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Hodgkin Lymphoma
Definition
A monoclonal B-cell neoplasm
characterized by a nodular pattern,
with or without diffuse areas, in
which large neoplastic lymphocytic
and/or histiocytic (L&H) cells are
present against a background of
numerous lymphocytes and
histiocytes, and follicular dendritic
cells in nodular areas
Epidemiology
5% of all cases of Hodgkin
Male > Female
Can occur at all age; peak at 30-50
years old
Pathogenesis
Traditionally has been included in the
classification of HL
L&H cells
B-cell origin; functional B-cell differentiation
program
immunophenotype are dissimilar from H/R-S cells
strongly express a variety of panB-cell antigens
and transcription factors or cofactors, as well as
germinal center B-cellassociated antigens and
leukocyte common antigen (LCA; CD45).
almost always negative for CD15 and CD30
Pathogenesis
L&H cells
Progressive transformation of germinal
centers (PTGC) as precursor lesion
Clinical features
Localized; lymph node enlargement
frequently perpheral (cervical, axillary,
inguinal)
Clinically indolent; may be treated with
involved-field radiotherapy without
chemotherapy
Unfavorable prognostic features or
advanced stage treated as cHL (ABVD)
Excellent overall survival, but relapses
frequent
Histologic features
large, expansile nodules;
partially/totally replace the lymph
node architecture; displace and
compress uninvolved lymphoid
tissue.
Histologic features
Nodules vary in size; larger, vaguer
outlines than reactive lymphoid
follicles
Histologic features
nodules are typically arranged
closely, often in back-to-back fashion
composed of:
numerous, small, round lympho-cytes;
typical and epithelioid histiocytes;
follicular dendritic cells; and
neoplastic L&H cells
Histologic features
L&H cells:
1% of all cells in the nodules
Large cells, scant pale cytoplasm
Nuclei: multilobated or folded, has
vesicular nucleoplasm, inconspicuous
nucleolus, thin nuclear membrane
popcorn cells
Mitoses are uncommon
Histologic features
Reactive germinal centers are
unusual
Epithelioid histiocytes are present
(single or in small clusters)
Fibrosis is usually absent
Eosinophils, neutrophils, and plasma
cells are not a part of NLPHL.
Histologic variants
Classic nodular
Serpiginous/interconnected nodular
Irregular outlines, fuse with other nodules
Histologic variants
The T-cellrich B-cell lymphoma-like
diffuse pattern
composed of scattered large L&H cells
against a background of numerous T cells
and histiocytes
Immunophenotypic findings
Positive for panB-cell markers
CD20, CD22, CD75, and CD79a