T-cell prolymphocytic leukemia
| T-cell-prolymphocytic leukemia | |
|---|---|
 | |
| Prolymphocyte | |
| Specialty | Hematology, oncology |
T-cell-prolymphocytic leukemia (T-PLL) is a mature
Signs and symptoms
People affected by T-cell prolymphocytic leukemia typically have systemic disease at presentation, including enlargement of the liver and spleen, widespread enlargement of the lymph nodes, and skin infiltrates.[1]
Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood,
Causes
It is postulated that the originating cell line for this disease is a mature (post-thymic) T-cell.[1]
Diagnosis
Morphology
In the peripheral blood, T-PLL consists of medium-sized
Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood.
Immunophenotype
T-PLL has the
Genetic findings
Clonal TCR gene rearrangements for the γ and δ chains are typically found. The most frequent chromosomal abnormality is the inversion of chromosome 14, specifically inv 14(q11;q32). This is found in 80% of cases, while 10% of cases show a reciprocal translocation of chromosome 14 (t(14;14)(q11;q32)).[4]
[5] Also, abnormalities of chromosome 8 are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and trisomy 8.[6]
Treatment
Most patients with T-cell prolymphocytic leukemia require immediate treatment.[7]
T-cell prolymphocytic leukemia is difficult to treat, and it does not respond to most available chemotherapeutic drugs.
Alemtuzumab (Campath), an anti-CD52 monoclonal antibody that attacks white blood cells, has been used in treatment with greater success than previous options.[7] In one study of previously treated people with T-PLL, people who had a complete response to alemtuzumab survived a median of 16 months after treatment.[7]
Some patients who successfully respond to treatment also undergo
Prognosis
T-PLL is an extremely rare aggressive disease, and patients are not expected to live normal lifespans. Before the recent introduction of better treatments, such as alemtuzumab, the median survival time was 7.5 months after diagnosis.[7] More recently, some patients have survived five years and more, although the median survival is still low.
Epidemiology
About four men are diagnosed with this disease for every three women.[8] Despite its overall rarity, it is also the most common type of mature T cell leukemia.[9]