The different Stages of Hodgkin’s Lymphoma and the Survival Rate

Overview

Hodgkin’s lymphoma is a type of blood-cancer that is named after the researcher Thomas Hodgkin who documented its pathology in 1832. The condition occurs in four stages, and further, there are five staging categories. All these details help monitor the progress of the condition closely and derive the prognosis or long-term outlook for the patient. Compared to Non-Hodgkin lymphoma, Hodgkin Lymphoma is more predictable, easily treatable and shows good survival rates, thanks to rapid advances in treatment methods, over the years.

Introduction

Hodgkin Lymphoma or HL, is a type of blood-cancer that affects the lymphatic system. In this condition, one of the types of white-blood-cells called lymphocytes, which circulates both in the blood and lymphatic system, grow abnormally. They crowd out the healthy WBCs in the blood, and in the lymphatic system, they accumulate in various lymph-nodes throughout the body as well as lymphoid-organs such as spleen and thymus-gland. The condition can also spread to organs outside the lymphatic system (that is other than spleen and thymus-gland, which includes liver, lungs, heart, etc).

Since lymphocytes are critical to the overall immunity of the person, HL can affect the person’s immunity making him/her prone to infection and disease.

Stages of HL

HL progresses in stages that shows how the disease has progressed. The treatment methods chosen, and prognosis for the patient depend on which stage of the condition he/she is in.

Stage 1

• The cancer is seen in one lymph node region, or one lymphoid organ.
• Treatment consists of 2-4 rounds of chemotherapy, as well as radiation therapy in some cases.

Stage 2

• The cancer is seen in two or more lymph node regions, on the same side of the body, that is either above or below the diaphragm. The diaphragm is a thin wall of muscle that separates the chest from the abdomen.
• The cancer also affects one organ outside the lymphatic system, and a nearby lymph node region. This is considered as stage 2E Hodgkin lymphoma.
• Treatment consists of 2-4 rounds of chemotherapy, as well as radiation therapy in some cases.

Stage 3

• The cancer is seen in both sides of the diaphragm.
• In addition to lymph nodes, it has quite possibly affected the spleen by now.
• Treatment consists of 6-8 rounds of chemotherapy, steroidal drugs and radiation therapy.

Stage 4

• In addition to cancer in the lymph nodes, the cancer has now spread to a minimum of one organ outside the lymphatic system, such as the lungs, liver or bone-marrow.
• Treatment consists of 6-8 rounds of chemotherapy, steroidal drugs and radiation therapy.

In addition to the above 4 stages, there is also a type of HL that deserves mention.

Refractory or recurrent Hodgkin lymphoma

Refractory disease is the name given to HL when it does not respond to initial therapy. Recurrent disease is the name given to HL that has come back after it was treated. The relapse may happen shortly after treatment, or a few years later.

Staging Categories

In addition to 4 stages, there are 5 staging categories. These refer to how the condition has progressed beyond the lymph nodes, and also what are the symptoms present and absent then. These categories are denoted by letters.

A – This indicates that no symptoms have developed.

B – This is used when symptoms include unexplained weight loss, fever and intense sweating in the nights.

E – This indicates extra-nodal cancers. That is, the condition has spread beyond the lymph nodes into other tissues or organs.

S – This is used when the cancer has definitely spread to the spleen. In nearly 30 percent of the cases, the patient will have an enlarged spleen and this condition is called splenomegaly.

X – This is used when the condition is considered “bulky,” with tumours as large as 10 cms and above, spanning the chest region.

So, a combination of stage and category is used to identify the condition in a particular patient. For example, 1E, 2E, 3S, 4S, etc.

Survival Rates and Prognosis

In the past, patients treated for HL often developed long-term health issues including leukaemia, breast cancer, myelodysplastic syndrome, thyroid disease, heart disease, lung cancer, lung disease and infertility. Most of these conditions were caused by treatment methods used for HL.

