T-cell prolymphocytic leukemia: Difference between revisions

	T-cell prolymphocytic leukemia Microchapters
Home
Patient Information
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
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
T-cell prolymphocytic leukemia On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of T-cell prolymphocytic leukemia All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on T-cell prolymphocytic leukemia
CDC on T-cell prolymphocytic leukemia
T-cell prolymphocytic leukemia in the news
Blogs on T-cell prolymphocytic leukemia
Directions to Hospitals Treating Psoriasis
Risk calculators and risk factors for T-cell prolymphocytic leukemia

VisualWikitext

Revision as of 16:38, 6 March 2019

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

Differentiating T-cell-prolymphocytic leukemia overview from other Diseases

Natural History, Complications and Prognosis

Diagnosis

Treatment

Case Studies

Case #1

Overview

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.

Historical Perspective

40 years ago, in 1973, Catovsky first described four cases of T-cell prolymphocytic leukemia.^[1]^[2]
In 1994, Harris a pathologist from Boston and his colleagues made an effort to classify T-cell prolymphocytic leukemia.^[3]

Classification

T-cell prolymphocytic leukemia classification is based on its morphology. ^[4]^[5]^[6]^[7]

Morphological Variant	Percentage of total number
Typical T-cell prolymphocytic leukemia	75 percent
Small cell variant	20 percent
Cerebriform (Sézary cell-like) variant	5 percent

Causes

Common causes of T-cell prolymphocytic leukemia, include genetic factors and chromosomal abnormalities:^[4]

Trisomy 8, chromosomal abnormalities
Ataxia telangiectasia (ATM) gene mutation
TP53 gene mutation

Differentiating T-cell Prolymphocytic Leukemia from Other Diseases

T-cell prolymphocytic leukemia must be differentiated from other diseases that cause lymphadenopathy, hepatomegaly, and fever, such as:^[4]

	Symptoms			Physical examination			Lab Findings			Imaging			Histopathology
Diseases	Clinical manifestations						Para-clinical findings							Gold standard	Additional findings
	Symptoms			Physical examination			Para-clinical findings
	Symptom 1	Symptom 2	Symptom 3	Physical exam 1	Physical exam 2	Physical exam 3	Lab 1	Lab 2	Lab 3	Imaging 1	Imaging 2	Imaging 3	Histopathology
Sézary syndrome
Cutaneous T cell lymphoma ma
Angioimmunoblastic T cell lymphoma

Epidemiology and Demographics

T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.
The incidence of T-cell prolymphocytic leukemia increases with age; the median age at diagnosis is 65 years.^[4]
Patients with ataxia telangiectasia and T-cell prolymphocytic leukemia are young adults; the median age at diagnosis is 30 years.

Males are slightly more affected with T-cell prolymphocytic leukemia than females.

There is no racial predilection for T-cell prolymphocytic leukemia.

Risk Factors

There are no risk factors associated with the development of T-cell prolymphocytic leukemia.^[4]

Screening

There is insufficient evidence to recommend routine screening for T-cell prolymphocytic leukemia.

Natural History, Complications and Prognosis

The majority of patients with T-cell prolymphocytic leukemia are symptomatic at the time of diagnosis.
Early clinical features include fever, fatigue, and lymphadenopathy.
If left untreated, patients with T-cell prolymphocytic leukemia may progress to develop multiple organ failure.
Common complications of T-cell prolymphocytic leukemia, include:^[4]

Graft-versus-host disease (allogeneic transplant)
Infections
Bleeding

Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately one to two years.^[4]
Patients with CD45RO+/CD45RA- immunophenotype tend to have a more indolent course.
It seems following factors are associated with worse prognosis:
- Increased expression of TCL1
- Increased activity of the serine-threonine kinase AKT

Diagnosis

Diagnostic Study of Choice

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.

