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T-Cell Prolymphocytic Leukemia, Small Cell Variant

MedGen UID:
236967
•Concept ID:
C1336552
•
Neoplastic Process

Definition

A T-cell prolymphocytic leukemia characterized by the presence of small neoplastic prolymphocytes often with invisible nucleolus by light microscopy. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVT-Cell Prolymphocytic Leukemia, Small Cell Variant

Recent clinical studies

Etiology

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
A putative exonic splicing polymorphism in the BCL6 gene and the risk of non-Hodgkin lymphoma.
Zhang Y, Lan Q, Rothman N, Zhu Y, Zahm SH, Wang SS, Holford TR, Leaderer B, Boyle P, Zhang B, Zou K, Chanock S, Zheng T
J Natl Cancer Inst 2005 Nov 2;97(21):1616-8. doi: 10.1093/jnci/dji344. PMID: 16264183
Chronic lymphocytic leukemia.
Keating MJ
Semin Oncol 1999 Oct;26(5 Suppl 14):107-14. PMID: 10561025
T-small lymphocyte disorders.
Bartlett NL, Longo DL
Semin Hematol 1999 Apr;36(2):164-70. PMID: 10319385
Pentostatin in prolymphocytic leukemia: phase II trial of the European Organization for Research and Treatment of Cancer Leukemia Cooperative Study Group.
Döhner H, Ho AD, Thaler J, Stryckmans P, Sonneveld P, de Witte T, Lechner K, Lauria F, Bödewadt-Radzun S, Suciu S
J Natl Cancer Inst 1993 Apr 21;85(8):658-62. doi: 10.1093/jnci/85.8.658. PMID: 8468724

Diagnosis

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
Small cell variant anaplastic large cell lymphoma presenting as leukemia: A case report and review of Literature.
Dutta R, Ramteke P, Mathur SR, Saxena R, Pati HP, Mallick S
Indian J Pathol Microbiol 2022 Jul-Sep;65(3):705-708. doi: 10.4103/ijpm.ijpm_443_21. PMID: 35900509
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
T-cell prolymphocytic leukemia in Japan: is it a variant?
Kameoka J, Takahashi N, Noji H, Murai K, Tajima K, Kameoka Y, Sato S, Shichishima T, Ishida Y, Harigae H, Sawada K
Int J Hematol 2012 Jun;95(6):660-7. Epub 2012 Apr 24 doi: 10.1007/s12185-012-1077-y. PMID: 22527853
Chronic lymphocytic leukemia.
Keating MJ
Semin Oncol 1999 Oct;26(5 Suppl 14):107-14. PMID: 10561025

Therapy

Generalized leukaemia cutis from a small cell variant of T-cell prolymphocytic leukaemia presenting with exfoliative dermatitis.
Jeong KH, Lew BL, Sim WY
Acta Derm Venereol 2009;89(5):509-12. doi: 10.2340/00015555-0672. PMID: 19734979
Emerging drugs for rarer chronic lymphoid leukemias.
Robak T
Expert Opin Emerg Drugs 2008 Mar;13(1):95-118. doi: 10.1517/14728214.13.1.95. PMID: 18321151
Chronic lymphocytic leukemia.
Keating MJ
Semin Oncol 1999 Oct;26(5 Suppl 14):107-14. PMID: 10561025
Pentostatin in prolymphocytic leukemia: phase II trial of the European Organization for Research and Treatment of Cancer Leukemia Cooperative Study Group.
Döhner H, Ho AD, Thaler J, Stryckmans P, Sonneveld P, de Witte T, Lechner K, Lauria F, Bödewadt-Radzun S, Suciu S
J Natl Cancer Inst 1993 Apr 21;85(8):658-62. doi: 10.1093/jnci/85.8.658. PMID: 8468724
Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia.
Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D
Blood 1991 Dec 15;78(12):3269-74. PMID: 1742486

Prognosis

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
T-cell prolymphocytic leukemia in Japan: is it a variant?
Kameoka J, Takahashi N, Noji H, Murai K, Tajima K, Kameoka Y, Sato S, Shichishima T, Ishida Y, Harigae H, Sawada K
Int J Hematol 2012 Jun;95(6):660-7. Epub 2012 Apr 24 doi: 10.1007/s12185-012-1077-y. PMID: 22527853
Immunophenotyping of mature T/NK cell neoplasm presenting as leukemia.
Gujral S, Polampalli S, Badrinath Y, Kumar A, Subramanian PG, Nair R, Sengar M, Nair C
Indian J Cancer 2010 Apr-Jun;47(2):189-93. doi: 10.4103/0019-509X.63020. PMID: 20448385
Chronic lymphocytic leukemia.
Keating MJ
Semin Oncol 1999 Oct;26(5 Suppl 14):107-14. PMID: 10561025
T-small lymphocyte disorders.
Bartlett NL, Longo DL
Semin Hematol 1999 Apr;36(2):164-70. PMID: 10319385

Clinical prediction guides

T-Cell Prolymphocytic Leukemia, Small Cell Variant, Possibly at the Stage of Intracytoplasmic Expression of CD3 in T-Cell Ontogenesis.
Yoshioka Y, Nagao M, Saitoh T, Yoshioka S, Tsunemine H, Itoh K, Kodaka T, Takahashi T
J Clin Exp Hematop 2015;55(1):17-21. doi: 10.3960/jslrt.55.17. PMID: 26106002
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
T-cell prolymphocytic leukemia in Japan: is it a variant?
Kameoka J, Takahashi N, Noji H, Murai K, Tajima K, Kameoka Y, Sato S, Shichishima T, Ishida Y, Harigae H, Sawada K
Int J Hematol 2012 Jun;95(6):660-7. Epub 2012 Apr 24 doi: 10.1007/s12185-012-1077-y. PMID: 22527853
A case of T cell prolymphocytic leukemia involving blast transformation.
Ichikawa K, Noguchi M, Imai H, Sekiguchi Y, Wakabayashi M, Sawada T, Komatsu N
Int J Hematol 2011 May;93(5):667-672. Epub 2011 Apr 21 doi: 10.1007/s12185-011-0826-7. PMID: 21505955
Pentostatin in prolymphocytic leukemia: phase II trial of the European Organization for Research and Treatment of Cancer Leukemia Cooperative Study Group.
Döhner H, Ho AD, Thaler J, Stryckmans P, Sonneveld P, de Witte T, Lechner K, Lauria F, Bödewadt-Radzun S, Suciu S
J Natl Cancer Inst 1993 Apr 21;85(8):658-62. doi: 10.1093/jnci/85.8.658. PMID: 8468724

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