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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{T-cell prolymphocytic leukemia}} |
| {{CMG}}; {{AE}}{{Qurrat}}, {{MV}} | | {{CMG}}; {{AE}}{{Qurrat}}, {{MV}} |
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| {{SK}} T-cell chronic lymphocytic leukemia; "Knobby" type of T-cell leukemia; T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel; T-PLL | | {{SK}} T-cell chronic lymphocytic leukemia; "Knobby" type of T-cell leukemia; T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel; T-PLL |
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| ==Overview==
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| '''T-cell-prolymphocytic leukemia''' (also known as ''T-PLL'') is a rare, mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin. T-cell prolymphocytic leukemia was first described by Catovsky in 1973. There is no classification system for T-cell prolymphocytic leukemia. The inversion of [[chromosome 14]] (14q11) has been associated with the development of T-cell prolymphocytic leukemia. T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults. T-cell prolymphocytic leukemia is more commonly observed among young adult patients aged between 30 to 40 years old. Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females. Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include: high [[lymphocyte]] count (> 100 x 109/L), [[anemia]], [[thrombocytopenia]], and negative HTLV-1 serology. There are no specific imaging findings associated with T-cell prolymphocytic leukemia. Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately 7 months. The mainstay of therapy for T-cell prolymphocytic leukemia is [[alemtuzumab]] (anti-CD52). However, T-cell prolymphocytic leukemia is often resistant to therapy. Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission.
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| ==Historical Perspective==
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| *40 years ago, in 1973, Catovsky first described four cases of T-cell prolymphocytic leukemia.<ref name="pmid4124423">{{cite journal |vauthors=Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G |title=Prolymphocytic leukaemia of B and T cell type |journal=Lancet |volume=2 |issue=7823 |pages=232–4 |date=August 1973 |pmid=4124423 |doi= |url=}}</ref><ref name="pmid28340878">{{cite journal |vauthors=Sud A, Dearden C |title=T-cell Prolymphocytic Leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=31 |issue=2 |pages=273–283 |date=April 2017 |pmid=28340878 |doi=10.1016/j.hoc.2016.11.010 |url=}}</ref>
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| *In 1994, Harris a pathologist from Boston and his colleagues made an effort to classify T-cell prolymphocytic leukemia.<ref name="pmid8068936">{{cite journal |vauthors=Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC |title=A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group |journal=Blood |volume=84 |issue=5 |pages=1361–92 |date=September 1994 |pmid=8068936 |doi= |url=}}</ref>
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| ==Classification==
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| *T-cell prolymphocytic leukemia classification is based on its morphology. <ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref><ref name="pmid26980727">{{cite journal |vauthors=Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES |title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms |journal=Blood |volume=127 |issue=20 |pages=2375–90 |date=May 2016 |pmid=26980727 |pmc=4874220 |doi=10.1182/blood-2016-01-643569 |url=}}</ref><ref name="pmid1742486">{{cite journal |vauthors=Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D |title=Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia |journal=Blood |volume=78 |issue=12 |pages=3269–74 |date=December 1991 |pmid=1742486 |doi= |url=}}</ref>
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| *
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| {| align="center"
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| ! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Morphological Variant
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| ! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Percentage of total number
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| |-
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| ! colspan="1" align="center" style="background:#DCDCDC;" |Typical T-cell prolymphocytic leukemia
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| ! colspan="1" align="center" style="background:#DCDCDC;" |75 percent
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| |-
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| ! colspan="1" align="center" style="background:#DCDCDC;" |Small cell variant
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| ! colspan="1" align="center" style="background:#DCDCDC;" |20 percent
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| |-
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| ! colspan="1" align="center" style="background:#DCDCDC;" |Cerebriform (Sézary cell-like) variant
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| ! colspan="1" align="center" style="background:#DCDCDC;" |5 percent
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| |}
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|
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| ==Pathophysiology==
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| *T-cell prolymphocytic leukemia is a uncommon T- cell malignancy.
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| *It arises from mature (post-thymic) T-cell, which are normally involved in in cell-mediated immunity.<ref name="pmid283408782">{{cite journal |vauthors=Sud A, Dearden C |title=T-cell Prolymphocytic Leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=31 |issue=2 |pages=273–283 |date=April 2017 |pmid=28340878 |doi=10.1016/j.hoc.2016.11.010 |url=}}</ref>
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| *It is a relatively aggressive malignancy, characterized by marked lymphocytosis and involving peripheral blood, liver, spleen and bone marrow.