History and Symptoms

Symptoms of T-cell prolymphocytic leukemia may include the following:^[4]^[8]

Physical Examination

Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
Physical examination may be remarkable for:^[4]

Hepatomegaly
Splenomegaly
Generalized lymphadenopathy
Skin infiltration
Serous effusions:
- Pleural effusion
- Peritoneal effusion
Central nervous system involvement (very rare)

Laboratory Findings

Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include:^[4]

High lymphocyte count (> 100 x 109/L)
Anemia
Thrombocytopenia
Negative human T lymphotropic virus (HTLV) serology
Peripheral Blood Smear demonstrated predominance of lymphocytes:
- Typical variant:
  - Medium-sized lymphocytes
  - Condensed chromatin and a visible nucleolus
  - Round nucleus
  - Slightly basophilic cytoplasm
  - Cytoplasmic protrusion
- Small cell variant
  - Small tumor cells with condensed chromatin
  - Small nucleolus visible by electron microscopy
- Cerebriform (Sézary cell-like) variant
  - Irregular nuclear outline
  - Similar to cerebriform nucleus of Sézary cells seen in mycosis fungoides

Electrocardiogram

There are no ECG findings associated with T-cell prolymphocytic leukemia.

X-ray

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

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.

CT scan

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

There are no MRI findings associated with T-cell prolymphocytic leukemia.

Other Imaging Findings

There are no specific imaging findings associated with T-cell prolymphocytic leukemia.^[4]

Other Diagnostic Studies

Flow cytometry and immunohistopathology must be done to diagnose T-cell prolymphocytic leukemia.

Treatment

Medical Therapy

The mainstay of therapy for T-cell prolymphocytic leukemia, include:^[4]^[9]

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

There are no established measures for the secondary prevention of T-cell prolymphocytic leukemia.

References

↑ Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G (August 1973). "Prolymphocytic leukaemia of B and T cell type". Lancet. 2 (7823): 232–4. PMID 4124423.
↑ Sud A, Dearden C (April 2017). "T-cell Prolymphocytic Leukemia". Hematol. Oncol. Clin. North Am. 31 (2): 273–283. doi:10.1016/j.hoc.2016.11.010. PMID 28340878.
↑ Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC (September 1994). "A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group". Blood. 84 (5): 1361–92. PMID 8068936.
↑ ^4.00 ^4.01 ^4.02 ^4.03 ^4.04 ^4.05 ^4.06 ^4.07 ^4.08 ^4.09 ^4.10 ^4.11 Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.
↑ Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES (May 2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
↑ Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D (December 1991). "Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia". Blood. 78 (12): 3269–74. PMID 1742486.
↑ Foucar K (April 2007). "Mature T-cell leukemias including T-prolymphocytic leukemia, adult T-cell leukemia/lymphoma, and Sézary syndrome". Am. J. Clin. Pathol. 127 (4): 496–510. doi:10.1309/KWJYBCCGTB90B6AE. PMID 17369126.
↑ Sud A, Dearden C (April 2017). "T-cell Prolymphocytic Leukemia". Hematol. Oncol. Clin. North Am. 31 (2): 273–283. doi:10.1016/j.hoc.2016.11.010. PMID 28340878.
↑ Robak T, Robak P (April 2007). "Current treatment options in prolymphocytic leukemia". Med. Sci. Monit. 13 (4): RA69–80. PMID 17392661.

[pmid4124423-1] Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G (August 1973). "Prolymphocytic leukaemia of B and T cell type". Lancet. 2 (7823): 232–4. PMID 4124423.

[pmid28340878-2] Sud A, Dearden C (April 2017). "T-cell Prolymphocytic Leukemia". Hematol. Oncol. Clin. North Am. 31 (2): 273–283. doi:10.1016/j.hoc.2016.11.010. PMID 28340878.

[pmid8068936-3] Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC (September 1994). "A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group". Blood. 84 (5): 1361–92. PMID 8068936.

[pmid23382603-4] 4.00 ^4.01 ^4.02 ^4.03 ^4.04 ^4.05 ^4.06 ^4.07 ^4.08 ^4.09 ^4.10 ^4.11 Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.

[pmid26980727-5] Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES (May 2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.

[pmid1742486-6] Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D (December 1991). "Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia". Blood. 78 (12): 3269–74. PMID 1742486.

[pmid17369126-7] Foucar K (April 2007). "Mature T-cell leukemias including T-prolymphocytic leukemia, adult T-cell leukemia/lymphoma, and Sézary syndrome". Am. J. Clin. Pathol. 127 (4): 496–510. doi:10.1309/KWJYBCCGTB90B6AE. PMID 17369126.

[pmid283408783-8] Sud A, Dearden C (April 2017). "T-cell Prolymphocytic Leukemia". Hematol. Oncol. Clin. North Am. 31 (2): 273–283. doi:10.1016/j.hoc.2016.11.010. PMID 28340878.

