Clinical manifestations, pathologic features, and diagnosis of T cell prolymphocytic leukemia
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
- Claire Dearden, MD, BSc, FRCP, FRCPath
Claire Dearden, MD, BSc, FRCP, FRCPath
- Consultant Haematologist
- The Royal Marsden Hospital and Institute of Cancer Research, London
T cell prolymphocytic leukemia (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 actually 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.
The epidemiology, clinical presentation, pathology, and diagnosis of T-PLL are discussed here. The treatment of T-PLL is presented separately. (See "Treatment of T cell prolymphocytic leukemia".)
T-PLL is an extremely rare disease, comprising approximately 2 percent of mature lymphocytic leukemias in adults . Sporadic T-PLL mainly affects the elderly with a mean age at presentation of 65 years . It has not been reported in children or young adults. There is a slight male predominance with a male:female ratio of 1.33 .
Patients with ataxia telangiectasia have a greatly increased incidence of T-PLL with a different epidemiologic profile . In contrast to patients with sporadic T-PLL, the median age of onset of T-PLL in patients with ataxia telangiectasia is about 30 years of age, and some cases appear in adolescence . (See "Ataxia-telangiectasia".)
Most patients with T-PLL present with an elevated white blood count (typically >100,000/microL), hepatosplenomegaly (75 percent), and generalized lymphadenopathy (50 percent); anemia (36 percent) and thrombocytopenia (51 percent) can be seen, but are less common than in B cell prolymphocytic leukemia . In addition, skin infiltration and serous effusions (ie, pleural) occur in approximately 25 and 15 percent of patients, respectively . Involvement of the central nervous system is rare. Infrequently, asymptomatic patients are diagnosed as part of the evaluation of abnormal laboratory studies.
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- CLINICAL FEATURES
- - Peripheral blood and bone marrow
- - Skin
- - Other tissues
- Genetic features
- DIFFERENTIAL DIAGNOSIS
- B cell prolymphocytic leukemia
- Mycosis fungoides/Sézary syndrome
- Chronic lymphocytic leukemia
- Adult T cell leukemia-lymphoma
- Hairy cell leukemia
- T cell large granular lymphocyte leukemia