PMID- 23328508
OWN - NLM
STAT- MEDLINE
DCOM- 20131021
LR  - 20130118
IS  - 0376-2491 (Print)
IS  - 0376-2491 (Linking)
VI  - 92
IP  - 46
DP  - 2012 Dec 11
TI  - [Richter syndrome: clinical characteristics and treatment experiences].
PG  - 3254-6
AB  - OBJECTIVE: To summarize the clinical characteristics of Richter syndrome and 
      explore the methods of successful treatment and timely diagnosis. METHODS: Five 
      patients with Richter syndrome in the last three years (from January 2009 to 
      December 2011) were analyzed retrospectively at our hospital, including their 
      clinical features and therapy before and after transformation. RESULTS: There 
      were 4 males and 1 female with a median age on a diagnosis of chronic lymphocytic 
      leukemia (CLL) at 47 (44 - 68) years. The median duration from a diagnosis of CLL 
      to transformation was 52 (5 - 90) months. As for cytogenetic abnormalities, 3/4 
      patients had 17p deletion by fluorescence in situ hybridization (FISH). The 
      clinical manifestations on transformation included regional enlargement of lymph 
      node (n = 2) and systemic enlargement of lymph nodes (n = 3). All diagnoses were 
      confirmed by lymph node biopsy and all transformed into diffuse large B cell 
      lymphoma (classical transformation). The subgroups were germinal center B-cell 
      like (GCB) (n = 3) and non-GCB (n = 1). After transformation, one patient 
      underwent sibling allo-stem cell transplantation and survived 24 months until 
      April 2012. Another patient with auto-stem cell transplantation relapsed and died 
      12 months later. One patient lost the treatment opportunity due to worsening 
      condition. Another 2 patients gained partial remission after therapy and survived 
      20 and 8 months respectively. CONCLUSIONS: Richter syndrome may occur during a 
      late stage of CLL. Such a high-risk cytogenetic abnormality as del17p may be 
      correlated with transformation. Early identification and optimal therapy may 
      extend the survival of Richter syndrome. Allo-stem cell transplantation remains a 
      curable option.
FAU - Li, Zeng-jun
AU  - Li ZJ
AD  - Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical 
      Sciences & Peking Union Medical College, Tianjin 300020, China.
FAU - Xu, Yan
AU  - Xu Y
FAU - Zhang, Pei-hong
AU  - Zhang PH
FAU - Liu, Wei
AU  - Liu W
FAU - Yi, Shu-hua
AU  - Yi SH
FAU - Zou, De-hui
AU  - Zou DH
FAU - Qi, Jun-yuan
AU  - Qi JY
FAU - Zhao, Yao-zhong
AU  - Zhao YZ
FAU - Qiu, Lu-gui
AU  - Qiu LG
LA  - chi
PT  - English Abstract
PT  - Journal Article
PT  - Research Support, U.S. Gov't, Non-P.H.S.
PL  - China
TA  - Zhonghua Yi Xue Za Zhi
JT  - Zhonghua yi xue za zhi
JID - 7511141
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Humans
MH  - Leukemia, Lymphocytic, Chronic, B-Cell/*diagnosis/*therapy
MH  - Lymphoma, Large B-Cell, Diffuse/*diagnosis/*therapy
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Retrospective Studies
MH  - Syndrome
EDAT- 2013/01/19 06:00
MHDA- 2013/10/22 06:00
CRDT- 2013/01/19 06:00
PHST- 2013/01/19 06:00 [entrez]
PHST- 2013/01/19 06:00 [pubmed]
PHST- 2013/10/22 06:00 [medline]
PST - ppublish
SO  - Zhonghua Yi Xue Za Zhi. 2012 Dec 11;92(46):3254-6.