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| *Factors playing role in the parthenogenesis of these cells are:
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| **Genetic factors:<ref name="pmid1913594">{{cite journal |vauthors=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 |date=August 1991 |pmid=1913594 |doi= |url=}}</ref><ref name="pmid11920168">{{cite journal |vauthors=Hetet G, Dastot H, Baens M, Brizard A, Sigaux F, Grandchamp B, Stern MH |title=Recurrent molecular deletion of the 12p13 region, centromeric to ETV6/TEL, in T-cell prolymphocytic leukemia |journal=Hematol. J. |volume=1 |issue=1 |pages=42–7 |date=2000 |pmid=11920168 |doi=10.1038/sj/thj/6200008 |url=}}</ref><ref name="pmid10930996">{{cite journal |vauthors=Brito-Babapulle V, Hamoudi R, Matutes E, Watson S, Kaczmarek P, Maljaie H, Catovsky D |title=p53 allele deletion and protein accumulation occurs in the absence of p53 gene mutation in T-prolymphocytic leukaemia and Sezary syndrome |journal=Br. J. Haematol. |volume=110 |issue=1 |pages=180–7 |date=July 2000 |pmid=10930996 |doi= |url=}}</ref><ref name="pmid14580769">{{cite journal |vauthors=Costa D, Queralt R, Aymerich M, Carrió A, Rozman M, Vallespí T, Colomer D, Nomdedeu B, Montserrat E, Campo E |title=High levels of chromosomal imbalances in typical and small-cell variants of T-cell prolymphocytic leukemia |journal=Cancer Genet. Cytogenet. |volume=147 |issue=1 |pages=36–43 |date=November 2003 |pmid=14580769 |doi= |url=}}</ref>
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| ***Mutations in chromosome 14
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| ***Mutations in chromosome 8
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| ***Mutations in tumor suppressor gene 11q23, at the ataxia telangiectasia mutated (ATM) locus
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| ***Inversion of chromosome 14 (14q11)
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| ***TCR gene rearrangements for the γ and δ chains
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| *
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|
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| {{familytree/start}}
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| {{familytree | | | | | | | | | A01 | | | |A01='''Factors Involved in the [[Pathophysiology]] of [[T-cell Pro-Lymphocytic Leukemia]]'''}}
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| {{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }}
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| {{familytree | | C01 | | | | | | | | | | | | C02 |C01='''[[Genetic Factors]]'''|C02= '''Association with different [[Immunophenotypes]]''' }}
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| {{familytree | | |!| | | | | | | | | | | | | |!| }}
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| {{familytree|boxstyle=text-align: left| | D01 | | | | | | | | | | | | D02 |D01=Different types of genetic abnormalities are as follows, mostly involve chromosme 14:<ref name="pmid1913594">{{cite journal |vauthors=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 |date=August 1991 |pmid=1913594 |doi= |url=}}</ref><ref name="pmid9614908">{{cite journal |vauthors=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 |date=June 1998 |pmid=9614908 |doi= |url=}}</ref><ref name="pmid10077617">{{cite journal |vauthors=Pekarsky Y, Hallas C, Isobe M, Russo G, Croce CM |title=Abnormalities at 14q32.1 in T cell malignancies involve two oncogenes |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=96 |issue=6 |pages=2949–51 |date=March 1999 |pmid=10077617 |pmc=15875 |doi= |url=}}</ref><ref name="pmid8361760">{{cite journal |vauthors=Stern MH, Soulier J, Rosenzwajg M, Nakahara K, Canki-Klain N, Aurias A, Sigaux F, Kirsch IR |title=MTCP-1: a novel gene on the human chromosome Xq28 translocated to the T cell receptor alpha/delta locus in mature T cell proliferations |journal=Oncogene |volume=8 |issue=9 |pages=2475–83 |date=September 1993 |pmid=8361760 |doi= |url=}}</ref><ref name="pmid28194886">{{cite journal |vauthors=Hu Z, Medeiros LJ, Fang L, Sun Y, Tang Z, Tang G, Sun T, Quesada AE, Hu S, Wang SA, Pei L, Lu X |title=Prognostic significance of cytogenetic abnormalities in T-cell prolymphocytic leukemia |journal=Am. J. Hematol. |volume=92 |issue=5 |pages=441–447 |date=May 2017 |pmid=28194886 |doi=10.1002/ajh.24679 |url=}}</ref>
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| *Inv(14)
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| *t(14;14)(q11;q32)
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| *t(X;14)(q28;q11) which involves a homolog of TCL1, MTCP1 (mature T cell proliferation 1 gene)
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| Chromosome 8 defects:<ref name="pmid10077617">{{cite journal |vauthors=Pekarsky Y, Hallas C, Isobe M, Russo G, Croce CM |title=Abnormalities at 14q32.1 in T cell malignancies involve two oncogenes |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=96 |issue=6 |pages=2949–51 |date=March 1999 |pmid=10077617 |pmc=15875 |doi= |url=}}</ref>
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| *idic(8p11)
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| *t(8;8)
