Hodgkin Lymphoma

Epidemiology and Etiology

The incidence of Hodgkin lymphoma (HL) follows a bimodal distribution with a peak at age 25 and second peak at age 50 years, with an estimated 8480 new cases in the US in 2020.1 EBV and HIV infections, autoimmune conditions, and immunosuppressant use have been described as risk factors for HL.

Pathology

HL is subdivided into nodular lymphocyte predominant (NLPHL) and classical HL subtypes based on the pathologic appearance. The Reed–Sternberg (RS) cells consistently express CD30 and CD15. In contrast to the other histologic subtypes, RS cells are infrequent in NLPHL. Instead, “popcorn cells” are seen within a background of inflammatory cells in NLPHL.

Clinical Presentation

Most patients present with painless lymphadenopathy. Presence of B symptoms including fevers, significant weight loss, and drenching night sweats are more common in advanced stages.

Diagnostic Testing

FNA is often inadequate to make a diagnosis and therefore a minimum of a core-needle biopsy is necessary. If initial biopsy is nondiagnostic, excisional biopsy may be required. Additional workup includes routine labs, LDH, erythrocyte sedimentation rate (ESR), CT, FDG PET CT, and bone marrow examination.

Staging

The traditional Ann Arbor staging system has since been replaced by the Lugano classification (Table 22-9). Patients with early-stage disease (stages I–II) may be further stratified into favorable- and unfavorable-risk categories. Favorable risk is defined by the presence of ≤2 sites of disease, mediastinal width less than one-third of maximal thoracic diameter, ESR <50 mm/h (<30 mm/h with B symptoms), and absence of extranodal extension.2

Table 22-9: Lugano Staging of Lymphomasa
StageDescription
IInvolvement of a single lymph node region (I) or single extralymphatic organ (IE).
IIInvolvement of ≥2 lymph node regions in the same side of the diaphragm.
IIIInvolvement of lymph node regions in both sides of the diaphragm.
IVDiffuse or disseminated involvement of one or more extralymphatic organs, including any involvement of the CSF, bone marrow, liver, or multiple lung lesions (unless considered stage IIE).

aModifying features: HL, A: absence of B features; B: presence of B features. NHL no longer uses A and B modifiers. Both HL and NHL, E, involvement of a single extranodal site contiguous or proximal to the involved nodal site; S, spleen involvement; X, bulky disease definition varies by histology (HL: lymph node ≥10 cm or than ≥one-third of thoracic diameter; FL: ≥6 cm; DLBCL: ≥10 cm).

Treatment

  • The treatment of choice for early stage HL (stages I–II) is chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) for 2–4 cycles, with or without involved field radiotherapy (IFRT), depending on favorable versus unfavorable risk and interim PET response (Deauville 5-point scale).
  • The treatment of choice for advanced stage HL (stages III-IV, as well as stage II with bulky nodal disease) is chemotherapy with ABVD, BV+AVD (brentuximab vedotin. a CD30-directed ADC, plus doxorubicin, vinblastine, and dacarbazine), or escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone). BV+AVD and BEACOPP have been associated with improved PFS, but not OS, compared to ABVD.
  • Relapsed disease is treated with salvage chemotherapy with or without stem cell transplantation. The PD-1 inhibitors pembrolizumab or nivolumab are effective treatments for relapsed HL.2

References

  1. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2020. CA Cancer J Clin. 2020;70(1):7-30.  [PMID:31912902]
  2. Connors JM, Cozen W, Steidl C, et al. Hodgkin lymphoma. Nat Rev Dis Primers. 2020;6(1):61.  [PMID:32703953]

Outline


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