U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
T-Cell Prolymphocytic Leukemia With t(X;14)(q28;q11.2): A Clinicopathologic Study of 15 Cases.
Hu Z, Medeiros LJ, Xu M, Yuan J, Peker D, Shao L, Tang Z, Mai B, Thakral B, Rios A, Hu S, Wang W
Am J Clin Pathol 2023 Apr 4;159(4):325-336. doi: 10.1093/ajcp/aqac166. PMID: 36883805
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

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
B-prolymphocytic leukemia: Is it time to retire this entity?
El Hussein S, Khoury JD, Medeiros LJ
Ann Diagn Pathol 2021 Oct;54:151790. Epub 2021 Jul 16 doi: 10.1016/j.anndiagpath.2021.151790. PMID: 34293709
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

Therapy

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
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

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

Progression and transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma and B-cell prolymphocytic leukemia: Report from the 2021 SH/EAHP Workshop.
Czader M, Amador C, Cook JR, Thakkar D, Parker C, Dave SS, Dogan A, Duffield AS, Nejati R, Ott G, Xiao W, Wasik M, Goodlad JR
Am J Clin Pathol 2023 Jun 1;159(6):554-571. doi: 10.1093/ajcp/aqad027. PMID: 37052539Free PMC Article
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
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...