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Prolymphocytic leukemia

MedGen UID:
6062
•Concept ID:
C0023486
•
Neoplastic Process
Synonyms: Leukemia, Prolymphocytic; Leukemias, Prolymphocytic; Prolymphocytic Leukemia; Prolymphocytic Leukemias
SNOMED CT: PLL - Prolymphocytic leukemia (110006004); Prolymphocytic leukemia (clinical) (110006004); Prolymphocytic leukemia (128923008); Prolymphocytic leukemia (110006004)
 
Monarch Initiative: MONDO:0001023

Definition

A mature B- or T- cell leukemia with progressive clinical course. It is characterized by the presence of medium-sized lymphocytes with visible nucleoli (prolymphocytes) in the peripheral blood, bone marrow, and spleen. [from NCI]

Professional guidelines

PubMed

T-cell prolymphocytic leukemia: Review of an entity and its differential diagnostic considerations.
Frater JL
Int J Lab Hematol 2020 Jun;42 Suppl 1:90-98. doi: 10.1111/ijlh.13180. PMID: 32543075
Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia.
Staber PB, Herling M, Bellido M, Jacobsen ED, Davids MS, Kadia TM, Shustov A, Tournilhac O, Bachy E, Zaja F, Porkka K, Hoermann G, Simonitsch-Klupp I, Haferlach C, Kubicek S, Mayerhoefer ME, Hopfinger G, Jaeger U, Dearden C
Blood 2019 Oct 3;134(14):1132-1143. Epub 2019 Jul 10 doi: 10.1182/blood.2019000402. PMID: 31292114Free PMC Article
Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment.
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286

Recent clinical studies

Etiology

Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia.
Staber PB, Herling M, Bellido M, Jacobsen ED, Davids MS, Kadia TM, Shustov A, Tournilhac O, Bachy E, Zaja F, Porkka K, Hoermann G, Simonitsch-Klupp I, Haferlach C, Kubicek S, Mayerhoefer ME, Hopfinger G, Jaeger U, Dearden C
Blood 2019 Oct 3;134(14):1132-1143. Epub 2019 Jul 10 doi: 10.1182/blood.2019000402. PMID: 31292114Free PMC Article
T-cell prolymphocytic leukemia.
Khot A, Dearden C
Expert Rev Anticancer Ther 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365. PMID: 19275513
Prolymphocytic leukemias.
Krishnan B, Matutes E, Dearden C
Semin Oncol 2006 Apr;33(2):257-63. doi: 10.1053/j.seminoncol.2006.01.015. PMID: 16616073
T-small lymphocyte disorders.
Bartlett NL, Longo DL
Semin Hematol 1999 Apr;36(2):164-70. PMID: 10319385
Minimal residual disease.
Radich J
Curr Opin Hematol 1995 Jul;2(4):300-4. doi: 10.1097/00062752-199502040-00010. PMID: 9372011

Diagnosis

T-Cell Prolymphocytic Leukemia: Diagnosis, Pathogenesis, and Treatment.
Gutierrez M, Bladek P, Goksu B, Murga-Zamalloa C, Bixby D, Wilcox R
Int J Mol Sci 2023 Jul 28;24(15) doi: 10.3390/ijms241512106. PMID: 37569479Free PMC Article
Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia.
Staber PB, Herling M, Bellido M, Jacobsen ED, Davids MS, Kadia TM, Shustov A, Tournilhac O, Bachy E, Zaja F, Porkka K, Hoermann G, Simonitsch-Klupp I, Haferlach C, Kubicek S, Mayerhoefer ME, Hopfinger G, Jaeger U, Dearden C
Blood 2019 Oct 3;134(14):1132-1143. Epub 2019 Jul 10 doi: 10.1182/blood.2019000402. PMID: 31292114Free PMC Article
Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment.
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
T-cell prolymphocytic leukemia.
Dearden CE
Clin Lymphoma Myeloma 2009;9 Suppl 3:S239-43. doi: 10.3816/CLM.2009.s.018. PMID: 19778847
T-cell prolymphocytic leukemia.
Khot A, Dearden C
Expert Rev Anticancer Ther 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365. PMID: 19275513

