Subcutaneous T-cell lymphoma

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at July 25, 2016
StartDiseasesSubcutaneous T-cell lymphoma

Subcutaneous T-cell lymphoma is also known as subcutaneous panniculitis T-cell lymphoma. It is a blood cancer disease that produces nodules beneath the surface of the skin, usually on the legs and trunk. It is a rare form of lymphoma that affects the subcutaneous fat in the body but does not generally involve the skin. It is similar to other types of T-cell lymphomas but presents with slightly different symptoms and has no specific treatment protocol though a number of treatments are available.

Contents

Definition & Facts

Lymphomas are one of the most common types of blood cancer along with leukemia and myeloma, and they affect the lymphatic system. Non-Hodgkin's lymphoma is one type of lymphoma. T-cell lymphomas, a type of Non-Hodgkin's lymphoma, make up about 15 percent of all non-Hodgkin's lymphomas.

Subcutaneous T-cell lymphoma is considered a subtype of T-cell lymphoma that causes nodules to form in fatty tissue. It occurs quite rarely. There is debate and revision about how best to classify subcutaneous T-cell lymphoma with prior subtypes now classified under the cutaneous T-cell lymphoma umbrella. This type of cancer primarily refers to nodules that form on, as opposed to below, the skin.

Subcutaneous T-cell lymphoma is often referred to as “panniculitis-like” because the nodules in the fat cells beneath the skin look similar to panniculitis, an inflammation that occurs in fatty tissue. The disease generally presents two different types, one that is slow-growing and easily treated and a more aggressive form that is more difficult to treat. The disease generally affects young adults. 

Symptoms & Complaints 

Subcutaneous T-cell lymphoma presents with multiple nodules under the skin, generally on the extremities or the trunk of the body. Nodules may be 0.5 cm to several centimeters in size. Large nodules may become necrotic (cause cell death), but the dermis and epidermis are usually not affected, so they generally do not ulcerate on the surface of the skin.

The disease can sometimes activate the immune system of the body, causing an enlarged liver (hepatomegaly) and spleen (splenomegaly) and fever as well as depleting the body's supply of red blood cells, white blood cells, and platelets. This collection of symptoms is called hemophagocytic syndrome and can make treatment more difficult. 

Causes

Science has not yet identified the cause of the development of non-Hodgkin lymphomas like subcutaneous T-cell lymphoma. In addition, these cancers are very diverse, which makes it difficult to pinpoint specific factors that may trigger the abnormal cell growth.

Non-Hodgkin's cancers do not appear to be genetic in their origin. However, a number of factors are linked with its development. DNA changes leading to abnormal cell growth appear to be acquired after birth, such as from radiation exposure, viral infection, or contact with carcinogens (cancer-causing chemicals). Generally, individuals with immune system deficiencies, autoimmune diseases or those who have chronic infections appear to be most at risk. The following factors seem to favor the development of subcutaneous T-cell lymphoma and/or non-Hodgkin's lymphoma:

  • Age. The median age of onset for subcutaneous panniculitis-like lymphoma is 36.
  • Gender. Women are more likely than men to develop subcutaneous T-cell lymphoma.
  • Whites tend to develop non-Hodgkin lymphoma more often than African Americans or Asian Americans.
  • Living in a developed nation increases the risk for non-Hodgkin lymphoma.
  • Being overweight or obese appears to increase the risk for these cancers.
  • Women with breast implants may have a slightly higher risk for developing cutaneous T-cell lymphoma.

Diagnosis & Tests

The individual may seek medical attention because of the development of one or more nodules under the skin that don’t go away. In general, the nodules do not ulcerate. However, in some cases, this can occur causing open, painful lesions that must be treated aggressively to prevent widespread destruction of tissue.

Blood tests help to determine the state of red blood cells, white blood cells and platelets that can indicate the type of lymphoma in questions. Specialized imaging of the nodules can analyze the exact nature of the growths to allow specific diagnosis. Finally, biopsy of the lesions allows the physician to identify the cells that are affected to help diagnose the specific type of lymphoma. 

Subcutaneous T-cell lymphoma is sometimes misdiagnosed as benign panniculitis which impedes early detection and treatment and reduces survival outcomes.

Treatment & Therapy 

Because subcutaneous T-cell lymphoma is a rare type of cancer, specific treatments have not yet been determined. A number of different modalities may be used depending on the severity of the symptoms and the overall condition of the patient. A combination of chemotherapy drugs, commonly called CHOP, is used to treat this condition. The combination includes cyclophosphamide (C), doxorubicin/hydroxydaunorubicin (H), vincristine/oncovin (O), and prednisone (P) - a type of corticosteroid.

Patients with less aggressive forms of the disease may be given single medications, such as methotrexate, ciclosporin (an immunosuppressive drug), or prednisone, which can successfully control the disease for long periods of time. External beam radiation can be used to treat subcutaneous T-cell lymphoma.

Stem cell transplantation may be offered as an option for patients who do not appear to be responding to other methods of treatment. The prognosis for recovery from this condition depends on the subtype of subcutaneous T-cell lymphoma. Cancers of the gamma delta T-cell have a poor prognosis and high rates of metastasis. Cancers of the T-cells that have an alpha/beta T cell receptor chain have much better prognosis and a five-year survival rate that exceeds 80%.

Prevention & Prophylaxis 

There is no way to definitively prevent subcutaneous T-cell lymphoma because its exact causes are still mysterious. The cellular process that causes the DNA to repeat biological data incorrectly in cancers is not well understood.

However, because some data indicates that exposure to certain types of chemicals increase the risk for non-Hodgkin lymphomas such as subcutaneous T-cell lymphoma, an individual may be able to reduce his or her risk for developing this group of lymphomas by following certain precautions:

  • Individuals who work around certain environmental toxicants like benzene which is often found in laboratories, insecticides and herbicides as well as in agricultural and horticultural industries, should be particularly careful about exposure and should wear appropriate safety gear when working around these chemicals. 
  • Individuals who have autoimmune diseases such as lupus erythematosus, rheumatoid arthritis and other problems should receive appropriate treatment and be under a physician’s care to monitor them for other conditions. 
  • Individuals with compromised immune systems should monitor their condition closely and report any unusual growths under the skin promptly to their physicians.
  • Certain immune system diseases and infections, such as HIV and Epstein-Barr virus infection, may increase the risk for developing this disease.
  • Individuals who have received radiation treatments for cancer are at higher risk for developing other types of cancers and should be monitored closely for any unusual growths.