T-cell large granular lymphocytic leukemia

T-cell large granular lymphocytic (TLGL) leukemia is a rare lymphoproliferative cancer of T-cell origin. Lymphoproliferative means that T lymphocytes are produced in large amounts. TLGL leukemia is characterized by large granular lymphocytes, which are bigger than normal lymphocytes. It may also be called T-cell granular lymphocytic leukemia.

 

TLGL leukemia occurs most often in older adults. The average age at diagnosis is about 60. However, it can occur in all age groups.

 

TLGL leukemia is often associated with autoimmune disorders.

  • About 25–35% of people with TLGL leukemia have rheumatoid arthritis.
  • Felty’s syndrome is a rare disorder also associated with TLGL leukemia. It includes a combination of rheumatoid arthritis, an enlarged spleen (splenomegaly) and a low white blood cell count (neutropenia).
  • Other autoimmune disorders are less frequently associated with TLGL leukemia. They include:

 

Some people with TLGL leukemia do not have any symptoms (asymptomatic). Others have recurring infections and anemia due to lower numbers of blood cells (cytopenia). The bone marrow, spleen and liver often are affected with TLGL leukemia. Lymph nodes are rarely enlarged.

 

In most cases, TLGL leukemia behaves in an indolent manner and grows slowly. The prognosis is often quite good for people with indolent TLGL leukemia. Although rare, TLGL leukemia can also behave aggressively.

Treatment

Treatment of TLGL leukemia depends on whether the person has symptoms or if the disease is causing problems. Since TLGL leukemia is a slow-growing (indolent) disease, it may not need to be treated right away.

Watchful waiting

People with TLGL leukemia may not need to be treated right away if they do not have symptoms or problems. The person is observed and treatment is started when TLGL leukemia causes symptoms or problems, such as:

  • recurring infections
  • very low white blood cell counts (neutropenia)
  • anemia
  • low platelet counts (thrombocytopenia)
  • an enlarged spleen that causes discomfort
  • other symptoms develop that require treatment

Chemotherapy

Drugs that suppress the immune system and work against the cancerous T cells are the main therapy for TLGL leukemia. The drugs most commonly used to treat TLGL leukemia are:

  • methotrexate
  • cyclophosphamide (Cytoxan, Procytox)
  • cyclosporine (Neoral)

 

Methotrexate, cyclophosphamide and cyclosporine are all effective. Each of these drugs may be used in combination with prednisone (Deltasone). Although prednisone alone improves cytopenia in most people, it can rarely produce a long-standing response on its own.

 

Other drugs that have been useful and may play a role in treating some people with TLGL leukemia include:

  • fludarabine (Fludara)
  • cladribine (2-CDA, Leustatin)
  • pentostatin (deoxycoformycin, Nipent)
  • alemtuzumab (Campath)

 

These drugs may be used if people do not respond to methotrexate, cyclophosphamide and cyclosporine.

 

Aggressive TLGL leukemia is often treated with more intensive chemotherapy, similar to that used to treat acute lymphocytic leukemia (ALL). For more information on ALL-like treatments used for aggressive TLGL leukemia, go to ALL.

 

For more detailed information on specific drugs, go to sources of drug information.

References

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