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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{T-cell prolymphocytic leukemia}} |
| {{CMG}} {{AE}} {{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.<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-cell prolymphocytic leukemia was first described by Catovsky in 1973.<ref name="wiki">Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G. Prolymphocytic leukaemia of B and T cell type. Lancet </ref> 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.<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> 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.<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 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.<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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| ==Historical Perspective== | | ==[[T-cell prolymphocytic leukemia overview|Overview]]== |
| *T-cell prolymphocytic leukemia was first described by Catovsky in 1973.<ref name="wiki">Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G. Prolymphocytic leukaemia of B and T cell type. Lancet </ref>
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| ==Classification== | | ==[[T-cell prolymphocytic leukemia historical perspective|Historical Perspective]]== |
| *There is no classification system for 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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| ==Pathophysiology== | | ==[[T-cell prolymphocytic leukemia classification|Classification]]== |
| *T-cell prolymphocytic leukemia arises from mature (post-thymic) T-cell, which is normally involved in in cell-mediated immunity.
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| *The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia.
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| *Patients with T-cell prolymphocytic leukemia have TCR gene rearrangements for the γ and δ chains.
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| *Mutations of chromosome 8 are seen approximately 75% of patients.
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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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| :*The immunophenotype CD4-/CD8+ (15% of cases)
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| '''Pan-T antigens'''
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| :*[[CD2]] negative
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| :*[[CD3]] negative
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| :*[[CD7]] negative
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| :*TdT positive
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| :*[[CD1a]] positive
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| ==Causes== | | ==[[T-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== |
| * 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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| ==Differentiating T-cell Prolymphocytic Leukemia from Other Diseases== | | ==[[T-cell prolymphocytic leukemia causes|Causes]]== |
| *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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| ==Epidemiology and Demographics== | | ==[[T-cell prolymphocytic leukemia differential diagnosis|Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases]]== |
| * T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.
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| ===Age===
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| *T-cell prolymphocytic leukemia is more commonly observed among patients aged between 30 to 40 years old.<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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| *T-cell prolymphocytic leukemia is more commonly observed among young adults.
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| ===Gender===
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| *Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females.
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| ===Race=== | | ==[[T-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]== |
| *There is no racial predilection for T-cell prolymphocytic leukemia.
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| ==Risk Factors== | | ==[[T-cell prolymphocytic leukemia risk factors|Risk Factors]]== |
| *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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| == 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 7 months.<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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| == Diagnosis == | | ==[[T-cell prolymphocytic leukemia screening|Screening]]== |
| === 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>
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| :*[[Fever]]
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| :*[[Weight loss]]
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| :*[[Night sweats]]
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| === Physical Examination === | | ==[[T-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| *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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| '''Peripheral Blood Smear'''
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| :*Medium-sized lymphocytes
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| :*Single nucleoli and basophilic cytoplasm
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| :*The nuclei are usually round to oval in shape,
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| :*Irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome.
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| :*A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases
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| === Laboratory Findings === | | ==T-cell prolymphocytic leukemia Diagnosis== |
| *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>
| | [[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]] |
| :*High lymphocyte count (> 100 x 109/L)
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| :*[[Anemia]]
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| :*[[Thrombocytopenia]]
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| :*Negative HTLV-1 serology
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| ===Imaging Findings=== | | ==Treatment== |
| *There are no specific imaging findings associated with 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>
| | [[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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| == 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>
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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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| === Prevention ===
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| *There are no primary preventive measures available for T-cell prolymphocytic leukemia.
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| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[T-cell-prolymphocytic leukemia case study one|Case #1]] |
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| | [[Category: (name of the system)]] |
| {{Hematological malignancy histology}}
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| | {{Reflist|2}} |
| | [[Category:Up-To-Date]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Hematology|T-cell prolymphocytic leukemia]] | | [[Category:Medicine]] |
| [[Category:Types of cancer|T-cell prolymphocytic leukemia]] | | [[Category:Hematology]] |
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