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| *Trisomy 8q
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| *Del(12p13)
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| *Abnormalities in chromosome 17
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| *Deletions in chromosome 6
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| *Deletion of TP53 gene<ref name="pmid1913594">{{cite journal |vauthors=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 |date=August 1991 |pmid=1913594 |doi= |url=}}</ref><ref name="pmid11920168">{{cite journal |vauthors=Hetet G, Dastot H, Baens M, Brizard A, Sigaux F, Grandchamp B, Stern MH |title=Recurrent molecular deletion of the 12p13 region, centromeric to ETV6/TEL, in T-cell prolymphocytic leukemia |journal=Hematol. J. |volume=1 |issue=1 |pages=42–7 |date=2000 |pmid=11920168 |doi=10.1038/sj/thj/6200008 |url=}}</ref><ref name="pmid10930996">{{cite journal |vauthors=Brito-Babapulle V, Hamoudi R, Matutes E, Watson S, Kaczmarek P, Maljaie H, Catovsky D |title=p53 allele deletion and protein accumulation occurs in the absence of p53 gene mutation in T-prolymphocytic leukaemia and Sezary syndrome |journal=Br. J. Haematol. |volume=110 |issue=1 |pages=180–7 |date=July 2000 |pmid=10930996 |doi= |url=}}</ref><ref name="pmid14580769">{{cite journal |vauthors=Costa D, Queralt R, Aymerich M, Carrió A, Rozman M, Vallespí T, Colomer D, Nomdedeu B, Montserrat E, Campo E |title=High levels of chromosomal imbalances in typical and small-cell variants of T-cell prolymphocytic leukemia |journal=Cancer Genet. Cytogenet. |volume=147 |issue=1 |pages=36–43 |date=November 2003 |pmid=14580769 |doi= |url=}}</ref>
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| *Activating mutation in Tyrosine kinase of Jak3<ref name="pmid24446122">{{cite journal |vauthors=Bergmann AK, Schneppenheim S, Seifert M, Betts MJ, Haake A, Lopez C, Maria Murga Penas E, Vater I, Jayne S, Dyer MJ, Schrappe M, Dührsen U, Ammerpohl O, Russell RB, Küppers R, Dürig J, Siebert R |title=Recurrent mutation of JAK3 in T-cell prolymphocytic leukemia |journal=Genes Chromosomes Cancer |volume=53 |issue=4 |pages=309–16 |date=April 2014 |pmid=24446122 |doi=10.1002/gcc.22141 |url=}}</ref><ref name="pmid24825865">{{cite journal |vauthors=Kiel MJ, Velusamy T, Rolland D, Sahasrabuddhe AA, Chung F, Bailey NG, Schrader A, Li B, Li JZ, Ozel AB, Betz BL, Miranda RN, Medeiros LJ, Zhao L, Herling M, Lim MS, Elenitoba-Johnson KS |title=Integrated genomic sequencing reveals mutational landscape of T-cell prolymphocytic leukemia |journal=Blood |volume=124 |issue=9 |pages=1460–72 |date=August 2014 |pmid=24825865 |pmc=4148768 |doi=10.1182/blood-2014-03-559542 |url=}}</ref>
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| *Deletions or missense mutations at the ataxia telangiectasia mutated (ATM) locus 11q23 <ref name="pmid9334731">{{cite journal |vauthors=Stilgenbauer S, Schaffner C, Litterst A, Liebisch P, Gilad S, Bar-Shira A, James MR, Lichter P, Döhner H |title=Biallelic mutations in the ATM gene in T-prolymphocytic leukemia |journal=Nat. Med. |volume=3 |issue=10 |pages=1155–9 |date=October 1997 |pmid=9334731 |doi= |url=}}</ref><ref name="pmid9288106">{{cite journal |vauthors=Vorechovský I, Luo L, Dyer MJ, Catovsky D, Amlot PL, Yaxley JC, Foroni L, Hammarström L, Webster AD, Yuille MA |title=Clustering of missense mutations in the ataxia-telangiectasia gene in a sporadic T-cell leukaemia |journal=Nat. Genet. |volume=17 |issue=1 |pages=96–9 |date=September 1997 |pmid=9288106 |doi=10.1038/ng0997-96 |url=}}</ref> |D02= [[T-cell prolymphocytic leukemia]] cells express different [[markers]] including:
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| *[[CD52]](strong association)
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| '''Pan-T cell markers such as''':
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| *[[CD2]]
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| *[[CD3]](might be low or high level)
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| *[[CD7]]
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| *Oncogene TCL1
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| *[[CD4+/CD8-]](present in 60% of cases)
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| *[[CD4+/CD8+]](present in 25%, unique for T-cell prolymphocytic leukemia)
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| *[[CD4-/CD8+]](15% of cases)
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| *Negative terminal deoxynucleotidyl transferase (TdT)}}
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| {{familytree/end}}
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|
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|
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| *On gross pathology, characteristic findings of T-cell prolymphocytic leukemia, include:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| :*No remarkable findings
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| *On microscopic histopathological analysis, characteristic findings of T-cell prolymphocytic leukemia, include:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| :*The immunophenotype CD4+/CD8- (present in 60% of cases)
