T-cell large granular lymphocyte leukemia

Other Names: Proliferation of large granular lymphocytes; T-LGL; T-cell LGL leukemia; Large Cell Granular Lymphogenous Leukemia; T-LGL leukemia (… sel larj GRAN-yoo-lur LIM-foh-site loo-KEE-mee-uh) A type of leukemia in which large T lymphocytes (a type of white blood cell) that contain granules (small particles) are found in the blood. It is a chronic disease that may last for a long time and get worse. Also called T-LGL leukemia.

T-cell large granular lymphocyte leukemia causes a slow increase in white blood cells called T lymphocytes, or T cells, which originate in the lymph system and bone marrow and help to fight infection. This disease usually affects people in their sixties.

Cause

The exact cause of LGL leukemia is unknown.

Signs and symptoms

Symptoms include anemia; low levels of platelets (thrombocytopenia) and infection-fighting neutrophils (neutropenia) in the blood; and an enlarged spleen. About one-third of patients are asymptomatic at the time of diagnosis.

Diagnosis

Doctors can diagnose this disease through a bone marrow biopsy, or by using a specialized technique in which various types of blood or bone marrow cells are separated, identified, and counted.

Treatment

There is currently no gold standard treatment for T-LGL leukemia. Most patients are treated with low doses of methotrexate, cyclophosphamide, and cyclosporine-A. As a rule, the treatment should be continuously monitored and, if necessary, adjusted to maintain the desired response. However, treatment compliance is frequently low, with a consequent relapse of the disease. Moreover, side effects are common and may limit the treatment effectiveness .

Patients with T-LGL were treated with fludarabine if they presented with B symptoms, progressive splenomegaly, recurrent infection or anemia. Fludarabine was used as a first-line treatment or salvage therapy. HIV-positive patients were excluded from the study.

Fludarabine was used at a dosage of 40 mg/m2/day pathway oral for three to five days monthly for 6/8 cycles. Granulocyte colony-stimulating factor was given to all patients beginning from the 10th day of each cycle until the end of the cycle, and it was given for five days if the neutrophil count was less than 1.0×109/L because it was not possible to differentiate between neutropenia secondary to the disease or due to drug toxicity. Prophylaxis for Pneumocystis jiroveci with trimethoprim-sulfamethoxazole was also indicated for all patients.

NIH genetic and rare disease info

• NIH info on T-cell large granular lymphocyte leukemia

T-cell large granular lymphocyte leukemia is a rare disease.

T-cell large granular lymphocyte leukemia Resources
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