Hodgkin Lymphoma Diagnosis and Treatment

Introduction to Hodgkin Lymphoma

The National Comprehensive Cancer Network recommends that Hodgkin lymphoma (HL) is a highly curable malignancy involving lymph nodes and the lymphatic system, characterized by Reed-Sternberg cells in classical Hodgkin lymphoma (CHL) or lymphocyte-predominant cells in nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) 1, 2

The World Health Organization classifies HL into two main types: Classical Hodgkin Lymphoma (CHL) and Nodular Lymphocyte-Predominant Hodgkin Lymphoma (NLPHL), with CHL accounting for approximately 95% of HL cases diagnosed in Western countries 1, 3
CHL is characterized by Reed-Sternberg cells in an inflammatory background, and is further subdivided into four subtypes: nodular sclerosing, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted 3, 4
NLPHL lacks Reed-Sternberg cells but contains lymphocyte-predominant cells ("popcorn cells") with a different immunophenotype (CD15-/CD30-/CD20+) compared to CHL (CD15+/CD30+/CD20-) 3, 4

HL has a bimodal age distribution with peaks at 15-30 years and after age 55, and most patients initially present with enlarged cervical lymph nodes 1, 3
B symptoms (fever, night sweats, weight loss) are important prognostic factors, and the disease is now curable in at least 80% of patients with modern treatment approaches 1, 3

Diagnosis always requires a lymph node biopsy to identify Reed-Sternberg cells or lymphocyte-predominant cells, and immunophenotyping is essential to distinguish between CHL and NLPHL 3, 4

Staging is based on the Ann Arbor staging system with Cotswolds modification, and required investigations include CT scans, bone marrow aspiration and biopsy, PET/CT, and blood tests 1, 3, 5, 6
Patients are classified into three risk groups: early-stage favorable, early-stage unfavorable, and advanced-stage disease 1, 3

The National Comprehensive Cancer Network recommends standard treatment for early-stage favorable disease as combined modality therapy with 2 cycles of ABVD followed by 30 Gy involved-field radiotherapy (IF-RT) 3, 4
For early-stage unfavorable disease, standard treatment is 4 cycles of ABVD followed by 30 Gy IF-RT, and for patients under 60 years eligible for more intensive treatment, 2 cycles of BEACOPPescalated followed by 2 cycles of ABVD and 30 Gy IF-RT may provide superior freedom from treatment failure 5, 6
For advanced-stage disease, treatment options include 6-8 cycles of ABVD with radiotherapy limited to residual masses, or 8 cycles of BEACOPPescalated for patients under 60 years, followed by radiation to residual disease >1.5 cm 5, 7
PET/CT is used to assess treatment response using the Deauville criteria, and patients older than 60 should receive 6-8 cycles of ABVD rather than BEACOPP due to increased toxicity in this age group 1, 7

High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) is the standard of care for relapsed or refractory disease, and salvage regimens such as DHAP, IGEV, or ICE are used to reduce tumor burden and mobilize stem cells prior to ASCT 7
Brentuximab vedotin, a CD30-directed antibody-drug conjugate, has shown encouraging results in relapsed/refractory disease 1

PET/CT is essential for response assessment using the Deauville criteria, and long-term follow-up is crucial due to potential late effects of treatment 1, 5
Fertility preservation should be offered to young patients prior to treatment, and treatment selection must balance efficacy with potential long-term toxicity 1, 5, 6