T-cell prolymphocytic leukemia
Synonyms: T-PLL | T-cell chronic lymphocytic leukemia
A rare mature T-cell neoplasm characterized by proliferation of small to medium-sized prolymphocytes with a mature post-thymic T-cell phenotype involving the peripheral blood bone marrow lymph nodes liver spleen and sometimes the skin. T-cell receptor genes are clonally rearranged. Patients typically present with hepatosplenomegaly generalized lymphadenopathy high leukocyte count with normal serum immunoglobulins anemia and thrombocytopenia. HTLV-1 serology is negative. The disease course is aggressive with generally poor prognosis.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
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