Clonality analysis and mutational status of IgVH gene in Hodgkin variant of Richter syndrome / 中华病理学杂志
Chinese Journal of Pathology
;
(12): 523-528, 2008.
Article
in Chinese
| WPRIM
| ID: wpr-315135
ABSTRACT
<p><b>OBJECTIVE</b>To detect the clonal relationship, the rearrangement, and the mutational status of IgVH gene; the influence of these molecular characteristics on the clinical outcome in Hodgkin variant of Richter syndrome; and the possible molecular pathogenesis in this transformation.</p><p><b>METHODS</b>The clonal rearrangements and mutational status of IgVH genes were analyzed in Hodgkin variant of Richter syndrome and B-CLL with Reed-Stemberg (R-S)-like cells by GeneScan analysis and sequencing. Semi-nest PCR based on laser capture microdissection was utilized to compare the clonal relationship between B-CLL and R-S/R-Slike cells. Immunohistochemical staining was used to detect the different expressions of ZAP70, p53, IRF-4 and LMP1 in the two components.</p><p><b>RESULTS</b>(1) 5/6 B-CLL cases transformed to Hodgkin lymphoma (HL)/R-S-like cells carried the mutated IgVH genes; (2) 2 cases of R-S cells and 1 case of R-S-like cells were clonally distinct from B-CLL clone and express LMP1, whereas 1 case of R-S-like cells was relating to the surrounding B-CLL cells and did not express LMP1; (3) 2/6 B-CLL cases transformed to HL convey VH4-34 and VH3-48 respectively.</p><p><b>CONCLUSIONS</b>(1) Richter transformation to HL/R-S-like cells evolves from the B-CLL which originates from the germinal center or post germinal center B cells, indicating that different lymphoma cells of different subtypes in Richter syndrome come from different B cell lineage and possibly involve a different pathogenesis and pathway; (2) HL and R-S-like cells evolve from either the B-CLL clone or may develop as a clonally unrelated lymphoma, the independent secondary malignancies are appear to be EBV-positive, possibly as a consequence of the underlying immunodeficiency; (3) The biased usage of IgVH genes suggested a role of antigens involved in the HL variant of Richter syndrome.</p>
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Syndrome
/
Virology
/
Immunoglobulin Variable Region
/
Hodgkin Disease
/
Leukemia, Lymphocytic, Chronic, B-Cell
/
Reed-Sternberg Cells
/
Classification
/
Clone Cells
/
Herpesvirus 4, Human
Limits:
Aged
/
Aged80
/
Female
/
Humans
/
Male
Language:
Chinese
Journal:
Chinese Journal of Pathology
Year:
2008
Type:
Article
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