Acute Large Cell Lymphoma- Types, Symptoms, Causes, Diagnosis, and Treatment

What Is Acute Large Cell Lymphoma?

Acute large-cell lymphoma is a rare type of lymphoma that is more likely to be associated with blood cancer. Lymphoma is caused by an abnormal increase in the white blood cells (lymphocytes). White blood cells are responsible for fighting against infection.

What Is the Etiology of Acute Large Cell Lymphoma?

Studies claim that acute large cell lymphoma occurs due to changes in a gene called anaplastic lymphoma kinase (ALK) or ALK-1. A marker on the surface of this changed gene is present, called the CD30 antigen, also known as Ki-1 antigen. When there is the presence of the ALK-1 gene, it is called ALK-positive anaplastic large cell lymphoma. When the gene ALK-1 is absent, the condition is called ALK-1 negative anaplastic large cell lymphoma.

What Is the Frequency of Acute Large Cell Lymphoma?

Most people above 34 are affected with ALK-positive anaplastic large cell lymphoma. The frequency of ALK-negative acute large cell lymphoma is seen in patients above the age of 54. Studies have shown that ALK-positive acute large cell lymphoma is more aggressive and fast-growing than ALK-negative acute large cell lymphoma. ALK-positive acute large cell lymphoma shows good results towards chemotherapy.

What Are the Types of Acute Large Cell Lymphoma?

Acute large cell lymphoma is of the following two types:

1. Cutaneous Acute Large Cell Lymphoma: It affects the skin. Lymphoma cells associated with this type are present only in the skin. There can usually be only one lump in the skin. It is related to an exceptional non-cancerous skin disorder called lymphomatoid papulosis. Lymphomatoid papulosis occurs due to the overgrowth of T-cells and develops to form various non-Hodgkin’s Lymphoma types, including anaplastic large cell lymphoma (ALCL).

2. Systemic Acute Large Cell Lymphoma: It affects lymph nodes. It can be present on extranodal sites such as the lungs, liver, bones, skin, bone marrow, and soft tissues.

What Are the Signs and Symptoms of Acute Large Cell Lymphoma?

The primary sign of systemic acute large cell lymphoma is swelling of the neck, armpits, groin, and the parts where the legs join the body's trunk.

Other symptoms of acute large cell lymphoma are as follows:

1. Fatigue.

2. Increased body temperature.

3. Loss of appetite (anorexia).

4. Night sweats or palpitations.

5. Loss of weight.

Cutaneous acute large cell lymphoma shows the following symptoms:

1. Raise red-colored bumps on the skin that last forever.

2. Open pores on the skin.

3. Itching on the skin.

What Are the Clinical Features of Acute Large Cell Lymphoma?

Acute large cell lymphoma shows the following features:

1. Lymph node enlargement.

2. Lymphomatoid papulosis (red or brown bumps in the skin that heals on their own).

3. The disease appears at extranodal sites such as the liver, lungs, skin, and bone marrow.

4. Soft tissue masses are also seen.

How to Make a Diagnosis of Acute Large Cell Lymphoma?

For diagnosing acute large cell lymphoma, lymph node biopsy is primarily done.

Other tests required to confirm the diagnosis are listed below:

1. Blood Tests: To check the count of lymphocytes.

2. Bone Marrow Biopsy: A special needle is used to extract a small amount of bone marrow from the bones to check for the presence of cancerous cells.

3. Chest X-ray: A low radiation dose is used to film the images of the chest organs.

4. CT (Computed Tomography): When the X-ray does not clarify the diagnosis, Computed tomography is used to get a detailed picture of acute large cell lymphoma.

5. MRI (Magnetic Resonance Imaging): This scan uses powerful magnets and radio waves to get detailed pictures of body organs and structures.

6. PET scan (Positron Emission Tomography): This scan uses radioactive materials called tracers to look for cancer.

What Is the Staging of Acute Large Cell Lymphoma?

Staging is done to check the spread ability of cancer in the body's organs. Following are the stages of acute large cell lymphoma:

1. Stage I: Lymph nodes in only one body part, like the neck or groin, are involved.

2. Stage II: Two or more groups of lymph nodes are involved. These involved lymph nodes are either below or above the diaphragm (a muscular sheet between the chest and stomach).

3. Stage III: Lymph nodes both above and below the diaphragm are involved.

4. Stage IV: Extra nodal sites such as the liver, bones, or lungs are involved.

What Is the Treatment of Acute Large Cell Lymphoma?

The following methods are used to treat acute large cell lymphoma:

1. Chemotherapy: Chemotherapy is used to treat systemic acute large cell therapy. Chemotherapy is done in combination with drugs. The most common chemotherapy combination is CHOP, which includes Cyclophosphamide, Doxorubicin such as Adriamycin, Vincristine, and Prednisolone.

2. Radiation Therapy: This method is also used to treat anaplastic large cell lymphoma.

3. External Beam Radiation Therapy: It is used in case the lymphoma cells are present in one group of lymph nodes.

4. Targeted Therapy: In this therapy, the drugs target specific molecules present on the surface of cancer cells, such as proteins. These molecules help trigger the response in the cells for growth and division. When these molecules are targeted, the drugs hinder the growth and division of cancer cells. The targeted therapy includes drugs such as Brentuximab vedotin (Adcetris) that target CD30.

5. Stem Cell Transplant: This therapy is used if the acute large cell lymphoma relapses.

What Is the Prognosis of Acute Large Cell Lymphoma?

1. The cutaneous anaplastic large-cell lymphoma (cutaneous CD30+ ALCL) shows a favorable clinical course with a five-year survival rate in 90 % of the patients. It offers an occasional spontaneous regression of the skin lesions in around one-fourth of the cases.

2. Primary cutaneous CD30- large-cell lymphoma shows a worse prognosis with five years survival rate in 15 % of the patients. Systemic anaplastic large cell lymphoma shows a worse prognosis.

Conclusion:

Acute large cell lymphoma is a type of lymphoma that majorly affects T-cells. T-cells are responsible for killing foreign invaders. Genetic changes like anaplastic lymphoma kinase (ALK) are responsible for the occurrence of acute large cell lymphoma. The article reviews the clinical features, signs, symptoms, and treatment plans for acute large cell lymphoma.