Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Hodgkin Lymphoma (HL)
Definition and Incidence
The crude incidence of Hodgkin lymphoma in the European
Union is 2.2/100,000/year and mortality is 0.7/100,000/year. HLs
account for approximately 15% of lymphomas and, in contrast to
non-Hodgkin lymphomas, their absolute incidence has not
increased in recent decades.
The goal of HL therapy is the “least complicated cure” with a
personalised approach. The associated risks of treatment,
including sterility and the risk of second malignancy, have
influenced the current management of early stage non-bulky HL
towards brief chemotherapy followed by involved field irradiation
with more chemotherapy and less irradiation in those with higherstaged
illness. ABVD remains the international standard cytotoxic
chemotherapy. The role of BEACOPP in advanced disease is
under investigation with a view to optimising frontline treatment
to avoid the need for salvage therapy and transplant. F 18 DG
PET/CT scan is a now well established in staging, response
assessment and evaluation of residual masses.
ICD – O Codes:
Nodular lymphocyte predominant
Hodgkin lymphoma NLPHL 9659/3
Classical Hodgkin lymphoma CHL 9650/3
■ Nodular sclerosis classical
Hodgkin lymphoma NSHL 9663/3
■ Mixed cellularity classical
Hodgkin lymphoma MCHL 9652/3
■ Lymphocyte-rich classical
Hodgkin lymphoma LRCHL 9651/3
■ Lymphocyte-depleted classical
Hodgkin lymphoma LDHL 9653/3
Clinical Presentation:
Classical Hodgkin lymphoma (CHL) accounts for 95% of HL
with a bimodal age curve showing a peak at 15-35 years and a
second peak in later life. Patients with a history of infectious
mononucleosis have a higher incidence of HL and both familial
and geographic clustering has been described. Patients usually
present with peripheral lymphadenopathy at one or two sites.
Mediastinal involvement is most frequently seen with Nodular
Sclerosis Hodgkin lymphoma (NSHL) while abdominal and
splenic involvement is more common with Mixed Cellularity
Hodgkin lymphoma (MCHL). About 40% will have systemic
symptoms consisting of fever, drenching night sweats and
significant weight loss.
Patients with Nodular Lymphocyte Predominant Hodgkin
lymphoma (NLPHL) usually present with localized peripheral
lymphadenopathy (stage I or II). Between 5% and 20% of
patients present with advanced stage disease. NLPHL is an
indolent disease and previous lymph node biopsies may have
been interpreted as representing a reactive process. Relapses are
frequent but the disease usually remains responsive to treatment
and is thus rarely fatal. Transformation to diffuse large B-cell
lymphoma (DLBCL) may rarely occur.
Pathology and Genetics
Pathologic diagnosis should be made according to the
WHO classification as described above.
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