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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: 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

Recent clinical studies

Etiology

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
Mature T-cell leukemias: Molecular and Clinical Aspects.
Bailey NG, Elenitoba-Johnson KS
Curr Hematol Malig Rep 2015 Dec;10(4):421-8. doi: 10.1007/s11899-015-0288-8. PMID: 26433891
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
Mature T-cell leukemias.
Ravandi F, Kantarjian H, Jones D, Dearden C, Keating M, O'Brien S
Cancer 2005 Nov 1;104(9):1808-18. doi: 10.1002/cncr.21405. PMID: 16136598

Diagnosis

Prolymphocytic Leukaemia: an Update on Biology and Treatment.
El-Sharkawi D, Dearden C
Curr Oncol Rep 2024 Feb;26(2):129-135. Epub 2024 Jan 2 doi: 10.1007/s11912-023-01485-3. PMID: 38214879
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
T-cell prolymphocytic leukemia/lymphoma with TCRB::TCL1 translocation.
Torabi A, Naresh KN
Blood 2023 Jul 6;142(1):119. doi: 10.1182/blood.2023020401. PMID: 37410504
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
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
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
Prolymphocytic leukemia.
Stone RM
Hematol Oncol Clin North Am 1990 Apr;4(2):457-71. PMID: 2182602

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
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
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
Prolymphocytic leukemia.
Stone RM
Hematol Oncol Clin North Am 1990 Apr;4(2):457-71. PMID: 2182602

Clinical prediction guides

Therapeutic leukocytapheresis for leukostasis in chronic lymphocytic leukemia: A case report and literature review.
Auen T, Renavikar P, Habib E, Koepsell SA
J Clin Apher 2023 Dec;38(6):764-769. Epub 2023 Jul 30 doi: 10.1002/jca.22082. PMID: 37519096
Pretransplant adaptive NKG2C+ NK cells protect against cytomegalovirus infection in kidney transplant recipients.
Ataya M, Redondo-Pachón D, Llinàs-Mallol L, Yélamos J, Heredia G, Pérez-Sáez MJ, Vila J, Costa-García M, Raïch-Regué D, Vilches C, Pascual J, Crespo M, López-Botet M
Am J Transplant 2020 Mar;20(3):663-676. Epub 2019 Nov 19 doi: 10.1111/ajt.15658. PMID: 31612635
SAMHD1 is recurrently mutated in T-cell prolymphocytic leukemia.
Johansson P, Klein-Hitpass L, Choidas A, Habenberger P, Mahboubi B, Kim B, Bergmann A, Scholtysik R, Brauser M, Lollies A, Siebert R, Zenz T, Dührsen U, Küppers R, Dürig J
Blood Cancer J 2018 Jan 19;8(1):11. doi: 10.1038/s41408-017-0036-5. PMID: 29352181Free PMC Article
Rosacea-Like Leukemia Cutis: A Case Report.
Cruz Manzano M, Ramírez García L, Sánchez Pont JE, Velázquez Mañana AI, Sánchez JL
Am J Dermatopathol 2016 Aug;38(8):e119-21. doi: 10.1097/DAD.0000000000000538. PMID: 27043335
Differential diagnosis in chronic lymphocytic leukaemia.
Matutes E, Wotherspoon A, Catovsky D
Best Pract Res Clin Haematol 2007 Sep;20(3):367-84. doi: 10.1016/j.beha.2007.03.001. PMID: 17707827

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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