|
|
| (96 intermediate revisions by 3 users not shown) |
| Line 1: |
Line 1: |
| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{T-cell prolymphocytic leukemia}} |
| {{CMG}} {{AE}} {{MV}} | | {{CMG}}; {{AE}}{{Qurrat}}, {{MV}} |
| | | |
| {{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 |
|
| |
| ==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==
| |
| *In 1973, T-cell prolymphocytic leukemia was first described by Catovsky.<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>
| |
| *In 1994, T-cell prolymphocytic leukemia was described as a separate entity from other large grandular lymphocytic disorders.<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>
| |
|
| |
| ==Classification==
| |
| *Morphologically T-cell prolymphocytic leukemia has three variants:<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>
| |
|
| |
|
| ** Typical (75 percent)
| | ==[[T-cell prolymphocytic leukemia overview|Overview]]== |
| ** Small cell variant (20 percent)
| |
| ** Cerebriform (Sézary cell-like) variant (5 percent)
| |
|
| |
|
| ==Pathophysiology== | | ==[[T-cell prolymphocytic leukemia historical perspective|Historical Perspective]]== |
| *T-cell prolymphocytic leukemia arises from mature (post-thymic) T-cell, which is normally involved in in cell-mediated immunity.
| |
| *The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia.
| |
| *Patients with T-cell prolymphocytic leukemia have TCR gene rearrangements for the γ and δ chains.
| |
| *Mutations of chromosome 8 are seen in approximately 75% of patients.
| |
| *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)
| |
|
| |
|
| ==== '''Immunophenotype''' ==== | | ==[[T-cell prolymphocytic leukemia classification|Classification]]== |
| * T-cell prolymphocytic leukemia cells express different markers including:
| |
| ** CD52 (strongly)
| |
| ** 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)
| |
|
| |
|
| ==== '''Genetic''' ==== | | ==[[T-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== |
| * There are many chromosomal abnormalities in T-cell prolymphocytic leukemia, which mostly involve chromosome 14. Different types of genetic abnormalities are as follows:
| |
| ** 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)
| |
| ** idic(8p11)
| |
| ** t(8;8)
| |
| ** Trisomy 8q
| |
| ** Del(12p13)
| |
| ** Abnormalities in chromosome 6
| |
| ** Abnormalities in chromosome 17
| |
| ** Deletion of TP53 gene
| |
| ** Deletions of or missense mutations at the ataxia telangiectasia mutated (ATM) locus 11q23
| |
|
| |
|
| ==Causes== | | ==[[T-cell prolymphocytic leukemia causes|Causes]]== |
| * 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>
| |
| :*Genetic mutations (e.g. Trisomy 8, chromosomal abnormalities)
| |
|
| |
|
| ==Differentiating T-cell Prolymphocytic Leukemia from Other Diseases== | | ==[[T-cell prolymphocytic leukemia differential diagnosis|Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases]]== |
| *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>
| |
| :*[[Sézary syndrome]]
| |
| :*[[Cutaneous T cell lymphoma]]
| |
| :*Angioimmunoblastic T cell lymphoma
| |
| :*[[B-cell prolymphocytic leukemia]]
| |
|
| |
|
| ==Epidemiology and Demographics== | | ==[[T-cell prolymphocytic leukemia epidemiology and demographics|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.<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>
| |
| *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.
| | ==[[T-cell prolymphocytic leukemia risk factors|Risk Factors]]== |
|
| |
|
| *There is no racial predilection for T-cell prolymphocytic leukemia.
| | ==[[T-cell prolymphocytic leukemia screening|Screening]]== |
|
| |
|
| ==Risk Factors== | | ==[[T-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| *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>
| |
|
| |
| == Screening==
| |
| There is insufficient evidence to recommend routine screening for T-cell prolymphocytic leukemia.
| |
|
| |
|
| == Natural History, Complications and Prognosis== | | ==T-cell prolymphocytic leukemia Diagnosis== |
| *The majority of patients with T-cell prolymphocytic leukemia are symptomatic at the time of diagnosis.
| | [[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]] |
| *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:<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>
| |
| :*[[Graft-versus-host disease]] (allogeneic transplant)
| |
| :*[[Infection|Infections]]
| |
| :*[[Bleeding : Overview|Bleeding]]
| |
| *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>
| |
|
| |
|
| == Diagnosis == | | ==Treatment== |
| === Diagnostic Study of Choice ===
| | [[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]] |
| === History and Symptoms ===
| |
| *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>
| |
| :*[[Fever]]
| |
| :*[[Weight loss]]
| |
| :*[[Night sweats]]
| |
|
| |
|
| === Physical Examination === | | ==Case Studies== |
| *Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
| | [[T-cell-prolymphocytic leukemia case study one|Case #1]] |
| *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>
| |
| :*[[Hepatomegaly]]
| |
| :*[[Splenomegaly]]
| |
| :*[[Generalized lymphadenopathy]]
| |
| :*Skin infiltration
| |
| :*Serous effusions:
| |
| :**[[Pleural effusion]]
| |
| :**[[Peritoneum|Peritoneal]] effusion
| |
| :*Central nervous system involvement (very rare)
| |
| === 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)
| |
| :*[[Anemia]]
| |
| :*[[Thrombocytopenia]]
| |
| :*Negative [[Human T-lymphotropic virus|human T lymphotropic virus]] ([[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 protrusionn
| |
| :**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===
| |
| ===X-ray===
| |
| ===Echocardiography or Ultrasound===
| |
| ===CT scan===
| |
| ===MRI===
| |
| ===Other Imaging Findings===
| |
| | |
| === Other Diagnostic Studies ===
| |
| *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>
| |
|
| |
| == Treatment ==
| |
| === Medical Therapy ===
| |
| *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>
| |
| :*[[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.
| |
|
| |
|
| ==References==
| |
| {{Reflist|2}} | | {{Reflist|2}} |
| [[Category:Up-To-Date]] | | [[Category:Up-To-Date]] |
| Line 163: |
Line 42: |
| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Immunology]] | | [[Category:Immunology]] |
| [[Category:Primary care]]
| |