Exploring Anaplastic Large Cell Lymphoma: Symptoms, Causes, and Treatment Options

Anaplastic Large Cell Lymphoma (ALCL) is a rare form of cancer within the non-Hodgkin lymphoma group. It occurs when white blood cells, called lymphocytes, grow uncontrollably. Lymphocytes are vital components of your immune system, helping protect the body against infections and diseases.

There are two types of lymphocytes:

• B cells (B lymphocytes)
• T cells (T lymphocytes)

In ALCL, T lymphocytes become abnormal and grow in an uncontrolled way. The name “Anaplastic Large Cell Lymphoma” describes the appearance of these abnormal cells under a microscope:

• Anaplastic: Refers to the strange, irregular appearance of the cancer cells when compared to healthy T cells.
• Large Cell: The cancerous T lymphocytes appear larger than normal T cells.
• Lymphoma: This term refers to cancer that originates in the lymphatic system, and in ALCL, it involves the abnormal growth of T lymphocytes.

Types of ALCL

In 2016, the World Health Organization (WHO) classified ALCL into four types:

• ALK-positive ALCL (ALK+ ALCL)
• ALK-negative ALCL (ALK− ALCL)
• Primary Cutaneous ALCL (pcALCL)
• Breast Implant-Associated ALCL (BIA-ALCL)

ALK-positive and ALK-negative ALCL: These are more aggressive types of ALCL that affect the whole body. The difference between the two is based on the presence of a special protein called ALK.

ALK-positive ALCL: This type has a genetic mutation that results in an overactive ALK protein, leading to rapid cell growth.

ALK-negative ALCL: Although it doesn’t have this genetic mutation, it still has other genetic changes that may contribute to its development.

Primary Cutaneous ALCL (pcALCL): This type is less aggressive and usually affects only the skin. It may appear as a single bump or tumor, typically not spreading to other parts of the body. It can sometimes have some similar genetic changes to ALK-positive ALCL.

Cutaneous T-cell lymphoma (CTCL) is another rare type of non-Hodgkin lymphoma that affects the immune system. Unlike most forms of non-Hodgkin lymphoma, CTCL develops due to mutations in T cells. These abnormal T cells primarily target the skin, leading to the formation of lesions and other skin-related symptoms.

Breast Implant-Associated ALCL (BIA-ALCL): This type of ALCL develops around breast implants, often many years after the implant surgery. It usually causes changes in the breast’s texture, shape, or pain in the area. Unlike other types of ALCL, BIA-ALCL tends to remain localized to the area around the implant.

Overall, ALCL is a complex condition with different types that vary in how aggressively they spread and how they behave. Understanding the type of ALCL a person has is crucial for determining the best treatment.

Diagnosis of Anaplastic Large Cell Lymphoma

Anaplastic Large Cell Lymphoma (ALCL) is diagnosed by examining tissue samples under a microscope. The defining feature of ALCL is the presence of unusual cells, known as “hallmark cells”. These cells have distinct, irregularly shaped nuclei (often kidney- or horseshoe-shaped), a prominent Golgi body, and they express a specific protein on their surface called CD30.

Causes of Anaplastic Large Cell Lymphoma (ALCL)

ALCL occurs when lymphocytes (a type of white blood cell) grow uncontrollably and invade healthy tissues. While the exact cause is unknown, researchers have identified key genetic mutations linked to the disease.

Genetic Mutations and ALCL

• ALK Gene Mutation: Found in ALK-positive ALCL, this mutation causes abnormal ALK protein production, leading to rapid cell growth.
• Other Genetic Changes: Different mutations are linked to ALK-negative ALCL and other subtypes, contributing to the transformation of healthy cells into cancerous ones.

Although the specific triggers behind these mutations remain unclear, ongoing research aims to uncover what causes normal lymphocytes to turn cancerous.

Signs and Symptoms of Anaplastic Large Cell Lymphoma (ALCL)

ALCL can cause a variety of symptoms depending on the type and where the cancer is located in the body. Here are the key signs to watch for:

1. Swollen Lymph Nodes

One of the most common symptoms of ALCL is enlarged lymph nodes. These are small, bean-shaped structures that help fight infections and are located in various parts of the body, including the neck, armpits, and groin. In ALCL, the lymph nodes can swell due to the uncontrolled growth of abnormal T lymphocytes. These swollen nodes may feel firm, painless, and rubbery, but in some cases, they can be tender.

2. Fever, Unexplained Weight Loss, and Night Sweats

These symptoms, often called B symptoms, indicate that the lymphoma is affecting the entire body. They include:

• Fever: Persistent or recurring fevers without an apparent infection.
• Unintentional Weight Loss: Losing weight rapidly without dieting or exercise.
• Night Sweats: Heavy sweating, especially at night, soaking through clothing and sheets.

These symptoms may suggest that ALCL is progressing and affecting the body more systemically.

3. Skin Lesions (for Primary Cutaneous ALCL – pcALCL)

In primary cutaneous ALCL (pcALCL), the cancer mainly affects the skin, leading to:

• Red or purple skin lumps (nodules or plaques)
• Ulcers or open sores that may not heal properly
• Patches of irritated or thickened skin

These lesions are usually localized to one area, such as the arms, legs, or trunk, and they may grow slowly over time. However, in some cases, multiple skin areas may be affected. Unlike other types of ALCL, pcALCL rarely spreads to internal organs or lymph nodes.

If you notice any of these symptoms, it is important to seek medical attention, as early diagnosis and treatment can improve outcomes.