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Cutaneous T-Cell Lymphoma (CTCI) is a rare type of blood cancer that starts in the white blood cells and then attacks the skin. There are several types of cutaneous T-cell lymphoma, including mycosis fungoides, and Sezary syndrome.
What Is Cutaneous Lymphoma?
Cutaneous lymphoma refers to a group of lymphomas that present in the skin at the time of diagnosis. A lymphoma is a cancer of a type of white blood cell called a lymphocyte.
Lymphocytes are part of the body’s immune system, and their job is to protect the body against infections.
The two main types of lymphocytes are B lymphocytes (B cells) and T lymphocytes (T cells). In lymphoma, one or more lymphocytes (B-cell or T-cell) mutate and grow in an uncontrolled manner. Lymphomas are classified according to the cell type they originate from, thus there are B-cell lymphomas and T-cell lymphomas.
Lymphocytes are found within the lymphatic system, which includes the lymph nodes, lymphatic vessels, bone marrow, spleen, and thymus. Most systemic lymphomas present with enlargement within the lymphatic system, such as enlarged lymph nodes or an enlarged spleen, and may also present with symptoms such as fever, night sweats, and weight loss.
Cutaneous lymphomas develop from a small subgroup of lymphocytes that reside in the skin and are outside of the lymphatic system. Thus, cutaneous lymphomas present with lesions on the skin at the time of diagnosis. They do, however, have the potential to spread inside the body and may spread to lymph nodes or other organs, such as the liver or spleen.
Cutaneous lymphomas are rarer and behave very differently than the more common systemic lymphomas and require different types of treatments. Most are slow growing and not aggressive. They usually proceed over many years, typically do not make the patient seriously ill, and can be treated multiple times. Some rare types of cutaneous lymphomas can be more aggressive (fast growing and spreading). A physician will be able to provide more information and advice regarding these. Cutaneous lymphomas can be T-cell or B-cell. Cutaneous T-cell lymphomas (CTCLs) are more common than cutaneous B-cell lymphomas (CBCLs).
They are classified using a system created by the World Health Organization and the European Organization for Research and Treatment of Cancer. The system is called the WHO-EORTC classification. The system classifies a lymphoma based on its appearance under the microscope and whether certain proteins are present on the infected cells.
Types of Cutaneous T-Cell Lymphoma
Cutaneous T-cell lymphomas make up 75% to 80% of cutaneous lymphomas. Most CTCLs are indolent (slow growing) and not life threatening. CTCLs are treatable, but they are not curable unless the patient undergoes a stem cell transplant (see later). There are several different types of CTCL.
Mycosis Fungoides
Mycosis fungoides (MF) makes up about half of all cases (American Cancer Society 2014). MF can develop at any age, but most people with the disease are around 50 to 60 years old. The disease is nearly twice as likely to develop in men as it is in women (American Cancer Society 2014).
The physical symptoms of MF vary from patient to patient. Symptoms include flat, scaly, red lesions called patches; thicker, raised lesions called plaques; and raised lumps called tumors. Patches, plaques, and tumors are characteristically itchy. Tumors may be ulcerated (broken open) in the center.
MF in the early stages can be easily confused with other skin problems, such as eczema, psoriasis, or dermatitis, making it difficult to diagnose.
MF is generally a slow-growing disease. It may stay confined to the skin for many years. In fact, patients with early-stage, limited disease have a life expectancy similar to other members of the general population.
Sezary Syndrome
Sezary syndrome accounts for approximately 15% of cutaneous T-cell lymphoma cases (Cutaneous Lymphoma Foundation 2012). In this condition, patients develop a red rash that covers almost the entire body (called erythroderma). The skin is often thickened and can be hot, very itchy, and sore. Unlike other types of CTCLs, which affect only the skin, Sezary syndrome affects both the skin and the blood. Lymphoma cells called Sezary cells are found in the skin and the blood, and lymph nodes are often enlarged.
Sezary syndrome tends to grow and spread faster than mycosis fungoides. It is also harder to treat. Patients with Sezary syndrome often have highly compromised immune systems, which increases a patient’s risk of serious infection.
Primary Cutaneous CD30-Positive Lymphoproliferative Disorders (pcCD30+LPD)
This is the second most common group of CTCLs, making up about 30% of CTCL cases, and is used to define a group of lymphomas that share a common biology, in that the lymphoma cells exhibit a certain protein called CD30. This group of lymphomas includes two main subsets: lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (C-ALCL).
Lymphomatoid Papulosis
Lymphomatoid papulosis (LyP) is not a life-threatening disease and does not generally affect an individual’s overall health or wellbeing. The disease often presents as multiple lesions that may ulcerate in the center and typically comes and goes on its own without treatment. The lesions heal by turning scaly or forming a crust. The disease can take months, or even years, to disappear completely. Unlike other cutaneous T-cell lymphomas, LyP does not spread to the lymph nodes or other organs
The occurrence of a second lymphoma (most commonly mycosis fungoides) can happen in as many as one-fifth of LyP patients, regardless of treatment. It is therefore important for patients to be followed up long term by their physicians.
Primary Cutaneous Anaplastic Large Cell Lymphoma
Primary cutaneous anaplastic large cell lymphoma (C-ALCL) typically presents as one or a few lesions on the skin that are generally larger than those of LyP and do not typically go away without treatment, although in some cases they can. The lesions have a tendency to ulcerate in the center and can be itchy.
This particular type of CTCL generally has a very good prognosis (outlook). Spread of this lymphoma internally to the lymph nodes may happen in up to 10% of patients.
