Treatment of T cell prolymphocytic leukemia
- Daniel Catovsky, MD, DSc (Med), FRCPath, FRCP, FMedSci
Daniel Catovsky, MD, DSc (Med), FRCPath, FRCP, FMedSci
- Emeritus Professor of Haematology
- Institute of Cancer Research, Sutton, Surrey, UK
- Claire Dearden, MD, BSc, FRCP, FRCPath
Claire Dearden, MD, BSc, FRCP, FRCPath
- Consultant Haematologist
- The Royal Marsden Hospital and Institute of Cancer Research, London
Prolymphocytic leukemias (PLLs) are a group of rare neoplasms characterized by a predominance of activated lymphocytes referred to as prolymphocytes in the bone marrow and peripheral blood. PLL can be of B or T cell lineage:
●T cell PLL (T-PLL) is a rare T cell neoplasm composed of lymphoid cells, typically with involvement of the peripheral blood, bone marrow, lymph nodes, and spleen. The name "prolymphocyte" is a misnomer, as the tumor cells in this disease are of post-thymic T cell origin. This class of neoplasms includes many cases previously classified as T cell chronic lymphocytic leukemia, a category no longer included in the current World Health Organization (WHO) classification.
●B cell PLL (B-PLL) is a very rare B cell neoplasm comprised of so-called prolymphocytes, typically with involvement of the peripheral blood, bone marrow, and spleen. The name "prolymphocyte" in this case is also a misnomer, as the tumor cells in this disease are mature activated B cells. By definition, these prolymphocytes comprise more than 55 percent of the cells in the blood and bone marrow.
This topic will review the management of T-PLL. The diagnosis of T-PLL and the diagnosis and management of B-PLL are presented separately. (See "B cell prolymphocytic leukemia" and "Clinical manifestations, pathologic features, and diagnosis of T cell prolymphocytic leukemia".)
INDICATIONS FOR TREATMENT
Not all patients with T-PLL require treatment at the time of diagnosis. Active treatment is typically reserved for patients with symptomatic disease. This is principally because:
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