T-prolymphocytic leukaemia
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Article Attributes
T cell prolymphocytic leukaemia (T-PLL) is a rare and unusual haematological malignancy.
Epidemiology
It represents around 2% of all mature lymphocytic leukemias in adults over the age of 30 1. It usually affects older adults with an average age at presentation being around 65 years. There may be a slight male predominance.
Clinical presentation
While there can be wide clinical spectrum, many patients tend to present with hepatosplenomegaly and and generalised lymphadenopathy.
Pathology
It is characterised by the proliferation of small- to medium-sized prolymphocytes of pos-tthymic origin. There can be a wide spectrum where involvement of the peripheral blood, bone marrow, lymph nodes, liver, spleen, and skin can occur.
Radiographic features
General
The imaging spectrum can also be variable with hepatosplenomegaly and and generalised lymphadenopathy being common. Pleural effusions and cutaneous lesions may also be detectable in a small but sizable number of patients.
Treatment and prognosis
Clinical course is often very aggressive with poor response to conventional chemotherapy. Short survival rates have usually been reported with the usual potential long-term curative treatment being hematopoietic stem cell transplantation. Treatment with purine analogues and alemtuzumab have been reported to have significantly higher response rates and improved survival 2-3.
See also
-<p><strong>T cell prolymphocytic leukaemia (T-PLL)</strong> is a rare and unusual haematological malignancy.</p><h4>Epidemiology</h4><p>It represents around 2% of all mature lymphocytic leukemias in adults over the age of 30 <sup>1</sup>. It usually affects older adults with an average age at presentation being around 65 years. There may be a slight male predominance.</p><h4>Clinical presentation </h4><p>While there can be wide clinical spectrum, many patients tend to present with hepatosplenomegaly and and generalised lymphadenopathy.</p><h4>Pathology</h4><p>It is characterised by the proliferation of small- to medium-sized prolymphocytes of pos-tthymic origin. There can be a wide spectrum where involvement of the peripheral blood, bone marrow, lymph nodes, liver, spleen, and skin can occur.</p><h4>Treatment and prognosis</h4><p>Clinical course is often very aggressive with poor response to conventional chemotherapy. Short survival rates have usually been reported with the usual potential long-term curative treatment being hematopoietic stem cell transplantation. Treatment with purine analogues and alemtuzumab have been reported to have significantly higher response rates and improved survival<sup> 2</sup>.</p><h4>See also</h4><ul><li><a title="WHO classification of tumours of haematopoietic and lymphoid tissues" href="/articles/who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues">WHO classification of tumours of haematopoietic and lymphoid tissues</a></li></ul>- +<p><strong>T cell prolymphocytic leukaemia (T-PLL)</strong> is a rare and unusual haematological malignancy.</p><h4>Epidemiology</h4><p>It represents around 2% of all mature lymphocytic leukemias in adults over the age of 30 <sup>1</sup>. It usually affects older adults with an average age at presentation being around 65 years. There may be a slight male predominance.</p><h4>Clinical presentation </h4><p>While there can be wide clinical spectrum, many patients tend to present with hepatosplenomegaly and and generalised lymphadenopathy.</p><h4>Pathology</h4><p>It is characterised by the proliferation of small- to medium-sized prolymphocytes of pos-tthymic origin. There can be a wide spectrum where involvement of the peripheral blood, bone marrow, lymph nodes, liver, spleen, and skin can occur.</p><h4>Radiographic features</h4><h5>General </h5><p>The imaging spectrum can also be variable with hepatosplenomegaly and and generalised lymphadenopathy being common. Pleural effusions and cutaneous lesions may also be detectable in a small but sizable number of patients.</p><h4>Treatment and prognosis</h4><p>Clinical course is often very aggressive with poor response to conventional chemotherapy. Short survival rates have usually been reported with the usual potential long-term curative treatment being hematopoietic stem cell transplantation. Treatment with purine analogues and alemtuzumab have been reported to have significantly higher response rates and improved survival<sup> 2-3</sup>.</p><h4>See also</h4><ul><li><a href="/articles/who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues">WHO classification of tumours of haematopoietic and lymphoid tissues</a></li></ul>
References changed:
- 1. Graham R, Cooper B, Krause J. T-Cell Prolymphocytic Leukemia. Proc (Bayl Univ Med Cent). 2013;26(1):19-21. <a href="https://doi.org/10.1080/08998280.2013.11928902">doi:10.1080/08998280.2013.11928902</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23382603">Pubmed</a>
- 2. Dearden C. T-Cell Prolymphocytic Leukemia. Clin Lymphoma Myeloma. 2009;9 Suppl 3:S239-43. <a href="https://doi.org/10.3816/CLM.2009.s.018">doi:10.3816/CLM.2009.s.018</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19778847">Pubmed</a>
- 3. Khot A & Dearden C. T-Cell Prolymphocytic Leukemia. Expert Rev Anticancer Ther. 2009;9(3):365-71. <a href="https://doi.org/10.1586/14737140.9.3.365">doi:10.1586/14737140.9.3.365</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19275513">Pubmed</a>
- 4. Adelson R, Samy R, Lindberg G. T-Cell Prolymphocytic Leukemia Presenting as an Ear Canal Mass. Otol Neurotol. 2004;25(6):1006-8. <a href="https://doi.org/10.1097/00129492-200411000-00024">doi:10.1097/00129492-200411000-00024</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15547434">Pubmed</a>
- 5. Matutes E, Garcia Talavera J, O'Brien M, Catovsky D. The Morphological Spectrum of T-Prolymphocytic Leukaemia. Br J Haematol. 1986;64(1):111-24. <a href="https://doi.org/10.1111/j.1365-2141.1986.tb07579.x">doi:10.1111/j.1365-2141.1986.tb07579.x</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/3489482">Pubmed</a>
Systems changed:
- Haematology
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