T-cell prolymphocytic leukemia: Difference between revisions
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==Overview== | ==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. | '''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. | ||
==Causes== | ==Causes== | ||
Revision as of 16:42, 6 March 2019
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T-cell prolymphocytic leukemia Microchapters |
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Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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T-cell prolymphocytic leukemia On the Web |
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American Roentgen Ray Society Images of T-cell prolymphocytic leukemia |
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Risk calculators and risk factors for T-cell prolymphocytic leukemia |
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
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating T-cell-prolymphocytic leukemia overview 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 Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
Case Studies
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.
Causes
- Common causes of T-cell prolymphocytic leukemia, include genetic factors and chromosomal abnormalities:[1]
- 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:[1]
- Sézary syndrome
- Cutaneous T cell lymphoma
- Angioimmunoblastic T cell lymphoma
- B-cell prolymphocytic leukemia
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard | Additional findings | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | ||||||||||||||
| Lab Findings | Imaging | Histopathology | |||||||||||||
| 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 | ||||
| Sézary syndrome | |||||||||||||||
| Cutaneous | |||||||||||||||
| 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.[1]
- 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.[1]
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:[1]
- 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.[1]
- 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
Physical Examination
- Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
- Physical examination may be remarkable for:[1]
- 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:[1]
- 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
- Typical variant:
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.[1]
Other Diagnostic Studies
Flow cytometry and immunohistopathology must be done to diagnose T-cell prolymphocytic leukemia.
Treatment
Medical Therapy
- 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
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.
- ↑ 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.