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| :*The immunophenotype CD4+/CD8+ (present in 25%)
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|
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| :* The immunophenotype CD4-/CD8+ (15% of cases) Different variants of T-cell Lymphocytic leukemias can be differentiated based on microscopic and gross finding.<ref name="pmid17424862">{{cite journal |vauthors=Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D |title=Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia |journal=Blood |volume=78 |issue=12 |pages=3269–74 |date=December 1991 |pmid=1742486 |doi= |url=}}</ref><ref name="pmid16342661">{{cite journal |vauthors=Ravandi F, O'Brien S |title=Chronic lymphoid leukemias other than chronic lymphocytic leukemia: diagnosis and treatment |journal=Mayo Clin. Proc. |volume=80 |issue=12 |pages=1660–74 |date=December 2005 |pmid=16342661 |doi=10.4065/80.12.1660 |url=}}</ref>
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|
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| {| align="center"
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| ! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Morphological Variant
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| ! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gross Findings
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| ! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Microscopic Findings
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| |-
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| ! colspan="1" align="center" style="background:#DCDCDC;" |Typical T-cell
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| prolymphocytic
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|
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| leukemia
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|
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| | colspan="1" style="background:#DCDCDC;" |
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| | colspan="1" style="background:#DCDCDC;" |
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| * Medium-sized lymphoid cells
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| *Densely packed chromatin
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| *Prominent nucleolus with blisters on surface
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| |-
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| | colspan="1" align="center" style="background:#DCDCDC;" |'''Small cell variant'''
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| | colspan="1" style="background:#DCDCDC;" |
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| | colspan="1" style="background:#DCDCDC;" |
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| *Small cells
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| *Dense chromatin
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| *Minute electron microscopically visible nucleolus
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| |-
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| ! colspan="1" align="center" style="background:#DCDCDC;" |Cerebriform
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| (Sézary cell-like)
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|
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| variant
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| | colspan="1" style="background:#DCDCDC;" |
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| | colspan="1" style="background:#DCDCDC;" |
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| *Asymmetrical nuclear outline
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| *Cerebriform nucleus
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| |}
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|
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| ==Causes==
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| * Common causes of T-cell prolymphocytic leukemia, include:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| :*Genetic mutations (e.g. Trisomy 8, chromosomal abnormalities)
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|
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| ==Differentiating T-cell Prolymphocytic Leukemia from Other Diseases==
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| *T-cell prolymphocytic leukemia must be differentiated from other diseases that cause [[lymphadenopathy]], [[hepatomegaly]], and [[fever]], such as:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| :*[[Sézary syndrome]]
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| :*[[Cutaneous T cell lymphoma]]
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| :*Angioimmunoblastic T cell lymphoma
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| :*[[B-cell prolymphocytic leukemia]]
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|
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| ==Epidemiology and Demographics==
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| * T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.
