title: The mystery of chronic lymphocytic leukemia (CLL): Why is it absent in Asians and what does this tell us about etiology, pathogenesis and biology?
abstract: Chronic lymphocytic leukemia/small lymphocytic lymphoma is common in persons of predominately European descent but rare in Asians. Why is unknown but is likely genetically-determined. Environmental factors may also operate but are likely to be less important. When CLL occurs in Asians it has different features than CLL in persons of predominately European descent. The reason(s) for this is also not understood. We reviewed data on CLL in Asians (mostly Han Chinese but also other ethnic groups) and compared these data with those from persons of predominately European descent with CLL. CLL incidence was about 5-10-fold less in Asians. Asians with CLL are younger, have atypical morphologic and immunologic features, an increased proportion of IGHV mutations and rearrangements and briefer freedom-from-progression than persons of predominately European descent with CLL. These observations provide clues to the etiology and biology of CLL. But the mystery continues; more research is needed.
authors.affiliation: Peoples' Hospital of Peking University, Peking University Institute of Hematology, Beijing 100044, China.
authors.forename: Shen-Miao
authors.initials: SM
authors.lastname: Yang
authors.affiliation: Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
authors.forename: Jian-Yong
authors.initials: JY
authors.lastname: Li
authors.affiliation: Haematology Research Center, Division of Experimental Medicine, Department of Medicine, Imperial College London, London, UK. Electronic address: robertpetergale@gmail.com.
authors.forename: Robert Peter
authors.initials: RP
authors.lastname: Gale
authors.affiliation: Peoples' Hospital of Peking University, Peking University Institute of Hematology, Beijing 100044, China; Peking-Tsinghua Center for Life Sciences, Beijing 100871, China. Electronic address: xjhrm@medmail.com.cn.
authors.forename: Xiao-Jun
authors.initials: XJ
authors.lastname: Huang
mesh_terms: D000367:Age Factors; D044466:Asian People / Q000235:genetics; D002869:Chromosome Aberrations; D004777:Environment; D020022:Genetic Predisposition to Disease; D006680:HLA Antigens / Q000235:genetics / Q000276:immunology; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D007135:Immunoglobulin Variable Region / Q000235:genetics; D016130:Immunophenotyping; D015994:Incidence; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D009154:Mutation; D010641:Phenotype; D011379:Prognosis; D012307:Risk Factors; D044465:White People / Q000235:genetics
chemical_list: D006680:HLA Antigens; D007143:Immunoglobulin Heavy Chains; D007135:Immunoglobulin Variable Region
keywords: Asians; CLL; Chronic lymphocytic leukemia; IGHV; Small lymphocytic lymphoma