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Diagnosis

How Is Cutaneous Lymphoma Diagnosed?

A diagnostic work-up for cutaneous lymphoma may contain any number of the following exams or tests:

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Following the biopsy, the skin samples are sent to a lab to be examined under a microscope by a physician called a pathologist.

 

Lymph Node Biopsies

Lymph node biopsies are generally performed to confirm a diagnosis of cutaneous lymphoma or determine the extent of the disease. A lymph node biopsy may also be performed if the physician noticed swelling in the lymph nodes during the physical exam.

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Other Types of Biopsies

The following procedures may be done in special circumstances to confirm a diagnosis of lymphoma. Most often, these procedures are used to determine the extent of the disease (a process called staging).

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Lab Tests of Biopsy and Blood Samples

In order to accurately diagnose and classify cutaneous lymphomas, biopsy and blood samples are sent to a lab to be thoroughly examined under a microscope by a physician called a pathologist. The pathologist may need to perform one or more special tests on the samples to confirm the diagnosis. These tests include:

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Skin samples may also need to be examined by a dermatopathologist (a dermatologist or pathologist with additional training in the diagnosis of skin samples).

 

Blood Tests

Blood tests are generally performed to determine the extent of the disease. They can also be used to indicate how well a patient’s bone marrow and organs are functioning while the patient is undergoing certain types of treatment. For patients with cutaneous lymphoma, the physician may order a complete blood count (CBC) or blood chemistry tests.

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The results of one particular blood test can help physicians determine the extent of the disease. This test, called lactate dehydrogenase (LDH), measures levels of a certain enzyme found in the tissues and blood. Very high levels of LDH in the blood may mean the lymphoma has spread.

 

Imaging Tests

For cutaneous T-cell lymphoma patients who have only a few lesions, imaging tests may not be necessary. However, for patients with a lot of skin involvement or for whom lymphoma cells are present in the lymph nodes or the blood, physicians often order one or more of the following imaging tests:

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Imaging tests are also done as part of the process of diagnosing cutaneous B-cell lymphoma patients to make sure they do not have internal involvement by a systemic lymphoma.

 

Staging

The stage of a disease refers to the extent of a disease (how widespread it is). Staging is most commonly done for mycosis fungoides and Sezary syndrome. Once a patient has been diagnosed with these conditions, the next step is to determine the stage of the disease.

Physicians use a number of tests and exams to determine the stage, including physical exams, biopsies, imaging tests, and blood tests.

Staging helps physicians determine the best course of treatment for patients. It is also helpful in predicting prognosis (outlook).

The staging system for cutaneous lymphomas was developed by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization for Research and Treatment of Cancer (EORTC). There are two staging systems for cutaneous lymphomas, one for mycosis fungoides and Sezary syndrome and one for other cutaneous lymphomas.

 

Stage Grouping for Mycosis Fungoides and Sezary Syndrome

The stage of mycosis fungoides or Sezary syndrome is based on four factors: the level of skin involvement, if the disease is affecting the lymph nodes, if it has spread (metastasized) to other organs or tissues in the body, and if lymphoma cells are present in the blood. When these factors are combined to determine the overall stage of the disease, it is called stage grouping.

  • Stage IA and IB: The disease is limited to the skin and consists of patches and plaques, with Stage IA having less than 10% skin involvement and Stage IB having 10% or more skin involvement. The number of Sezary cells present in the blood is low.
  • Stage IIA: Skin lesions consisting of patches and plaques involve up to 80% of the skin, but no tumors are present. The lymph nodes are enlarged but are not involved with lymphoma. In other words, the enlargement is reactive and not due to disease spread. The disease has not spread to other organs or tissues in the body. The number of Sezary cells present in the blood is low.
  • Stage IIB: In addition to patches and plaques, at least one tumor is present that is one centimeter across or larger. The lymph nodes may or may not be enlarged and are not involved with lymphoma. In the latter case, they are reactive in nature. The disease has not spread to the lymph nodes or other organs or tissues in the body. The number of Sezary cells present in the blood is low.
  • Stage IIIA: At least 80% of the skin is involved. The lymph nodes may or may not be enlarged and are not involved with lymphoma. The disease has not spread to other organs or tissues in the body. Few or no Sezary cells are present in the blood.
  • Stage IIIB: At least 80% of the skin is involved. The lymph nodes may or may not be enlarged and are not involved with lymphoma. The disease has not spread to other organs or tissues in the body. The number of Sezary cells present in the blood is low.
  • Stage IVA1: Any amount of the skin may be involved. The lymph nodes may or may not be enlarged and are not involved with lymphoma. The disease has not spread to other organs or tissues in the body. The number of Sezary cells present in the blood is high.
  • Stage IVA2: Any amount of the skin may be involved. Lymph nodes are enlarged due to the spread of lymphoma here. The disease has not spread to other organs or tissues in the body. Sezary cells may or may not be present in the blood.
  • Stage IVB: Any amount of the skin may be involved. The lymph nodes may or may not be enlarged/involved by lymphoma. The disease has spread to other organs or tissues in the body. Sezary cells may or may not be present in the blood.
    (American Cancer Society 2014)

 

Staging for Other Cutaneous Lymphomas

The staging system for other cutaneous lymphomas is relatively new, and its effectiveness is still being determined. It is based on three factors: the level of skin involvement, if the disease is affecting the lymph nodes, and if the disease has spread to other organs or tissues in the body.

Unlike the system for mycosis fungoides and Sezary syndrome, this staging system does not assign an overall stage to the disease.

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. Cutaneous B-cell lymphoma. PDF.
Lymphoma Research Foundation. 2013. Getting the Facts: Cutaneous T-Cell Lymphoma (CTCL). PDF.