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| * The incidence of T-cell prolymphocytic leukemia increases with age; the median age at diagnosis is 65 years.<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| *Patients with ataxia telangiectasia and T-cell prolymphocytic leukemia are young adults; the median age at diagnosis is 30 years.
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|
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| *Males are slightly more affected with T-cell prolymphocytic leukemia than females.
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|
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| *There is no racial predilection for T-cell prolymphocytic leukemia.
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|
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| ==Risk Factors==
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| *There are no risk factors associated with the development of T-cell prolymphocytic leukemia.<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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|
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| == Screening==
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| There is insufficient evidence to recommend routine screening for T-cell prolymphocytic leukemia.
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|
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| == Natural History, Complications and Prognosis== | | ==[[T-cell prolymphocytic leukemia overview|Overview]]== |
| *The majority of patients with T-cell prolymphocytic leukemia are symptomatic at the time of diagnosis.
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| *Early clinical features include fever, fatigue, and lymphadenopathy.
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| *If left untreated, patients with T-cell prolymphocytic leukemia may progress to develop multiple organ failure.
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| *Common complications of T-cell prolymphocytic leukemia, include:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| :*[[Graft-versus-host disease]] (allogeneic transplant)
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| :*[[Infection|Infections]]
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| :*[[Bleeding : Overview|Bleeding]]
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| *Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately one to two years.<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| *Patients with CD45RO+/CD45RA- immunophenotype tend to have a more indolent course.
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| *It seems following factors are associated with worse prognosis:
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| **Increased expression of TCL1
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| **Increased activity of the serine-threonine kinase AKT
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|
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|
| == Diagnosis == | | ==[[T-cell prolymphocytic leukemia historical perspective|Historical Perspective]]== |
| === Diagnostic Study of Choice ===
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| * There are no established criteria for the diagnosis of T-cell prolymphocytic leukemia. Patients with T-cell prolymphocytic leukemia are diagnosed by clinical presentation, pathology evaluation of the peripheral blood and bone marrow. Flow cytometry and immunostains should be performed to diagnose a T cell immunophenotype.
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|
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| === History and Symptoms === | | ==[[T-cell prolymphocytic leukemia classification|Classification]]== |
| *Symptoms of T-cell prolymphocytic leukemia may include the following:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref><ref name="pmid283408783">{{cite journal |vauthors=Sud A, Dearden C |title=T-cell Prolymphocytic Leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=31 |issue=2 |pages=273–283 |date=April 2017 |pmid=28340878 |doi=10.1016/j.hoc.2016.11.010 |url=}}</ref>
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| :*[[Fever]]
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| :*[[Weight loss]]
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| :*[[Night sweats]]
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|
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|
| === Physical Examination === | | ==[[T-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== |
| *Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
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| *Physical examination may be remarkable for:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
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| :*[[Hepatomegaly]]
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| :*[[Splenomegaly]]
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| :*[[Generalized lymphadenopathy]]
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| :*Skin infiltration
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| :*Serous effusions:
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| :**[[Pleural effusion]]
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| :**[[Peritoneum|Peritoneal]] effusion
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| :*Central nervous system involvement (very rare)
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| === Laboratory Findings ===
| |
| *Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref>
| |
| :*High [[lymphocyte]] count (> 100 x 109/L)
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| :*[[Anemia]]
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| :*[[Thrombocytopenia]]
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| :*Negative [[Human T-lymphotropic virus|human T lymphotropic virus]] ([[Human T-lymphotropic virus|HTLV]]) serology
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| :*Peripheral Blood Smear demonstrated predominance of [[lymphocytes]]:
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| :**Typical variant:
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| :*** Medium-sized [[lymphocytes]]
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| :*** Condensed chromatin and a visible nucleolus
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| :*** Round nucleus
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| :*** Slightly basophilic cytoplasm
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| :*** Cytoplasmic protrusion
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| :**Small cell variant
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| :*** Small tumor cells with condensed chromatin
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| :*** Small nucleolus visible by electron microscopy
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| :**Cerebriform (Sézary cell-like) variant
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| :*** Irregular nuclear outline
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| :*** Similar to cerebriform nucleus of Sézary cells seen in mycosis fungoides
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|
| |
|
| ===Electrocardiogram=== | | ==[[T-cell prolymphocytic leukemia causes|Causes]]== |
| There are no ECG findings associated with T-cell prolymphocytic leukemia.
