Fact-sheet: Hodgkin's disease - lymphoma


Updated on 08/26/2020 at 2:15 PM

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Definition

Tumoral proliferation of lymphoid cells in one or more lymphoid organs, occasionally extending to extralymphatic sites.

Most common cancer in adolescents.
Peak incidence between 15 and 30 years.

Risk factors:
EBV infection, immune deficiencies, and familial forms.

Histologic type:
This is a low-grade B-cell lymphoma with an indolent course.
It is characterized by the presence of Reed-Sternberg cells (CD30+, CD15+, PAX5+, CD45-, CD20 +/-, ALK-).

The main location is cervicothoracic.

Clinical features

  • Sometimes symptomatic due to the indolent course
  • Superior vena cava syndrome
  • Dyspnea

Radiography

  • Mediastinal widening
  • "Chimney" appearance

Ultrasound

Complementary, to look for splenic involvement.

  • Single or multiple hypoechoic nodules
  • Inconsistent splenomegaly

CT

CTAP protocol

  • Mediastinal mass
  • Necrotic lymphadenopathy

Evaluation of nodal areas, thymus, spleen, bone marrow.
Determination of the best biopsy site.

MRI

  • Helpful for thymic, osseous, and CNS involvement.

Nuclear medicine

18F-FDG PET:

  • Initial staging workup.
  • Allows assessment of early response after 2 cycles (Deauville score <3) (see classification below)

Management

  • Chemotherapy and corticosteroid therapy.
    Radiotherapy is increasingly less used
    (survival > 95%)

Classification

Ann Arbor classification:

  • Stage I: involvement of a single nodal region (or spleen) above or below the diaphragm
  • Stage II: involvement of several nodal regions on the same side of the diaphragm (II) or of a single nodal region with contiguous extralymphatic extension (IIe)
  • Stage III: involvement on both sides of the diaphragm: spleen (IIIs) and/or limited to a contiguous extralymphatic region or organ (IIIe, IIIes)
  • Stage IV: non-contiguous visceral involvement (liver, lung) or bone marrow involvement
  • A/B according to systemic symptoms (fever, night sweats, weight loss)
  • a/b according to biological inflammatory syndrome
  • E: contiguous extralymphatic involvement (pleura, lung, pericardium, bone, chest wall)
  • Bulky: nodal mass > 200 mL

Deauville scale on PET-CT, recommended for treatment response assessment and based on the most intense hypermetabolism at a site initially involved

  • Score 1 = No hypermetabolism
  • Score 2 = hypermetabolism < mediastinum
  • Score 3 = mediastinum < hypermetabolism <= liver
  • Score 4 = hypermetabolism > liver
  • Score 5 = hypermetabolism >> liver and/or progression