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| ==[[T-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | | ==[[T-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==[[T-cell prolymphocytic leukemia Diagnosis|T-cell prolymphocytic leukemia Diagnosis Diagnosis]]== | | ==T-cell prolymphocytic leukemia Diagnosis== |
| [[T-cell prolymphocytic leukemia overview diagnostic study of choice|Diagnostic study of choice]] | [[T-cell prolymphocytic leukemia overview history and symptoms|History and Symptoms]] | [[T-cell prolymphocytic leukemia overview physical examination|Physical Examination]] | [[T-cell prolymphocytic leukemia overview laboratory findings|Laboratory Findings]] | [[T-cell prolymphocytic leukemia overview electrocardiogram|Electrocardiogram]] | [[T-cell prolymphocytic leukemia overview x ray|X-Ray Findings]] | [[T-cell prolymphocytic leukemia overview echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[T-cell prolymphocytic leukemia overview CT scan|CT-Scan Findings]] | [[T-cell prolymphocytic leukemia overview MRI|MRI Findings]] | [[T-cell prolymphocytic leukemia overview other imaging findings|Other Imaging Findings]] | [[T-cell prolymphocytic leukemia other diagnostic studies|Other Diagnostic Studies]] | | [[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]] |
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| ==Treatment== | | ==Treatment== |
| [[T-cell prolymphocytic leukemia medical therapy|Medical Therapy]] | [[T-cell prolymphocytic leukemia overview interventions|Interventions]] | [[T-cell prolymphocytic leukemia overview surgery|Surgery]] | [[T-cell prolymphocytic leukemia overview primary prevention|Primary Prevention]] | [[T-cell prolymphocytic leukemia overview secondary prevention|Secondary Prevention]] | [[T-cell prolymphocytic leukemia overview cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[T-cell prolymphocytic leukemia overview future or investigational therapies|Future or Investigational Therapies]] | | [[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]] |
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| ==Case Studies== | | ==Case Studies== |
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| [[Category: (name of the system)]] | | [[Category: (name of the system)]] |
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| ==Causes==
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| * Common causes of T-cell prolymphocytic leukemia, include genetic factors and chromosomal abnormalities:<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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| :*Trisomy 8, chromosomal abnormalities
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| :*Ataxia telangiectasia (ATM) gene mutation
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| :*TP53 gene mutation
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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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| *Males are slightly more affected with T-cell prolymphocytic leukemia than females.
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| *There is no racial predilection for T-cell prolymphocytic leukemia.
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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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| == Screening==
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| There is insufficient evidence to recommend routine screening for T-cell prolymphocytic leukemia.
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| == Natural History, Complications and Prognosis==
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| *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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| == Diagnosis ==
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| === 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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| === History and Symptoms ===
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| *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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| === Physical Examination ===
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| *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 ===
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| *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>
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| :*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===
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| There are no ECG findings associated with T-cell prolymphocytic leukemia.
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| ===X-ray===
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| 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.
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| ===Echocardiography or Ultrasound===
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| There are no echocardiography findings associated with T-cell prolymphocytic leukemia.
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| 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.
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| ===CT scan===
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| 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.
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| ===MRI===
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| There are no MRI findings associated with T-cell prolymphocytic leukemia.
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| ===Other Imaging Findings===
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| There are no specific imaging findings associated with T-cell prolymphocytic leukemia.<ref name="pmid23382603" />
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| === Other Diagnostic Studies ===
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| Flow cytometry and immunohistopathology must be done to diagnose T-cell prolymphocytic leukemia.
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| == Treatment ==
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| === Medical Therapy ===
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| *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>
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| :*[[Alemtuzumab]] (anti-CD52)
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| *T-cell prolymphocytic leukemia is often resistant to therapy.
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| === Surgery ===
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| *Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission.
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| === Primary Prevention ===
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| *There are no established measures for the primary prevention of T-cell prolymphocytic leukemia.
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| === Secondary Prevention ===
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| * There are no established measures for the secondary prevention of T-cell prolymphocytic leukemia.
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| ==References==
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| {{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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