What are the Stages of Hodgkin's Lymphoma?

Following a Hodgkin’s lymphoma diagnosis, medical professionals will attempt to determine whether the cancer has spread and, if so, how far. This process is known as staging.

The staging process determines the spread of cancer in the body and helps evaluate the cancer's severity and the most effective course of treatment. When discussing survival rates, doctors also refer to the stage of the malignancy.

To determine the stage of lymphoma, several factors are considered:

1. Medical history of the patient.

2. The patient has specific symptoms known as B symptoms.

3. A physical examination is conducted.

4. Results of biopsy tests.

5. Imaging tests are carried out, including a chest X-ray, CT scan of the chest/abdomen/pelvis, and PET scan.

6. Occasionally, a bone marrow aspiration and biopsy are also conducted, although not always.

Lugano Classification

The Lugano classification is a system used by the cancer care team to determine the extent of the spread of Hodgkin's lymphoma. 

• Hodgkin lymphoma has four stages: 1, 2, 3, and 4, each of which refers to the extent to which the lymphoma cancer stages have spread from a specific lymph node location to the rest of the body. Stage 4 lymphoma suggests a significant spread. 

• The categories—A, B, E, S, and X—evaluate the presence of particular symptoms and the potential extent of the disease's progress across the body.

Cancer staging of Hodgkin's lymphoma is a way to determine the extent and spread of the disease and Hodgkin's lymphoma stages within the body. It helps doctors understand where the cancer is located, whether it has spread, and if it affects other body parts. Below are the details about the Hodgkin’s lymphoma stages.

Stage I Hodgkin’s Lymphoma

Stage I indicates that Hodgkin lymphoma is limited and can be defined as follows:

1. The lymphoma is found in only one lymph node portion or lymphoid organ, such as the thymus.

2. This stage is localised to one part of an organ outside the lymphatic system.

Stage II Hodgkin’s Lymphoma

Stage II suggests the following possibilities:

1. The lymphoma is seen in two or more lymph node portions on the same facet of the diaphragm, which is the tissue between the chest and abdomen.

2. Or else, the cancer spreads from one lymph node area into a nearby organ outside the lymphatic system or the spleen.

Stage III Hodgkin’s Lymphoma

Stage III indicates the following scenarios:

1. The lymphoma is present in lymph node areas on both sides of the diaphragm, possibly with localized involvement of an organ outside the lymphatic system or the spleen.

Stage IV Hodgkin’s Lymphoma

Stage 4 lymphoma implies that Hodgkin lymphoma has spread extensively to at least one organ outside the lymphatic system, for example, the liver, bone marrow, or lungs.

The doctors may also use other ways to describe Hodgkin’s lymphoma stages.

Bulky Disease 

This term is used when tumors in the chest are at least ⅓ the width of the chest or tumors in other areas are at least 10 centimeters (about 4 inches) across. It is denoted by adding the letter X to the stage. The bulky disease is particularly significant in stage II lymphomas, as it may require more intensive treatment.

A, B, E, S, and X Categories

Different categories are also used to stage cancer and indicate the presence or absence of particular symptoms and the extent of cancer spread beyond the lymph nodes. 

Here's what each category represents:

1. Category A: No symptoms have developed.

2. Category B: Symptoms like unexplained weight loss, drenching night sweats, or fever of at least 100.4°F (38°C) have appeared.

3. Category E: Extranodal cancers have spread from the lymph nodes to other tissues or organs.

4. Category S: The cancer has spread to the spleen. Around 30 percent of Hodgkin lymphoma patients experience an enlarged spleen called splenomegaly.

5. Category X: The disease is considered "bulky," with large tumors more than 10 cm in size spanning the chest region.

Resistant or Recurrent Hodgkin Lymphoma

Resistant or recurrent Hodgkin lymphoma is not officially part of the staging system, but doctors or nurses may use these terms to describe the lymphoma's status:

1. Resistant or Progressive Disease: Used when the lymphoma does not respond to the therapy or progresses during the treatment duration

2. Recurrent or Relapsed Disease: Indicates that the lymphoma went into remission with treatment but has now returned. The recurrence can happen in the exact location or a different body part shortly after treatment or years later.

The stage of the disease influences treatment for Hodgkin's lymphoma, assisting medical professionals in creating a suitable and efficient treatment strategy. 

The available treatments may change depending on the precise Hodgkin's lymphoma stage. According to its various stages, Hodgkin's lymphoma is treated as follows:

Stage IA and IIA: Favorable

These stages include Hodgkin's lymphoma (HL), which is restricted to only one side of the diaphragm (either above or below), and possesses no unfavorable factors. Here are the characteristics of this stage:

1. It is not bulky.

2. HL is present in less than three different lymph node areas.

3. It does not cause any of the B symptoms.

4. The erythrocyte sedimentation rate (ESR is a blood test detecting inflammation in the body) is not elevated.

Treatment approach for favourable stages:

1. Treatment for many patients in this stage involves chemotherapy, typically 2 to 4 cycles of the ABVD (Doxorubicin Hydrochloride (Adriamycin), Bleomycin Sulfate, Vinblastine sulfate, and Dacarbazine) regimen. 