As C-ALCL and LyP belong to the same family of conditions, they are often found together in the same patient.
Subcutaneous Panniculitis-Like T-Cell Lymphoma
Subcutaneous panniculitis-like T-cell lymphoma is a rare, slow-growing type of cutaneous T-cell lymphoma that causes lumps to form in the deepest layers of the skin. It can occur anywhere in the body but most commonly on the legs. It generally has a good prognosis.
Primary Cutaneous Peripheral T-Cell Lymphoma, Unspecified
Cutaneous T-cell lymphomas that do not fall into any of the above categories are called primary cutaneous peripheral T-cell lymphoma, unspecified. The lymphomas in this group are very rare and often fast growing.
Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T-Cell Lymphoma
The physical symptoms of this particular lymphoma include widespread patches, nodules, and tumors that are ulcerated in the center. It can look similar to mycosis fungoides, but a biopsy can differentiate between the two.
Primary Cutaneous Gamma/Delta T-Cell Lymphoma
Primary cutaneous gamma/delta T-cell lymphoma develops primarily on the arms and legs (and occasionally in the intestines or the lining of the nose) as thickened plaques or tumors.
Primary Cutaneous CD4+ Small/Medium Sized Pleomorphic T-Cell Lymphoma
This type of lymphoma tends to begin as a single lesion or a tumor on the face, neck, or upper portion of the body. There may be multiple lesions, but this is less common. The disease is generally slow growing. Patients with this type of lymphoma tend to have a better prognosis than patients with other lymphomas in this group.
Other rare types of lymphomas include adult T-cell leukemia/lymphoma (which is linked to the HTLV-1 virus) and extranodal NK/T-cell lymphoma, nasal type (which is linked to infection with the Epstein-Barr virus [EBV]). Though it is possible for these types of T-cell lymphomas to be confined to the skin, they are more likely to start elsewhere in the body.
Types of Cutaneous B-Cell Lymphoma
Cutaneous B-cell lymphomas (CBCLs) are much rarer than cutaneous T-cell lymphomas and make up about 20% to 25% of cutaneous lymphomas. More common systemic B-cell lymphomas, such as Hodgkin’s disease, can spread to the skin and mimic a CBCL. A skin biopsy alone would not be able to tell the difference between the two presentations. Thus, at diagnosis, following a skin biopsy, a CT scan is done to determine if there is internal involvement by a systemic lymphoma. The three main types of CBCL are:
Primary Cutaneous Marginal Zone B-Cell Lymphoma
This slow-progressing lymphoma often presents with multiple small, pink bumps that frequently recur on the skin after treatment. The outcome for these patients is excellent and similar to that of the general population.
Primary Cutaneous Follicle-Center Lymphoma
This also has a slowly progressing course and presents with single or multiple pink/violet skin lesions that tend to be on the head and face or, less commonly, on the trunk and legs. The disease rarely spreads internally, and patients generally have an excellent outcome.
Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type
Unlike the other two CBCLs, this lymphoma characteristically presents with fast-growing red/violet lesions on the legs, although it can also present elsewhere. The disease is more common in elderly female patients. It also has a higher probability of spreading internally in affected individuals.
Symptoms of Cutaneous Lymphoma
The physical symptoms of cutaneous lymphoma vary from patient to patient, but typically include:
- Papules (pimple-like lesions)
- Nodules (larger papules)
- Patches (flat, scaly lesions)
- Plaques (thicker, raised lesions)
- Tumors (raised lumps)
- Erythroderma (a red rash that can cover most of the skin on the body)
They are characteristically itchy and can vary in color from red to brown to purple. Some lesions may ulcerate (break open) in the center, giving them the appearance of open sores.
Cutaneous T-cell lymphomas are more likely to present as patches or plaques on the trunk or buttocks, whereas cutaneous B-cell lymphomas tend to present as papules or nodules on the head and neck, back, or legs.
On rare occasions, cutaneous T-cell lymphoma can cause general symptoms, such as fever, severe itchiness, profuse sweating, and unexplained weight loss. It is uncommon for cutaneous B-cell lymphomas to cause these symptoms.
Most of these symptoms are more likely caused by other, less serious conditions.
Causes of Cutaneous Lymphoma
Although the exact causes remain unknown, certain risk factors connected to the disease have been identified. While risk factors may be useful in identifying high-risk individuals, they do not determine whether a person develops a disease. Some risk factors are within our control, while others are not.
Possible risk factors for cutaneous lymphoma include:
- Age: They generally develop in people 50 years of age and older. One notable exception is lymphomatoid papulosis, which can also affect younger people.
- Gender: They tend to affect men more than women.
- Race: African Americans are at higher risk than Caucasians.
- Compromised immune system: They often develop in people with weakened immune systems. Any number of things can weaken a person’s immune system, including infection and disease, certain medications used to suppress the immune system, or an organ transplant (people who receive an organ transplant are often given medications to suppress the immune system to ensure the donor organ is not rejected by the host’s body).
- Infection and disease: As mentioned above, infection and disease can weaken the immune system. People with human immunodeficiency virus (HIV) or acquired immune deficiency syndrome (AIDS) are at higher risk.
References
American Cancer Society. 2014. Lymphoma of the Skin. PDF.
Cutaneous Lymphoma Foundation. 2012. A Patient’s Guide to Understanding Cutaneous Lymphoma. PDF.
Lymphoma Association. 2013. Lymphoma Association. 2013. Cutaneous B-cell lymphoma. PDF.. PDF.
Lymphoma Research Foundation. 2013. Getting the Facts: Cutaneous T-Cell Lymphoma (CTCL). PDF.