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Hodgkin's lymphoma

Definition
HL is a rare lymphatic neoplasm, typically of B-cell origin, characterized by large malignant lymphoid cells, and Reed-Sternberg cells.
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Pathophysiology
HL is most frequently caused by the EBV. Other causes include tuberculosis and genetic factors (HLA class II polymorphisms).
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Epidemiology
HL incidence in the US is estimated at 3.23 per 100,000 people per year.
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Disease course
Clinical manifestations include painless lymphadenopathy enlarging over months, mainly involving the mediastinum, left neck node, right neck node, splenic, axillary, abdominal, hilar, or inguinofemoral nodes in descending order. The clinically occult disease is characterized by severe unremitting pruritus without obvious skin pathology. B symptoms include fever, night sweats, and unexplained weight loss > 10% of body weight. Alcohol-induced lymph node pain is highly specific for HL.
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Prognosis and risk of recurrence
Prognosis is determined by disease stage (i.e. best prognosis in early-stage HL) and pathology (i.e. better prognosis in Nodular sclerosis cHL).
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Key sources
The following summarized guidelines for the evaluation and management of Hodgkin's lymphoma are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2022), the American Society of Blood and Marrow Transplantation (ASBMT/EBMT/CIBMTR 2019), the European Society of Medical Oncology (ESMO 2018), and the British Committee for Standards In Haematology (BCSH/BSH 2015).
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Guidelines

1.Screening and diagnosis

Diagnosis
Diagnose classical HL based on the presence of Hodgkin and Reed-Sternberg cells.
Diagnose nodular lymphocyte-predominant HL based on the presence of lymphocyte-predominant cells.
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2.Classification and risk stratification

Staging: use the Ann Arbor classification of staging HL. Allocate patients to one of three categories (limited, intermediate, and advanced stages).
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  • Risk classification

3.Diagnostic investigations

Imaging for staging, classical Hodgkin's lymphoma, BSH: obtain PET for staging in patients with Hodkin's lymphoma.
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  • Imaging for staging (nodular lymphocyte-predominant HL)

  • Routine laboratory tests (classical HL)

  • Routine laboratory tests (nodular lymphocyte-predominant HL)

  • Screening for viral infections

  • Cardiac and pulmonary function tests

4.Diagnostic procedures

Bone marrow biopsy, classical Hodgkin's lymphoma, BSH: do not perform routine bone marrow biopsy in patients with HL.
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  • Bone marrow biopsy (nodular lymphocyte-predominant HL)

5.Medical management

General principles
Treat patients with HL within clinical trial protocols whenever possible.
Decide on the intensity of treatment depending on the patient's risk profile at diagnosis and the result of interim PET-CT evaluation (if available).

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  • Management of early-stage classic HL

  • Management of advanced-stage classic HL

  • Management of nodular lymphocyte-predominant HL (early-stage disease)

  • Management of nodular lymphocyte-predominant HL (advanced-stage disease)

  • Management of relapsed/refractory disease (classical HL, chemotherapy and autologous HSCT)

  • Management of relapsed/refractory disease (classical HL, post-HSCT consolidation)

  • Management of relapsed/refractory disease (nodular lymphocyte-predominant HL)

6.Therapeutic procedures

Technical considerations for radiotherapy: consider deciding on offering or omitting radiotherapy in early and advanced-stage HL based on the individual risk profile and acceptable balance between toxicity and efficacy.
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7.Specific circumstances

Adolescent and young patients, classical Hodgkin's lymphoma: consider using pediatric or adult protocols in adolescent and young adult patients with HL, depending on center expertise and the patient's opinion on the toxicity/efficacy balance between regimens.
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  • Adolescent and young patients (nodular lymphocyte-predominant HL)

  • Pregnant patients

  • Elderly patients

8.Patient education

Fertility counseling
As per BSH 2022 guidelines:
Offer fertility preservation/counseling where appropriate in patients with HL.
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Offer follow-up discussions about the impact of treatment on the fertility and/or menopausal status and referral for specialist advice where appropriate.
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9.Follow-up and surveillance

Assessment of treatment response, classical Hodgkin's lymphoma: report PET response according to DS and metabolic response categories. View DS 1-3 as a complete metabolic response.
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  • Assessment of treatment response

  • Assessment of treatment response (nodular lymphocyte-predominant HL)

  • Long-term survivorship care

  • Surveillance for other malignancies