[pmid17392661-9] Robak T, Robak P (April 2007). "Current treatment options in prolymphocytic leukemia". Med. Sci. Monit. 13 (4): RA69–80. PMID 17392661.

[1]

[2]

[3]

[4]

[5]

[6]

[7]

[8]

[9]

@@ Line 59: / Line 59: @@
 ! colspan="1" align="center" style="background:#DCDCDC;" |Cerebriform (Sézary cell-like) variant
 ! colspan="1" align="center" style="background:#DCDCDC;" |5 percent
-|}
-==Pathophysiology==
-*T-cell prolymphocytic leukemia is a uncommon T- cell malignancy.
-*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>
-*It is a relatively aggressive malignancy, characterized by marked lymphocytosis and involving peripheral blood, liver, spleen and bone marrow.<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>
-*Factors playing role in the parthenogenesis of these cells are:
-**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>
-***Mutations in chromosome 14
-***Mutations in chromosome 8
-***Mutations in tumor suppressor gene 11q23, at the ataxia telangiectasia mutated (ATM) locus
-***Inversion of chromosome 14 (14q11)
-***TCR gene rearrangements for the γ and δ chains
-*
-{{familytree/start}}
-{{familytree | | | | | | | | | A01 | | | |A01='''Factors Involved in the [[Pathophysiology]] of [[T-cell Pro-Lymphocytic Leukemia]]'''}}
-{{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }}
-{{familytree | | C01 | | | | | | | | | | | | C02 |C01='''[[Genetic Factors]]'''|C02= '''Association with different [[Immunophenotypes]]''' }}
-{{familytree | | |!| | | | | | | | | | | | | |!| }}
-{{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>
-*Inv(14)
-*t(14;14)(q11;q32)
-*t(X;14)(q28;q11) which involves a homolog of TCL1, MTCP1 (mature T cell proliferation 1 gene)
-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>
-*idic(8p11)
-*t(8;8)
-*Trisomy 8q
-*Del(12p13)
-*Abnormalities in chromosome 17
-*Deletions in chromosome 6
-*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>
-*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>
-*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:
-*[[CD52]](strong association)
-'''Pan-T cell markers such as''':
-*[[CD2]]
-*[[CD3]](might be low or high level)
-*[[CD7]]
-*Oncogene TCL1
-*[[CD4+/CD8-]](present in 60% of cases)
-*[[CD4+/CD8+]](present in 25%, unique for T-cell prolymphocytic leukemia)
-*[[CD4-/CD8+]](15% of cases)
-*Negative terminal deoxynucleotidyl transferase (TdT)}}
-{{familytree/end}}
-*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>
-:*No remarkable findings
-*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>
-:*The immunophenotype CD4+/CD8- (present in 60% of cases)
-:*The immunophenotype  CD4+/CD8+ (present in 25%)
-:* 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>
-{| align="center"
-! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Morphological Variant
-! rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Microscopic Findings
-|-
-! colspan="1" align="center" style="background:#DCDCDC;" |Typical T-cell
-prolymphocytic
-leukemia
-| colspan="1" style="background:#DCDCDC;" |
-* Medium-sized lymphoid cells
-*Densely packed [[chromatin]]
-*Prominent [[nucleolus]] with [[Blister|blisters]] on surface
-|-
-| colspan="1" align="center" style="background:#DCDCDC;" |'''Small cell variant'''
-| colspan="1" style="background:#DCDCDC;" |
-*Small cells
-*Dense [[chromatin]]
-*Minute electron microscopically visible nucleolus
-|-
-! colspan="1" align="center" style="background:#DCDCDC;" |Cerebriform
-(Sézary cell-like)
-variant
-| colspan="1" style="background:#DCDCDC;" |
-*Asymmetrical [[Cell nucleus|nuclear]] outline
-*Cerebriform [[Cell nucleus|nucleus]]
 |}

T-cell prolymphocytic leukemia: Difference between revisions

Revision as of 16:38, 6 March 2019

Differentiating T-cell-prolymphocytic leukemia overview from other Diseases

Diagnosis

Treatment

Case Studies

Overview

Historical Perspective

Classification

Causes

Differentiating T-cell Prolymphocytic Leukemia from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

Navigation menu