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10.1016/j.humpath.2016.04.019

http://scihub22266oqcxt.onion/10.1016/j.humpath.2016.04.019
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suck abstract from ncbi


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pmid27184478
      Hum+Pathol 2016 ; 55 (ä): 108-116
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  • 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.
  • |*Cell Transformation, Viral [MESH]
  • |*Clone Cells/pathology/virology [MESH]
  • |*Epstein-Barr Virus Infections/genetics/mortality/pathology/virology [MESH]
  • |*Hodgkin Disease/genetics/mortality/pathology/virology [MESH]
  • |*Leukemia, Lymphocytic, Chronic, B-Cell/genetics/mortality/pathology/virology [MESH]
  • |*Reed-Sternberg Cells/pathology/virology [MESH]
  • |Adult [MESH]
  • |Aged [MESH]
  • |Aged, 80 and over [MESH]
  • |Databases, Factual [MESH]
  • |Disease Progression [MESH]
  • |Female [MESH]
  • |Gene Rearrangement [MESH]
  • |Genes, Immunoglobulin Heavy Chain [MESH]
  • |Genetic Predisposition to Disease [MESH]
  • |Herpesvirus 4, Human/genetics/*isolation & purification [MESH]
  • |Humans [MESH]
  • |Immunohistochemistry [MESH]
  • |In Situ Hybridization [MESH]
  • |Kaplan-Meier Estimate [MESH]
  • |Male [MESH]
  • |Middle Aged [MESH]
  • |Phenotype [MESH]
  • |Polymerase Chain Reaction [MESH]
  • |Proportional Hazards Models [MESH]
  • |RNA, Viral/genetics [MESH]
  • |Retrospective Studies [MESH]


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