T-cell prolymphocytic leukemia: Difference between revisions
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*The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia. | *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. | *Patients with T-cell prolymphocytic leukemia have TCR gene rearrangements for the γ and δ chains. | ||
*Mutations of chromosome 8 are seen approximately 75% of patients. | *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> | *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 | :*No remarkable findings | ||
Revision as of 13:27, 24 May 2016
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
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
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.[1] T-cell prolymphocytic leukemia was first described by Catovsky in 1973.[2] 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.[3] 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.[3] 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.[3]
Historical Perspective
- T-cell prolymphocytic leukemia was first described by Catovsky in 1973.[2]
Classification
- There is no classification system for T-cell prolymphocytic leukemia.[3]
Pathophysiology
- 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:[3]
- No remarkable findings
- On microscopic histopathological analysis, characteristic findings of T-cell prolymphocytic leukemia, include:[3]
- The immunophenotype CD4+/CD8- (present in 60% of cases)
- The immunophenotype CD4+/CD8+ (present in 25%)
- The immunophenotype CD4-/CD8+ (15% of cases)
Pan-T antigens
Causes
- Common causes of T-cell prolymphocytic leukemia, include:[3]
- Genetic mutations (e.g. Trisomy 8, chromosomal abnormalities)
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:[3]
- Sézary syndrome
- Cutaneous T cell lymphoma
- Angioimmunoblastic T cell lymphoma
- B-cell prolymphocytic leukemia
Epidemiology and Demographics
- T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.
Age
- T-cell prolymphocytic leukemia is more commonly observed among patients aged between 30 to 40 years old.[3]
- T-cell prolymphocytic leukemia is more commonly observed among young adults.
Gender
- Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females.
Race
- 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.[3]
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:[3]
- 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 7 months.[3]
Diagnosis
Symptoms
- Symptoms of T-cell prolymphocytic leukemia may include the following:[3]
Physical Examination
- Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
- Physical examination may be remarkable for:[3]
- Hepatomegaly
- Splenomegaly
- Generalized lymphadenopathy
- Skin infiltration
Peripheral Blood Smear
- Medium-sized lymphocytes
- Single nucleoli and basophilic cytoplasm
- The nuclei are usually round to oval in shape,
- Irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome.
- A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases
Laboratory Findings
- Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include:[3]
- High lymphocyte count (> 100 x 109/L)
- Anemia
- Thrombocytopenia
- Negative HTLV-1 serology
Imaging Findings
- There are no specific imaging findings associated with T-cell prolymphocytic leukemia.[3]
Treatment
Medical Therapy
- The mainstay of therapy for T-cell prolymphocytic leukemia, include:[3]
- 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.
Prevention
- There are no primary preventive measures available for T-cell prolymphocytic leukemia.
References
- ↑ Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001
- ↑ 2.0 2.1 Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G. Prolymphocytic leukaemia of B and T cell type. Lancet
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.