U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

T-cell prolymphocytic leukemia

MedGen UID:
391707
Concept ID:
C2363142
Neoplastic Process
Synonyms: Leukemia, Prolymphocytic, T-Cell; Leukemia, T-Cell Prolymphocytic; Leukemias, T-Cell Prolymphocytic; Prolymphocytic Leukemia, T-Cell; Prolymphocytic Leukemias, T-Cell; T Cell Prolymphocytic Leukemia; T-Cell Prolymphocytic Leukemia; T-Cell Prolymphocytic Leukemias
SNOMED CT: Prolymphocytic leukemia, T-cell type (128821006); T-cell prolymphocytic leukemia (128821006); Prolymphocytic leukemia of T-cell type (277567002); T-cell prolymphocytic leukemia (277567002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019468
Orphanet: ORPHA86871

Definition

A rare mature T-cell neoplasm characterized by proliferation of small to medium-sized prolymphocytes with a mature post-thymic T-cell phenotype, involving the peripheral blood, bone marrow, lymph nodes, liver, spleen, and sometimes the skin. T-cell receptor genes are clonally rearranged. Patients typically present with hepatosplenomegaly, generalized lymphadenopathy, high leukocyte count with normal serum immunoglobulins, anemia, and thrombocytopenia. HTLV-1 serology is negative. The disease course is aggressive with generally poor prognosis. [from ORDO]

Professional guidelines

PubMed

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
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
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), T-Cell Lymphomas, 2024

Recent clinical studies

Etiology

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
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
Vardi A, Vlachonikola E, Papazoglou D, Psomopoulos F, Kotta K, Ioannou N, Galigalidou C, Gemenetzi K, Pasentsis K, Kotouza M, Koravou E, Scarfó L, Iskas M, Stavroyianni N, Ghia P, Anagnostopoulos A, Kouvatsi A, Ramsay AG, Stamatopoulos K, Chatzidimitriou A
Clin Cancer Res 2020 Sep 15;26(18):4958-4969. Epub 2020 Jul 2 doi: 10.1158/1078-0432.CCR-19-3827. PMID: 32616500
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
Khot A, Dearden C
Expert Rev Anticancer Ther 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365. PMID: 19275513

Diagnosis

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
Torabi A, Naresh KN
Blood 2023 Jul 6;142(1):119. doi: 10.1182/blood.2023020401. PMID: 37410504
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
Dearden CE
Clin Lymphoma Myeloma 2009;9 Suppl 3:S239-43. doi: 10.3816/CLM.2009.s.018. PMID: 19778847
Khot A, Dearden C
Expert Rev Anticancer Ther 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365. PMID: 19275513

Therapy

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
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
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
Dearden CE
Clin Lymphoma Myeloma 2009;9 Suppl 3:S239-43. doi: 10.3816/CLM.2009.s.018. PMID: 19778847
Khot A, Dearden C
Expert Rev Anticancer Ther 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365. PMID: 19275513

Prognosis

Sun S, Fang W
Biomed Pharmacother 2020 Jun;126:110107. Epub 2020 Apr 1 doi: 10.1016/j.biopha.2020.110107. PMID: 32247279
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
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
Dearden CE
Clin Lymphoma Myeloma 2009;9 Suppl 3:S239-43. doi: 10.3816/CLM.2009.s.018. PMID: 19778847
Khot A, Dearden C
Expert Rev Anticancer Ther 2009 Mar;9(3):365-71. doi: 10.1586/14737140.9.3.365. PMID: 19275513

Clinical prediction guides

Yamaguchi M, Fukuhara N, Takizawa J, Ishitsuka K, Yokohama A, Miyazaki K, Nato Y, Ichikawa S, Mitobe M, Shima K, Miyazawa Y, Izutsu K, Suzuki R, Nagai H, Nakamura N
J Clin Exp Hematop 2024 Sep 28;64(3):216-222. Epub 2024 Jul 2 doi: 10.3960/jslrt.24028. PMID: 38960696Free PMC Article
Erkeland SJ, Stavast CJ, Schilperoord-Vermeulen J, Dal Collo G, Van de Werken HJG, Leon LG, Van Hoven-Beijen A, Van Zuijen I, Mueller YM, Bindels EM, De Ridder D, Kappers-Klunne MC, Van Lom K, Van der Velden VHJ, Langerak AW
Haematologica 2022 Jan 1;107(1):143-153. doi: 10.3324/haematol.2020.263756. PMID: 33596640Free PMC Article
Sun S, Fang W
Biomed Pharmacother 2020 Jun;126:110107. Epub 2020 Apr 1 doi: 10.1016/j.biopha.2020.110107. PMID: 32247279
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
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

Recent systematic reviews

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

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.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), T-Cell Lymphomas, 2024

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...