Definition:
Chronic T-Cell Leukemia, also referred to as T-cell-prolymphocytic leukemia (T-PLL), is a post-thymic T-cell malignancy primarily inflicting adults aged 30 and above. This is a severe form of T-cell leukemia with aggressive behavior, predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement.
Chronic T-Cell Leukemia is a rare disease with distinct clinical features following an aggressive clinical course. Within the post-thymic T-cell dysfunctions that develop as a result of leukemia, T-PLL is seen in about a third of documented cases.
Diagnosis:
In diagnosing Chronic T-Cell Leukemia, a high lymphocyte count is a common indicator. Anemia and thrombocytopenia may also show up in lab results. Frequently, HTLV-1 serologies turn out negative, whereas serum immunoglobin levels are within normal ranges.
Treatment:
Afflicted patients are rarely responsive to treatment. Alkylating agents (e.g. chlorambucil) are used, but with little or short-lived success. Some patients respond well to CHOP therapy (cyclophosphamide, doxorubicin, vincristine, and prednisone), but most responses to this form of treatment are partial or incomplete. In most patients, chronic T-cell leukemia recurs.
Symptoms and Signs:
Afflicted patients usually present with systemic disease, which can range from hepatosplenomegaly, to generalized lymphadenopathy, to skin infiltrates. This is a rapidly progressing disease, and if left untreated, patients may die. The survival rate of patients with chronic T-cell leukemia is slim.
Causes:
The origins of Chronic T-Cell Leukemia is believed to be a mature (i.e. post-thymic) T-cell. It is an aggressive, rapidly progressing disease that evolves from leukemia.
Because of the disease's systemic nature, leukemic cells may be found in peripheral lymph nodes, spleen, blood, liver, bone marrow, and skin.
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