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{{DiseaseDisorder infobox |
__NOTOC__
  Name          = T-cell-prolymphocytic leukemia |
{{T-cell prolymphocytic leukemia}}                                                                 
  ICD10          =  |
{{CMG}}; {{AE}}{{Qurrat}}, {{MV}}
  ICD9          =  |
  ICDO          =  9834/3 |
{{SK}} T-cell chronic lymphocytic leukemia; "Knobby" type of T-cell leukemia; T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel;  T-PLL
  Image          =  |
  Caption        = |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  DiseasesDB    = |
}}
{{SI}}
{{CMG}}


==[[T-cell prolymphocytic leukemia overview|Overview]]==


==[[T-cell prolymphocytic leukemia historical perspective|Historical Perspective]]==


==Overview==
==[[T-cell prolymphocytic leukemia classification|Classification]]==
'''T-cell-prolymphocytic leukemia (T-PLL)''' is a mature [[T-cell]] [[leukemia]] with aggressive behavior and predilection for [[blood]], [[bone marrow]], [[lymph nodes]], [[liver]], [[spleen]], and [[skin]] involvement.<ref name="who1"> Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): [http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html World Health Organization Classification of Tumors]. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001</ref> T-PLL is a rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults.<ref name="mat1">{{cite journal |author=Matutes E, Brito-Babapulle V, Swansbury J, ''et al'' |title=Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia |journal=Blood |volume=78 |issue=12 |pages=3269-74 |year=1991 |pmid=1742486 |doi=}}</ref> Other names include: T-cell chronic lymphocytic leukemia, "Knobby" type of T-cell leukemia, T-prolymphocytic leukemia/T-cell lymphocytic leukemia<ref name="who1"/>


==Clinical Features==
==[[T-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]==
Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, [[lymph nodes]], [[bone marrow]], [[spleen]], [[liver]], [[skin]].<ref name="who1"/>


===Etiology===
==[[T-cell prolymphocytic leukemia causes|Causes]]==
It is postulated that the originating cell line for this disease is a mature (post-[[thymus|thymic]]) T-cell.<ref name="who1"/>


===Clinical Presentation===
==[[T-cell prolymphocytic leukemia differential diagnosis|Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases]]==
Patients typically have systemic disease at presentation, including [[hepatosplenomegaly]], generalized [[lymphadenopathy]], and skin infiltrates.<ref name="who1"/>


===Laboratory Findings===
==[[T-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]==
A high lymphocyte count (> 100 x 10<sup>9</sup>/L)along with [[anemia]] and [[thrombocytopenia]] are common findings. [[HTLV-1]] serologies are negative, and serum [[immunoglobin]]s are within normal limits with no [[paraprotein]]s present.<ref name="who1"/>


==Morphology==
==[[T-cell prolymphocytic leukemia risk factors|Risk Factors]]==


In the peripheral blood, T-PLL consists of medium-sized [[lymphocytes]] with single [[nucleoli]] and [[basophilic]] [[cytoplasm]] with occasional blebs or projections. The [[Cell nucleus|nuclei]] are usually round to oval in shape, with occasional patients having cells with a more irregular nuclear outline that is similar to the cerebriform nuclear shape seen in [[Sézary syndrome]].<ref name="mat2">{{cite journal |author=Matutes E, Garcia Talavera J, O'Brien M, Catovsky D |title=The morphological spectrum of T-prolymphocytic leukaemia |journal=Br. J. Haematol. |volume=64 |issue=1 |pages=111-24 |year=1986 |pmid=3489482 |doi=}}</ref> A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases.<ref name="mat2"/>
==[[T-cell prolymphocytic leukemia screening|Screening]]==


Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood.<ref name="who1"/> In the [[spleen]], the leukemic cell infiltrate both the [[red pulp]] and [[white pulp]], and [[lymph node]] involvement is typically diffuse through the [[paracortex]].<ref name="who1"/>. Skin infiltrates are seen in 20% of patients, and the infiltrates are usually dense and confined to the [[dermis]] and around the skin appendages.<ref name="mat1"/>
==[[T-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Molecular Findings==
==T-cell prolymphocytic leukemia Diagnosis==
===Immunophenotype===  
[[T-cell prolymphocytic leukemia diagnostic study of choice|T-cell prolymphocytic leukemia diagnostic study of choice]]|[[T-cell prolymphocytic leukemia history and symptoms|T-cell prolymphocytic leukemia history and symptoms]] | [[T-cell prolymphocytic leukemia physical examination|T-cell prolymphocytic leukemia physical examination]] | [[T-cell prolymphocytic leukemia laboratory findings|T-cell prolymphocytic leukemia laboratory findings]] | [[T-cell prolymphocytic leukemia electrocardiogram|T-cell prolymphocytic leukemia electrocardiogram]] | [[T-cell prolymphocytic leukemia x ray|T-cell prolymphocytic leukemia x ray]] | [[T-cell prolymphocytic leukemia echocardiography and ultrasound|T-cell prolymphocytic leukemia echocardiography and ultrasound]] | [[T-cell prolymphocytic leukemia CT scan|T-cell prolymphocytic leukemia CT-scan]] | [[T-cell prolymphocytic leukemia MRI |T-cell prolymphocytic leukemia MRI]] | [[T-cell prolymphocytic leukemia other imaging findings|T-cell prolymphocytic leukemia Other imaging findings]] | [[T-cell prolymphocytic leukemia other diagnostic studies|T-cell prolymphocytic leukemia Other diagnostic studies]]
T-PLL has the [[immunophenotype]] of a mature (post-thymic) T-lymphocyte, and the [[neoplastic]] cells are typically positive for pan-T antigens [[CD2]], [[CD3]], and [[CD7]] and negative for TdT and [[CD1a]]. The immunophenotype [[CD4]]+/[[CD8]]- is present in 60% of cases, the CD4+/CD8+ immunophenotype is present in 25%, and the CD4-/CD8+ immunophenotype is present in 15% of cases.<ref name="mat1"/> 


===Genetic Findings===
==Treatment==
Clonal TCR gene rearrangements for the γ and δ chains are typically found. The most frequent chromosomal abnormality is the inversion of chromosome 14, specifcally inv 14(q11;q32). This is found in 80% of cases, while 10% of cases show a reciprocal translocation of [[chromosome 14]] (t(14;14)(q11;q32)). <ref name="bri1">{{cite journal |author=Brito-Babapulle V, Catovsky D |title=Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia |journal=Cancer Genet. Cytogenet. |volume=55 |issue=1 |pages=1-9 |year=1991 |pmid=1913594 |doi=}}</ref>
[[T-cell prolymphocytic leukemia medical therapy|Medical Therapy]] | [[T-cell prolymphocytic leukemia surgery|Surgery]] | [[T-cell prolymphocytic leukemia  primary prevention|Primary Prevention]] | [[T-cell prolymphocytic leukemia secondary prevention|Secondary Prevention]]


<ref name="mal1">{{cite journal |author=Maljaei SH, Brito-Babapulle V, Hiorns LR, Catovsky D |title=Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization |journal=Cancer Genet. Cytogenet. |volume=103 |issue=2 |pages=110-6 |year=1998 |pmid=9614908 |doi=}}</ref> Also, abnormalities of [[chromosome 8]] are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and [[trisomy 8]].
==Case Studies==
<ref name="sor1">{{cite journal |author=Sorour A, Brito-Babapulle V, Smedley D, Yuille M, Catovsky D |title=Unusual breakpoint distribution of 8p abnormalities in T-prolymphocytic leukemia: a study with YACS mapping to 8p11-p12 |journal=Cancer Genet. Cytogenet. |volume=121 |issue=2 |pages=128-32 |year=2000 |pmid=11063795 |doi=}}</ref>
[[T-cell-prolymphocytic leukemia case study one|Case #1]]


==References==
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Latest revision as of 06:44, 28 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2], Maria Fernanda Villarreal, M.D. [3]

Synonyms and keywords: T-cell chronic lymphocytic leukemia; "Knobby" type of T-cell leukemia; T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel; T-PLL

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

T-cell prolymphocytic leukemia Diagnosis

T-cell prolymphocytic leukemia diagnostic study of choice|T-cell prolymphocytic leukemia history and symptoms | T-cell prolymphocytic leukemia physical examination | T-cell prolymphocytic leukemia laboratory findings | T-cell prolymphocytic leukemia electrocardiogram | T-cell prolymphocytic leukemia x ray | T-cell prolymphocytic leukemia echocardiography and ultrasound | T-cell prolymphocytic leukemia CT-scan | T-cell prolymphocytic leukemia MRI | T-cell prolymphocytic leukemia Other imaging findings | T-cell prolymphocytic leukemia Other diagnostic studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention

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Case #1

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