| |
|
| |
|
| ===X-ray=== | | ==[[T-cell prolymphocytic leukemia differential diagnosis|Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases]]== |
| There are no x-ray findings associated with T-cell prolymphocytic leukemia. However, an x-ray may be helpful in the diagnosis of complications of T-cell prolymphocytic leukemia, which include pleural effusion and lung involvement.
| |
|
| |
|
| ===Echocardiography or Ultrasound=== | | ==[[T-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]== |
| There are no echocardiography findings associated with T-cell prolymphocytic leukemia.
| |
|
| |
|
| Ultrasound may be helpful in the diagnosis of T-cell prolymphocytic leukemia. Findings on an ultrasound suggestive of/diagnostic of T-cell prolymphocytic leukemia include hepatomegaly and splenomegaly.
| | ==[[T-cell prolymphocytic leukemia risk factors|Risk Factors]]== |
|
| |
|
| ===CT scan=== | | ==[[T-cell prolymphocytic leukemia screening|Screening]]== |
| CT scan may be helpful in the diagnosis of T-cell prolymphocytic leukemia. Findings on an CT scan suggestive of/diagnostic of T-cell prolymphocytic leukemia include hepatomegaly and splenomegaly.
| |
|
| |
|
| ===MRI=== | | ==[[T-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| There are no MRI findings associated with T-cell prolymphocytic leukemia.
| |
|
| |
|
| ===Other Imaging Findings=== | | ==T-cell prolymphocytic leukemia Diagnosis== |
| There are no specific imaging findings associated with T-cell prolymphocytic leukemia.<ref name="pmid23382603" />
| | [[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]] |
|
| |
|
| === Other Diagnostic Studies === | | ==Treatment== |
| Flow cytometry and immunohistopathology must be done to diagnose T-cell prolymphocytic leukemia.
| | [[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]] |
|
| |
|
| == Treatment == | | ==Case Studies== |
| === Medical Therapy ===
| | [[T-cell-prolymphocytic leukemia case study one|Case #1]] |
| *The mainstay of therapy for T-cell prolymphocytic leukemia, include:<ref name="pmid23382603">{{cite journal |vauthors=Graham RL, Cooper B, Krause JR |title=T-cell prolymphocytic leukemia |journal=Proc (Bayl Univ Med Cent) |volume=26 |issue=1 |pages=19–21 |year=2013 |pmid=23382603 |pmc=3523759 |doi= |url=}}</ref><ref name="pmid17392661">{{cite journal |vauthors=Robak T, Robak P |title=Current treatment options in prolymphocytic leukemia |journal=Med. Sci. Monit. |volume=13 |issue=4 |pages=RA69–80 |date=April 2007 |pmid=17392661 |doi= |url=}}</ref>
| |
| :*[[Alemtuzumab]] (anti-CD52)
| |
| *T-cell prolymphocytic leukemia is often resistant to therapy.
| |
| === Surgery ===
| |
| *Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission.
| |
| === Primary Prevention ===
| |
| *There are no established measures for the primary prevention of T-cell prolymphocytic leukemia.
| |
|
| |
|
| === Secondary Prevention ===
| | [[Category: (name of the system)]] |
| * There are no established measures for the secondary prevention of T-cell prolymphocytic leukemia.
| |
|
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|
| ==References==
| |
| {{Reflist|2}} | | {{Reflist|2}} |
| [[Category:Up-To-Date]] | | [[Category:Up-To-Date]] |
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| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Immunology]] | | [[Category:Immunology]] |
| [[Category:Primary care]]
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