Nodular Lymphocyte-Predominant Hodgkin Disease - Clinical features, Stages, Diagnosis, and Treatment

Introduction:

Lymphocytes are white blood cells that play an important role in the immune system. There are two types of lymphocytes; they are T-lymphocytes and B-lymphocytes. The lymphocytes fight against foreign invaders in the body (antigens). The T cells kill the foreign bodies, and the B cell produces antibodies against the antigen. The T cell travel to the lymph node and the B cells travel to the lymph node and lymphatic system. The lymph system is a network of organs, vessels, and tissues that moves the lymph fluid into the circulatory system. The lymphatic system includes lymph nodes, lymph vessels, and collecting ducts. The lymph organs are the spleen, thymus, tonsils, payer’s patches, appendix, and bone marrow. The lymph system fights against infections in the body.

What Is Hodgkin’s Lymphoma?

It is a special group of lymphomas. These lymphomas are named after the doctor Hodgkin. They have a good prognosis and are easily diagnosed. It is a rare cancer of the lymph system of the body. In this condition, the B-lymphocytes develop genetically.

1. The B lymphocytes proliferate abnormally and accumulate as a cohesive mass in the lymph organs and lymphatic vessels. So the lymph system loses its ability to fight against infection.

2. The spread of disease in Hodgkin’s is very orderly. It follows a stepwise, contiguous spread. It follows an anatomically arranged pattern of spread from the first group to the last group of lymph nodes. They do not skip any lymph nodes in between. The non-hodgkin’s lymphoma spread randomly. This orderly spread of Hodgkin's disease makes it very easy to determine the stage of cancer and initiate the treatment.

3. The features of severe inflammation are present in Hodgkin’s lymphoma. The highly inflammatory cells disrupt the function of the hypothalamus.

4. Hodgkin's lymphoma usually starts as one lymph node or a chain of lymph nodes. Usually, axial lymph nodes are involved. Waldeyer’s ring and mesenteric lymph node are less commonly involved. The disease is characterized by special types of cells called Reedstein berg cells.

5. Reed-Sternberg cells are present only in Hodgkin’s lymphoma and live longer than others. Reed-Sternberg-like cells may be present in non-Hodgkin’s lymphoma. In Hodgkin’s disease, the Reedstein berg cells are present in an appropriate background of reactive inflammatory cells, the hallmark diagnostic feature of Hodgkin’s lymphoma.

6. There are five subtypes of Hodgkin’s lymphoma:

   1. Lymphocyte-rich Hodgkin’s disease.

   2. Nodular lymphocyte predominant Hodgkin’s disease.

   3. Nodular sclerosis.

   4. Mixed cellularity lymphoma.

   5. Lymphocyte-depleted lymphoma.

What is Nodular Lymphocyte-Predominant Hodgkin's Disease?

It is a rare type of Hodgkin’s lymphoma that grows very slowly. They develop from B lymphocytes. It grew in lymph nodes and got its name (nodular). The cells of these lymphocytes look like popcorn under the microscope, so they are called popcorn cells. Around 200 to 300 people are diagnosed with this condition in the United Kingdom. It most commonly occurs in the young adult group from 30 up to the age of 50 and is more common in males than females. The disease expresses CD20 predominantly and lacks CD30 cells. The presence of lymphocyte predominant cells is the diagnostic prerequisite of NLPHL. The NLPHL is an indolent type of lymphoma because it grows very slowly.

What Are the Clinical Features of Nodular Lymphocyte Predominant Hodgkin’s Disease?

It has distinct clinical and pathological features, they are:

• Classically the disease presents as a cohesive mass. Not rubbery and non-tender, not painful but less commonly painful.

• The lumps develop in the neck, groin, and armpits.

• Swollen lymph nodes due to the build-up of lymphocytes in the lymph nodes.

• Painless lymph nodes.

• Fever

• Unexplained weight loss

• Night sweats

• Unexplained pruritis.

• Anemia.

• Palestine intermittent fever.

• In very severe cases, the patient develops hepatosplenomegaly if the treatment is done at the appropriate time.

What Are the Stages of the Disease?

There are two stages as follows:

• Early Stage - Stages 1 and 2 are early. It is generally found localized or in nearby areas of the body,

• Advanced - Stages 3 and 4 are advanced stages. They spread to many parts of the body from the original site.

What Are the Diagnostic Tests Done for Nodular Lymphocyte-Predominant Hodgkin’s Disease?

The diagnosis is done by taking a biopsy. A swollen lymph node is removed, and are looked for lymphoma cells. The staging of the disease is done by visually seeing the location of the lymphomas in the CT (computed tomography) scan, PET (positron emission tomography) scan, and MRI (magnetic resonance imaging) scan. Blood tests are done to see the general health of the body,

What Is the Treatment for Nodular Lymphocyte-Predominant Hodgkin’s Disease?

The prognosis of the early stage is very good, with survival rates exceeding 90 percent. On the contrary, the late-stage diagnosis leads to relapse with either the same disease or aggressive B cell non-hodgkin’s lymphoma. Local radiotherapy is done for Hodgkin’s lymphoma. Hodgkin’s lymphoma is a curable disease. The prognosis is very good in stage 1 and 2 cancer. But without proper treatment, the disease is fatal. But if diagnosed and treated appropriately, it has the best prognosis. Chemotherapy and radiotherapy are gold-standard treatments for Hodgkin’s lymphoma. Long-term survivors of Hodgkin’s lymphoma develop acute myeloid leukemias, lung cancers, breast cancers, or any other chemotherapy or radiotherapy-related cancers. Surgical treatment is not done for this disease. However, people with multiple NLPHL respond well to salvage therapy. Salvage therapies involve single-agent anti-CD20 antibody treatment to high-dose chemotherapy followed by stem cell transplantation.

The chemotherapy drugs for this disease are as follows:

• Adriamycin, Bleomycin, Vinblastine, Dacarbazine (ABVD).

• Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone (CHOP).

• Cyclophosphamide, Vinblastine, Prednisolone (CVP).

• Bleomycin, Etoposide, Doxorubicin, Cyclophosphamide, Vincristine, Procarbazine, and Prednisolone (BEACOPP).

• Rituximab is added to any of the above-mentioned combinations.

What Is a Relapse in NLPHD?

Though the treatment is effective the chances of relapse also happen that need more treatment, relapsed lymphoma is treated in other ways. In this case, a different dose and regimen are used and Rituximab is given along with chemotherapy. A stem cell transplant is done when the relapse occurs soon after the first treatment. Sometimes after the initial treatment, it can transform into aggressive B-non-Hodgkin’s lymphoma. The chance of transformation is 10 percent. In patients with histologically confirmed recurrent NLPHL, different approaches include conventional chemotherapy, radiotherapy, and single-agent anti-CD20 antibody treatment followed by autologous stem cell transplantation.

Conclusion:

Nodular lymphocyte predominant Hodgkin’s disease is a subtype of Hodgkin’s lymphoma. Swollen lymph nodes, fever, weight loss, and night sweats are the common symptoms. In some cases, there is relapse or recurrent so the individuals will continue the treatment again.