2. This is followed by radiation therapy targeting the initial site of the disease, known as involved site radiation therapy (ISRT). 

3. Alternatively, selected patients may receive chemotherapy alone for 3 to 6 cycles.

4. After a few rounds of chemotherapy, doctors often order a PET/CT scan to assess the effectiveness of the treatment and determine if further treatment is necessary.

5. Radiation therapy alone may be considered if a person cannot undergo chemotherapy due to other health conditions.

Stages I and II: Unfavourable

These stages refer to Hodgkin's lymphoma (HL) that is localized on one side of the diaphragm (above or below) but exhibits one or more of the following risk factors:

1. The tumor is bulky (large).

2. HL is present in three or more distinct areas of lymph nodes.

3. Cancer is detected outside the lymph nodes (extranodal involvement).

4. B symptoms are observed.

5. The erythrocyte sedimentation rate (ESR) is high.

Treatment approach for unfavourable stages:

1. The treatment approach is generally more intensive than for favorable diseases. Typically, it involves initiating chemotherapy, often with the ABVD regimen, for four to six cycles or using alternative regimens like three cycles of Stanford V.

2. PET/CT scans are frequently conducted after several rounds of chemotherapy to assess the necessity and extent of further treatment. 

3. Subsequently, additional chemotherapy may be administered, which may differ from the previous regimen. 

4. At this stage, radiation therapy (specifically involved field radiation therapy or IFRT) is commonly employed to target the tumor sites, mainly if the disease is initially bulky.

Stages III and IV

In stages three and stage 4 Hodgkin's lymphoma, cancer has spread extensively throughout the body, involving both lymph nodes above and below the diaphragm and other organs. 

1. To treat these advanced stages, doctors usually use more intensive chemotherapy regimens compared to earlier stages. 

2. The ABVD regimen, consisting of at least six cycles, is commonly employed. However, some doctors prefer a more aggressive approach, using the Stanford V regimen for three cycles or the BEACOPP regimen for up to 8 cycles, especially if there are multiple negative prognostic factors. 

3. Another treatment option for specific individuals is combining chemotherapy with the drug brentuximab vedotin (Adcetris).

4. During or after chemotherapy, PET/CT scans may be performed to assess the comprehensive effectiveness of the treatment. Based on the scan results, additional chemotherapy may be administered if necessary. 

5. In some instances, radiation therapy may be given following chemotherapy, mainly if there are large tumor areas.

6. For individuals whose Hodgkin's lymphoma does not respond to initial treatment, alternative chemotherapy drugs or high-dose chemotherapy, possibly accompanied by radiation therapy, followed by a stem cell transplant, may be recommended. 

7. Another possibility is treatment with immunotherapy drugs like Brentuximab Vedotin, Nivolumab, or Pembrolizumab.

It is widely recommended by experts that HL patients who show resistance to treatment or experience a relapse should consider participating in a clinical trial. Clinical trials offer alternative medicines and therapies for patients in such cases.

Hodgkin's lymphoma survival rates can vary based on a number of variables, including the disease stage at the time of diagnosis. It's crucial to keep in mind that survival statistics are broad projections and might not correctly forecast a person's prognosis. Here is a summary of Hodgkin's lymphoma survival rates at various stages:

1. Stage I and II: The overall survival rate for early-stage Hodgkin's lymphoma is generally high, with a five-year survival rate ranging from 90% to 95%.

1. With appropriate treatment, the majority of patients achieve long-term remission and have a good prognosis.

2. Stage III and IV: Advanced-stage Hodgkin's lymphoma has a slightly lower overall survival rate compared to early-stage disease.

1. The five-year survival rate for stage III Hodgkin's lymphoma ranges from 80% to 85%, while for stage IV, it ranges from 65% to 70%.

2. However, it's important to note that with advancements in treatment options, including more effective chemotherapy regimens and targeted therapies, survival rates continue to improve.

Prognostic Factors

Besides the stage of the disease, other factors can influence the prognosis of Hodgkin's lymphoma.

1. Prognostic factors may include the presence of certain symptoms, such as weight loss, fever, and night sweats, as well as the level of certain proteins or markers in the blood.

2. Additionally, factors like age, gender, overall health, and response to initial treatment can impact a person's prognosis

We learned in this article that Hodgkin's lymphoma is a lymphatic system-related cancer. You have read about Hodgkin's lymphoma condition, its risk factors, symptoms, types, many Hodgkin's lymphoma stages, and treatment options according to lymphoma cancer stages, which is essential for people and families who want awareness. Increasing awareness and encouraging early detection can improve the prognosis and survival rates and strengthen care for Hodgkin's lymphoma patients.

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