
; Raffeld M
; Jaffe ES
Hum Pathol
2016[Sep]; 55
(ä): 108-116
PMID27184478
show ga
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.