T-cell prolymphocytic leukemia overview: Difference between revisions
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==Overview== | ==Overview== | ||
T-cell-prolymphocytic leukemia (also known as T-PLL) is a rare, mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin. T-cell prolymphocytic leukemia was first described by Catovsky in 1973. There is no classification system for T-cell prolymphocytic leukemia. The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia. T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults. T-cell prolymphocytic leukemia is more commonly observed among young adult patients aged between 30 to 40 years old. Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females. Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include | T-cell-prolymphocytic leukemia (also known as T-PLL) is a rare, mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin. T-cell prolymphocytic leukemia was first described by Catovsky in 1973. There is no classification system for T-cell prolymphocytic leukemia. The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia. T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults. T-cell prolymphocytic leukemia is more commonly observed among young adult patients aged between 30 to 40 years old. Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females. Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include high lymphocyte count (> 100 x 109/L), anemia, thrombocytopenia, and negative HTLV-1 serology. There are no specific imaging findings associated with T-cell prolymphocytic leukemia. Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately 7 months. The mainstay of therapy for T-cell prolymphocytic leukemia is alemtuzumab (anti-CD52). However, T-cell prolymphocytic leukemia is often resistant to therapy. Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission. | ||
==Historical Perspective== | ==Historical Perspective== | ||
T-cell prolymphocytic leukemia was first described 40 years ago and was classified in 1994. | |||
==Classification== | ==Classification== | ||
T-cell prolymphocytic leukemia is classified into three variants based on its morphology. | |||
==Pathophysiology== | ==Pathophysiology== | ||
T-PLL has the [[immunophenotype]] of a mature (post-thymic) T-lymphocyte, and the [[neoplastic]] cells are typically positive for pan-T antigens. Clonal TCR gene rearrangements for the γ and δ chains are typically found.It arises from mature (post-thymic) T-cell, which are normally involved in in cell-mediated immunity. On microscopic histopathological analysis, T-cell prolymphocytic leukemia has characteristic findings. In the peripheral blood, T-PLL consists of medium-sized [[lymphocytes]] with single [[nucleoli]] and [[basophilic]] [[cytoplasm]] with occasional blebs or projections. Different variants of T-cell Lymphocytic leukemias can be differentiated based on the microscopic and gross finding. | |||
==Causes== | ==Causes== | ||
T-cell prolymphocytic leukemia is caused by gene mutations and chromosomal abnormalities. Details of which are described below. | |||
==Differentiating Xyz from Other Diseases== | ==Differentiating Xyz from Other Diseases== | ||
T-cell prolymphocytic leukemia presents with [[Lymphadenopathy]], [[hepatomegaly]], and [[fever]] which are also presenting symptoms of a number of other conditions. | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
Revision as of 18:32, 30 March 2019
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T-cell prolymphocytic leukemia Microchapters |
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Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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T-cell prolymphocytic leukemia overview On the Web |
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American Roentgen Ray Society Images of T-cell prolymphocytic leukemia overview |
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Risk calculators and risk factors for T-cell prolymphocytic leukemia overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2]
Synonyms and keywords: T-cell chronic lymphocytic leukemia; "Knobby" type of T-cell leukemia; T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel; T-PLL
Overview
T-cell-prolymphocytic leukemia (also known as T-PLL) is a rare, mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin. T-cell prolymphocytic leukemia was first described by Catovsky in 1973. There is no classification system for T-cell prolymphocytic leukemia. The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia. T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults. T-cell prolymphocytic leukemia is more commonly observed among young adult patients aged between 30 to 40 years old. Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females. Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include high lymphocyte count (> 100 x 109/L), anemia, thrombocytopenia, and negative HTLV-1 serology. There are no specific imaging findings associated with T-cell prolymphocytic leukemia. Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately 7 months. The mainstay of therapy for T-cell prolymphocytic leukemia is alemtuzumab (anti-CD52). However, T-cell prolymphocytic leukemia is often resistant to therapy. Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission.
Historical Perspective
T-cell prolymphocytic leukemia was first described 40 years ago and was classified in 1994.
Classification
T-cell prolymphocytic leukemia is classified into three variants based on its morphology.
Pathophysiology
T-PLL has the immunophenotype of a mature (post-thymic) T-lymphocyte, and the neoplastic cells are typically positive for pan-T antigens. Clonal TCR gene rearrangements for the γ and δ chains are typically found.It arises from mature (post-thymic) T-cell, which are normally involved in in cell-mediated immunity. On microscopic histopathological analysis, T-cell prolymphocytic leukemia has characteristic findings. In the peripheral blood, T-PLL consists of medium-sized lymphocytes with single nucleoli and basophilic cytoplasm with occasional blebs or projections. Different variants of T-cell Lymphocytic leukemias can be differentiated based on the microscopic and gross finding.
Causes
T-cell prolymphocytic leukemia is caused by gene mutations and chromosomal abnormalities. Details of which are described below.
Differentiating Xyz from Other Diseases
T-cell prolymphocytic leukemia presents with Lymphadenopathy, hepatomegaly, and fever which are also presenting symptoms of a number of other conditions.