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 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:To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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