Therapy

B and T cell prolymphocytic leukaemia.
Cross M, Dearden C
Best Pract Res Clin Haematol 2019 Sep;32(3):217-228. Epub 2019 Jun 6 doi: 10.1016/j.beha.2019.06.001. PMID: 31585622
Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment.
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
T-cell prolymphocytic leukemia.
Dearden CE
Clin Lymphoma Myeloma 2009;9 Suppl 3:S239-43. doi: 10.3816/CLM.2009.s.018. PMID: 19778847
Prolymphocytic leukemia.
Absi A, Hsi E, Kalaycio M
Curr Treat Options Oncol 2005 May;6(3):197-208. doi: 10.1007/s11864-005-0003-4. PMID: 15869731
Alemtuzumab.
Ravandi F, O'Brien S
Expert Rev Anticancer Ther 2005 Feb;5(1):39-51. doi: 10.1586/14737140.5.1.39. PMID: 15757437

Prognosis

B and T cell prolymphocytic leukaemia.
Cross M, Dearden C
Best Pract Res Clin Haematol 2019 Sep;32(3):217-228. Epub 2019 Jun 6 doi: 10.1016/j.beha.2019.06.001. PMID: 31585622
Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia.
Staber PB, Herling M, Bellido M, Jacobsen ED, Davids MS, Kadia TM, Shustov A, Tournilhac O, Bachy E, Zaja F, Porkka K, Hoermann G, Simonitsch-Klupp I, Haferlach C, Kubicek S, Mayerhoefer ME, Hopfinger G, Jaeger U, Dearden C
Blood 2019 Oct 3;134(14):1132-1143. Epub 2019 Jul 10 doi: 10.1182/blood.2019000402. PMID: 31292114Free PMC Article
Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment.
Collignon A, Wanquet A, Maitre E, Cornet E, Troussard X, Aurran-Schleinitz T
Curr Oncol Rep 2017 Apr;19(4):29. doi: 10.1007/s11912-017-0581-x. PMID: 28324286
T-cell prolymphocytic leukemia.
Dearden CE
Clin Lymphoma Myeloma 2009;9 Suppl 3:S239-43. doi: 10.3816/CLM.2009.s.018. PMID: 19778847
Prolymphocytic leukemia.
Absi A, Hsi E, Kalaycio M
Curr Treat Options Oncol 2005 May;6(3):197-208. doi: 10.1007/s11864-005-0003-4. PMID: 15869731

Clinical prediction guides

Current understandings on T-cell prolymphocytic leukemia and its association with TCL1 proto-oncogene.
Sun S, Fang W
Biomed Pharmacother 2020 Jun;126:110107. Epub 2020 Apr 1 doi: 10.1016/j.biopha.2020.110107. PMID: 32247279
Patient-Customized Drug Combination Prediction and Testing for T-cell Prolymphocytic Leukemia Patients.
He L, Tang J, Andersson EI, Timonen S, Koschmieder S, Wennerberg K, Mustjoki S, Aittokallio T
Cancer Res 2018 May 1;78(9):2407-2418. Epub 2018 Feb 26 doi: 10.1158/0008-5472.CAN-17-3644. PMID: 29483097
T-cell chronic lymphocytic leukemia or small-cell variant of T-cell prolymphocytic leukemia: a historical perspective and search for consensus.
Rashidi A, Fisher SI
Eur J Haematol 2015 Sep;95(3):199-210. Epub 2015 Apr 24 doi: 10.1111/ejh.12560. PMID: 25846234
Minimal residual disease.
Radich J
Curr Opin Hematol 1995 Jul;2(4):300-4. doi: 10.1097/00062752-199502040-00010. PMID: 9372011
Clinical, immunological, ultrastructural, and cytogenetic studies in black patients with adult T-cell leukemia/lymphoma.
Matutes E, Brito-Babapulle V, Catovsky D
Princess Takamatsu Symp 1984;15:59-70. PMID: 6152760

Recent systematic reviews

Advances and Perspectives in the Treatment of T-PLL.
Braun T, von Jan J, Wahnschaffe L, Herling M
Curr Hematol Malig Rep 2020 Apr;15(2):113-124. doi: 10.1007/s11899-020-00566-5. PMID: 32034661Free PMC Article
PEITC in End-Stage B-Cell Prolymphocytic Leukemia: Case Report of Possible Sensitization to Salvage R-CHOP.
Nachat A, Turoff-Ortmeyer S, Liu C, Mcculloch M
Perm J 2016 Spring;20(2):74-80. doi: 10.7812/TPP/15-153. PMID: 27168399Free PMC Article

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