What Are the Different Stages of Hodgkin Lymphoma?

Hodgkin lymphoma, a type of blood cancer, has four stages, each classified depending on how far throughout the body the cancer cells have metastasized from the original site.

Hodgkin lymphoma (HL), also called Hodgkin’s disease, is a type of cancer that occurs when white blood cells called B lymphocytes (B cells) grow out of control. 

Non-Hodgkin lymphoma (NHL) is slightly different and occurs when white blood cells called T lymphocytes (T cells) grow out of control. 

What Are Symptoms of Hodgkin Lymphoma?

Symptoms of Hodgkin lymphoma include: 

• Lump(s) under the skin
  • Most common symptom
  • Lumps are enlarged lymph nodes
  • May occur in the neck, under the arm, or in the groin
  • Usually not painful but may become painful after drinking alcohol
  • May get bigger over time, or new lumps might appear near it or even in other parts of the body
• “B symptoms” (usually indicate the lymphoma is more advanced)
  • Fever (which may come and go over several weeks) without an infection
  • Drenching night sweats
  • Weight loss without trying (at least 10% of body weight over 6 months)
• General (non-specific) symptoms
  • Itching skin
  • Tiredness (fatigue)
  • Loss of appetite
  • Cough, trouble breathing, and chest pain if lymph nodes in the chest are affected

The cause of Hodgkin lymphoma is unknown. 

Risk factors for developing Hodgkin lymphoma include: 

• Epstein-Barr virus infection/mononucleosis (“mono”)
• Age: most common in early adulthood (especially in a person’s 20s) and in late adulthood (after age 55)
• Gender: occurs slightly more often in males than in females.
• Family history
• Weakened immune system, such as in people infected with human immunodeficiency virus, people who take medicines to suppress the immune system after an organ transplant, and people with auto-immune diseases 

Hodgkin lymphoma is diagnosed with a physical examination, patient history, and tests such as: 

• Biopsies
  • Excisional biopsy: removal of entire lymph node
  • Incisional biopsy: removal of a small part of a larger tumor or node 
  • Needle biopsy
    • Fine needle aspiration (FNA) biopsy 
    • A core needle biopsy 
  • Bone marrow aspiration and biopsy
• Imaging tests
• Chest X-ray
• Computed tomography (CT) scan
  • CT-guided needle biopsy
• Magnetic resonance imaging (MRI)
• Positron emission tomography (PET) scan
  • PET/CT scan
• Bone scan
• Blood tests 
  • Used to determine how advanced the HL is and how well a patient might tolerate certain treatment
    • Complete blood count (CBC) 
    • Erythrocyte sedimentation rate (ESR) 
    • Liver and kidney function 
    • Pregnancy test
    • HIV test: if symptoms might be related to HIV infection
    • Hepatitis B and C virus test: Certain chemo drugs could cause problems in patients who have these infections
• Tests of heart and lung function
• Echocardiogram (an ultrasound of the heart) 
• Multiple-gated acquisition (MUGA) scan to check the heart
• Lung (pulmonary) function tests (PFTs) 

Treatments for Hodgkin lymphoma may include one or more of the following: 

• Chemotherapy 
  • Along with radiation therapy, chemotherapy is one of the main treatments for Hodgkin lymphoma
  • Usually given in combinations
  • ABVD is the most common regimen used in the U.S.
    • Adriamycin (doxorubicin)
    • Bleomycin
    • Vinblastine
    • Dacarbazine (DTIC)
  • Other common regimens include:
    • BEACOPP
    • Bleomycin
    • Etoposide (VP-16)
    • Adriamycin (doxorubicin)
    • Cyclophosphamide
    • Oncovin (vincristine)
    • Procarbazine
    • Prednisone
    • Stanford V
    • Doxorubicin (Adriamycin)
    • Mechlorethamine (nitrogen mustard)
    • Vincristine
    • Vinblastine
    • Bleomycin
    • Etoposide
    • Prednisone
• Radiation therapy 
  • Along with chemotherapy, radiation therapy is one of the main treatments for Hodgkin lymphoma
  • Radiation is given after chemo in the Stanford V regimen and sometimes after the ABVD or BEACOPP regimens
    • External beam radiation
    • Involved site radiation therapy (ISRT)
    • Involved field radiation therapy (IFRT)
    • Extended field radiation (rarely done today)
    • Total body irradiation: often used along with high-dose chemotherapy in patients getting a stem cell transplant 
• Immunotherapy 
  • Monoclonal antibodies: brentuximab vedotin (Adcetris) and rituximab (Rituxan)
  • Immune checkpoint inhibitors: nivolumab (Opdivo) and pembrolizumab (Keytruda)
• High-dose chemotherapy and stem cell transplant 
  • Autologous stem cell transplant
    • Most common type of transplant for Hodgkin lymphoma
    • Uses a patient’s own blood stem cells 
  • Allogeneic stem cell transplant 
    • Uses blood stem cells from someone else, usually a brother or sister, but could be an unrelated donor or umbilical cord blood

The Lugano classification is used to determine the different stages of Hodgkin lymphoma. 

For limited stage (I or II) Hodgkin lymphoma that affects an organ outside of the lymph system, the letter E is added to the stage (for example, stage IE or IIE).

• Stage I 
  • Cancer is found in only 1 lymph node area or lymphoid organ such as the thymus (I) or
  • Cancer is found only in 1 part of 1 organ outside the lymph system (IE)
• Stage II 
  • Cancer is found in 2 or more lymph node areas on the same side of (above or below) the diaphragm, which is the thin muscle beneath the lungs that separates the chest and abdomen (II) or
  • Cancer extends locally from one lymph node area into a nearby organ (IIE)
• Stage III 
  • Cancer is found in lymph node areas on both sides of (above and below) the diaphragm (III) or
  • Cancer is found in lymph nodes above the diaphragm and in the spleen
• Stage IV 
  • Cancer has spread (metastasized) widely into at least one organ outside of the lymph system, such as the liver, bone marrow, or lungs

Each stage may also be assigned a letter (A or B). B is added (stage IIIB, for example) if a patient has “B symptoms,” including:

• Loss of more than 10% of body weight over the previous 6 months (without dieting)
• Unexplained fever of at least 100.4°F (38°C)
• Drenching night sweats

B symptoms usually indicate the lymphoma is more advanced, and more intensive treatment is typically recommended.