T-cell large granular lymphocyte leukemia is a subtype of acute lymphocytic leukemia. In this case, the leukemic blasts displace the cells of the bone marrow that are responsible for the formation of blood.
Cytopenia of all three cell lines (erythrocytes, platelets, granulocytes) develops clinically with symptoms of anemia, clotting disorders and susceptibility to infection. All other lymphatic or non-lymphatic organs (lymph nodes, spleen, testicles, liver, skin, bones) may also be affected. Lymph node enlargements, hepatosplenomegaly, joint and bone pain are the result. Using immunological classification, the disease can be divided into two major groups: B-cell type and T-cell type. 25% of the disease occurs in adulthood and 15% in children and adolescents, in which these is a differentiation of the subtypes with early, thymic (cortical) and mature types of the disease.
Characteristic of the disease are the typicaö features of normal T lymphocytes on the cell surface of the lymphoblasts. Relatively often, there is a mediastinal tumor that can lead to specific ailments, such as dyspnea and upper-flow congestion.
If there is no bone marrow involvement or the proportion of leukemia cells in bone marrow is below 25%, but lymph node T-ALL cells can be detected, it is a case of a T-lymphoblastic lymphoma.
The exact causes of a disease are not fully understood, but various risk factors are discussed: exposure to radioactive and certain chemical agents, treatment with certain cytotoxic agents and viral infections.
A special case here is the adult T-cell leukemia, which is triggered by an infection with the retrovirus HTLV-1 but occurs almost exclusively in Japan.
The prognosis in patients with the disease is generally rather unfavorable.