Classical Hodgkin lymphoma
Classical Hodgkin lymphoma (CHL) is a monoclonal lymphoid neoplasm probably derived from germinal center B cells. It is histologically characterized by mononuclear Hodgkin and multinucleated Reed-Sternberg (HRS) cells in a variable infiltrate of non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts and collagen
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
International Agency for Research on Cancer: Lyon 2008
CHL accounts for 95% od all Hodgkin lymphomas and has age peaks at 15-35 years and in late life. It involves cervical lymph nodes in approximately 75% of cases and patients typically present with localized disease (stages I and II). Primary extranodal involvement is rare. Current therapy for Hodgkin lymphoma has resulted in an excellent outcome and cure for up to 90% of patients.
RHS cells carry clonal immunoglobin (IG) gene rearrangements and show frequent infection with Epstein-Barr virus (EBV). CHL is a hallmark of X-linked lymphoproliferative disease (XLP)
, chracterized by extreme sensitivity to EBV infection.
> Classical Hodgkin lymphoma variants
Nodular sclerosis classical Hodgkin lymphoma
Lymphocyte-rich classical Hodgkin lymphoma
Mixed cellularity classical Hodgkin lymphoma
Lymphocyte-depleted classical Hodgkin lymphoma
CHL may involve the gastrointestinal tract, but this is usually secondary to nodal disease. Primary gastric classical Hodgkin lymphoma is extremely rare
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, and should be differentiated from EBV-positive DLBCL of the elderly
[A rare stomach tumor--Hodgkin's lymphoma of the stomach].
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International Agency for Research on Cancer: Lyon 2010