Classical Hodgkin's disease

Epidemiology

Classical HL accounts for 95% of all cases of Hodgkin lymphoma. There is a bimodal distribution with age peaks at 15-35 years and in the elderly.

Clinical features

Sites of involvement are commonly cervical (75% of cases), mediastinal (60% of cases, mainly nodular sclerosis HL), axillary and para-aortic. 55% of cases present with stage I or II disease. 20% have splenic involvement and 5% bone marrow involvement. 40% of patients have systemic symptoms.

Histopathology

Classification should be based on pre-treatment biopsy material. Treatment leads to a histological appearance of residual or recurrent disease with numerous atypical Hodgkin and RS cells in a lymphocyte-depleted background.

Variants:

NS-CHL: cellular phase. There is clear-cut nodularity without overt collagen band deposition. Lacunar cells are present, often at the periphery of nodules. The lymphocytes are mainly of mantle cell type (CD20+, CD79a+, CD5+, IgM+, IgD+, CD3-)2.
NS-CHL: syncytial. When lacunar cells are highly aggregated, the term syncytial variant has been used. It constitutes 16% of all NS-CHL and runs a more aggressive course, with bulky mediastinal disease and stage III/IV in 88% of patients2.
Grading:

grade 2 constitutes 15-25% of all NS-CHL. EBV-encoded LMP1 is seen in only 10-40% of cases.

Subtypes:

HL, unclassifiable. Cases with ambiguous features or insufficient material should be placed in this category, rather than categorised as MC CHL as was past practice.

Immunohistochemistry

CD3

CD15

CD20

CD30

CD45

CD79a

BSAP

EBV encoded LMP1

EMA

ALK

 

var

~80% of cases2

~20% of cases

98% of cases2

usually negative

sometimes

~90% of cases

positive

<5% of cases

negative

 

Differential diagnosis: see table of differential diagnoses and immunohistochemistry

Prognosis

References

World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

1Jones D. Dismantling the germinal center: comparing the processes of transformation, regression and fragmentation of the lymphoid follicle. Advances in Anatomic Pathology 2002;9:129-138.

2Pileri, S.A., Ascani, S., Leoncini, L., Sabattini, E., Zinzani, P.L., Piccaluga, P.P., Pileri, A., Jr., Giunti, M., Falini, B., Bolis, G.B. and Stein, H. Hodgkin's lymphoma: the pathologist's viewpoint. J Clin Pathol 2002;55:162-76.

This page last revised 3.12.2002.

©SMUHT/PW Bishop