T-cell prolymphocytic leukemia (T-PLL), also known as T-cell chronic lymphocytic leukemia, T-prolymphocytic leukemia and T-cell lymphocytic leukemia, is a rare form of T-cell leukemia (a subtype of chronic lymphocytic leukemia (CLL)).
Affecting only 2% of all adult small lymphocytic leukemia patients, it is a chemotherapy-resistant, aggressive cancer unique to adults over the age of 30.
Risk Factors
There are no real known risk factors in developing T-cell prolymphocytic leukemia. However, patients have the following in common:
- Age: T-cell prolymphocytic leukemia affects people over 30 years of age.
- Gender: T-cell prolymphocytic leukemia patients tend to be more male than female.
- Race: This type of leukemia is most common in people of Japanese, West Indian, and African American descent.
Signs and Symptoms
While some patients of T-cell leukemia do not exhibit any symptoms at all, the following is a list of common symptoms:
- Bleeding or Bruising easily
- Enlarged Lymph Nodes
- Frequent Urination and/or Constipation: This is attributed to high levels of calcium in the blood.
- Recurrent Infections: These are due to low numbers of white blood cells that fight infections.
- Fatigue
- Persistent Pain in Left Abdominal Region: This may be due to an enlarged spleen.
- Rash or Skin Lesions
Diagnosis
Following a complete physical exam and medical history, a number of laboratory tests may be conducted to determine whether or not a patient has T-cell prolymphocytic leukemia. These tests may include:
- Blood Tests: A complete blood count (CBC) will measure the different types of cells present in the blood and show if the blood contains high levels of white blood cells, which may indicate T-cell leukemia.
- Bone Marrow Biopsy: This test involves taking a sample of bone marrow, usually from the patient’s hip, with a needle. This procedure can usually be done within the doctor’s office. A pathologist will analyze the extracted cells to determine which type of leukemia, if any, is present.
- Immunophenotyping: By examining the antigens, or proteins, on the cell surfaces, the doctor can discover which type of leukemia the patient has.
- Cytogenetics: This text involves examining the leukemic cells for chromosomal abnormalities. It can help confirm the diagnosis as well as determine chance of recovery.
- Computed Tomography (CT or CAT) Scan: By creating a three-dimensional picture of the patient, while sometimes by utilizing a special dye to highlight the inside of the body, a CT scan can clearly show abnormalities present with the aid of a computer. A CT scan can also confirm enlarged lymph nodes or a swollen spleen.
- Skin Biopsy: This procedure involves sampling a piece of skin tissue from the body and examining it beneath a microscope to determine the presence of T cells.
Treatment
T-cell prolymphocytic leukemia normally requires immediate treatment. T-PLL does not respond to most chemotherapy treatments and is difficult to treat. However, some treatments have had limited success. They are:
- Drug Treatments: Some drugs have been successful in certain patients, including chlorambucil and purine analogues (pentostatin, fludarabine, cladribine), as well as combination chemotherapy treatments such as cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP), cyclophosphamide, vincristine, prednisone (COP), and vincristine, doxorubicin, prednisone, etoposide, cyclophosphamide, bleomycin (VAPEC-B).
- Antibiotics: A monoclonal antibody that kills white blood cells, called Alemtuzumab (Campath), has had some success in prolonging a patient’s life. In one study, some patients were able to live an average of 16 months following treatment.
- Stem Cell Transplants: Stem cells may be used to replace cancerous cells with healthy new cells.
Prognosis
As T-cell prolymphocytic leukemia is a highly aggressive disease, patients are not expected to live a normal lifespan. Though some patients may survive five years or more with T-PLL, the average survival period following diagnosis has been, until the development of the monoclonal antibody, approximately seven and a half months.
Prevention
Without any known preventable risk factors, there is no known preventative course to take against T-cell prolymphocytic leukemia.
Resources
U.S. National Library of Medicine
American Society of Clinical Oncology
The Journal of the American Society of Hematology