Since 1975, the survival rates for people diagnosed with HL has been steadily going up. While the 5-year survival rate in 1975 was 69.9%, it was 85.4% in 2009. In fact, the death rate for people with HL has dropped by 4.5% every year between 2010 and 2019. (Source: NCI)

The progress is attributed to improvements or advances in treatment methods. Previously, treatment involved heavy doses of radiation and chemotherapy. These methods would destroy the cancer cells as well as healthy cells in the vicinity, triggering secondary cancers and other complications over time. But over the years, treatment for HL has become very advanced. This includes:

• Special types of Chemotherapy such as ABVD chemotherapy and BEACOPP chemotherapy
• Radiation therapy that is more focused on the cancer cells, so healthy cells are unharmed
• Stem cell therapy. The patient’s stem cells are harvested before start of the treatment and then pumped back into his/her bone-marrow after treatment to trigger the generation of healthy blood-cells.

The survival rates have improved over the years thanks to various factors:

• Stem cell transplants and high-dose salvage chemotherapy has improved survival rates for HL patients who had a relapse
• For people who are not responding to treatment initially, today, there are various therapies used such as Targeted-drug therapy using monoclonal antibodies, immunotherapy using checkpoint inhibitors, and nonmyeloablative stem-cell transplants.
• Complications like infections due to neutropenia (low white blood cell count) caused by chemotherapy are managed better today, which have improved survival rates.

So today, for people across different stages of HL, survival rates are as follows:

• At one year: 92% (92% of the patients were still alive, one year after the treatment was stopped)
• At five years: 87%
• At 10 years: 80%

Five-year survival rates by stage are as follows. This is a relative survival rate and what this means is explained in the 1^st point:

• Stage I: 5-year survival rate is 90% (patient had a 90% chance of survival compared to a person who never had HL, after 5 years after the condition was diagnosed)
• Stage II: 5-year survival rate is 90%
• Stage III: 5-year survival rate is 80%
• Stage IV: 5-year survival rate is 65%

All the above figures are very heartening, and indicate that of all types of blood-cancer, HL has the most positive prognosis or outlook, or is the most treatable, with a high chance of survival. Most patients do make it out of the condition, getting back to a normal, healthy and long life.

Factors affecting prognosis

In spite of the above conclusions, it is important to note that there are a whole lot of factors that affect the prognosis, given that all patients are not alike. Factors that decide the actual survival and recovery are:

• Stage of the disease: Compared to patients in stage 3 or 4, patients in Stage I or stage II had a better prognosis/outlook/survival-rate/life-expectancy.
• Age: Patients in their 20s and 30s had a higher rate of survival than those above 45 years of age.
• Gender: Women have a better prognosis than men.
• Presence of B symptoms: People with B symptoms of lymphoma that includes weight-loss, fever and sweating in the night had a poorer prognosis than those who did not. Yet, most of them can achieve long-term survival.
• Relapse: Patients who had a relapse in the first year following treatment had a poorer prognosis than those who had no relapse, or relapse much later.
• Response to treatment: Patients who respond positively to first-line therapies have a better prognosis than those who show no response.
• Albumin level: patients with a low albumin level (less than 4 g/dL) had poorer prognosis.
• WBC count: A high WBC count (more than 15,000 cells per mm3) is associated with a poorer prognosis.
• Absolute-Lymphocyte-Count (ALC): An ALC of less than 600 cells per mm3 is associated with a poorer prognosis.
• Bulky tumours: Patients with less bulky tumours have a better prognosis than those with bulky tumours.
• Anaemia: A low haemoglobin level (less than 10.5 g/dL) is linked with a poorer prognosis, while high levels of haemoglobin is associated with high survival-rates.
• ESR: An Erythrocyte Sedimentation Rate (ESR) more than 30 is linked with a poorer prognosis.
• Type of HL: Some types of HL have a better prognosis than others. For example, survival rate for nodular lymphocyte predominant HL and nodular sclerosing HL is better than some other types.
• Secondary cancers: The presence or absence of secondary cancers. Patients who did not develop secondary cancers had a better prognosis.

It is also important to note that all the figures and factors mentioned above are all purely historical. Which means, this is what the records till date tell us. However, with rapid advances in treatment methods, these figures and factors could change for the better.

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