ABSTRACT
Prolymphocytic leukemia is a rare disease with typical characteristics, including aggressive progression and high mortality rate, and T-cell prolymphocytic leukemia (T-PLL) is regarded as an intractable subtype. Current therapeutic approaches mainly aim to improve their efficacy and remission against this disease. This paper presents a review about the research progress on T-PLL therapies. With remarkable research progress on leukemia pathogenesis, therapies for T-PLL have been greatly enhanced. Among treatment strategies, epigenetic therapy shows potential for clinical applications.
ABSTRACT
T-cell prolymphocytic leukemia (T-PLL) is a rare mature post-thymic T-cell malignancy with infiltration to the blood, bone marrow, lymph node, liver, spleen and skin; this disease has a poor prognosis and an aggressive clinical course. We report here on a case of CD56+ T-PLL that was diagnosed by hematological examination, immunophenotyping and molecular studies including determining the TCL1 expression by using reverse-transcriptase polymerase chain reaction (RT-PCR), and direct sequencing of the RT-PCR product.
Subject(s)
Bone Marrow , Immunophenotyping , Leukemia, Prolymphocytic, T-Cell , Liver , Lymph Nodes , Oncogenes , Polymerase Chain Reaction , Prognosis , Skin , Spleen , T-LymphocytesABSTRACT
T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy that has an aggressive clinical course and it is a distinct clinico-biological entity from other T-cell disorders. It is now apparent that this disease represents a separate entity from CLL. Clinically, T-PLL presents with hepatosplenomegaly, lymphadenopathy, skin lesion, and marked lymphocytosis exceeding 100x109/L. Because its clinical course is aggressive, the treatment is difficult. We report a case of small cell variant of T-cell with a review of literatures.