T-cell prolymphocytic leukemia
Name = T-cell-prolymphocytic leukemia
ICDO = 9834/3
T-cell-prolymphocytic leukemia (T-PLL) is a mature
T-cell leukemiawith aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skininvolvement. 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] T-PLL is a rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults.cite journal |author=Matutes E, Brito-Babapulle V, Swansbury J, "et al" |title=Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia |journal=Blood |volume=78 |issue=12 |pages=3269–74 |year=1991 |pmid=1742486 |doi=] Other names include: T-cell chronic lymphocytic leukemia, "Knobby" type of T-cell leukemia, T-prolymphocytic leukemia/T-cell lymphocytic leukemia
It is postulated that the originating cell line for this disease is a mature (post-thymic) T-cell.
A high lymphocyte count (> 100 x 109/L)along with
anemiaand thrombocytopeniaare common findings. HTLV-1serologies are negative, and serum immunoglobinsare within normal limits with no paraproteinspresent.
In the peripheral blood, T-PLL consists of medium-sized
lymphocyteswith single nucleoliand basophilic cytoplasmwith occasional blebs or projections. The nuclei are usually round to oval in shape, with occasional patients having cells with a more irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome.cite journal |author=Matutes E, Garcia Talavera J, O'Brien M, Catovsky D |title=The morphological spectrum of T-prolymphocytic leukaemia |journal=Br. J. Haematol. |volume=64 |issue=1 |pages=111–24 |year=1986 |pmid=3489482 |doi=] A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases.
Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood. In the
spleen, the leukemic cell infiltrate both the red pulpand white pulp, and lymph nodeinvolvement is typically diffuse through the paracortex.. Skin infiltrates are seen in 20% of patients, and the infiltrates are usually dense and confined to the dermisand around the skin appendages.
T-PLL has the
immunophenotypeof a mature (post-thymic) T-lymphocyte, and the neoplasticcells are typically positive for pan-T antigens CD2, CD3, and CD7and negative for TdTand CD1a. The immunophenotype CD4+/ CD8- is present in 60% of cases, the CD4+/CD8+ immunophenotype is present in 25%, and the CD4-/CD8+ immunophenotype is present in 15% of cases.
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)). cite journal |author=Brito-Babapulle V, Catovsky D |title=Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia |journal=Cancer Genet. Cytogenet. |volume=55 |issue=1 |pages=1–9 |year=1991 |pmid=1913594 |doi=]
cite journal |author=Maljaei SH, Brito-Babapulle V, Hiorns LR, Catovsky D |title=Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization |journal=Cancer Genet. Cytogenet. |volume=103 |issue=2 |pages=110–6 |year=1998 |pmid=9614908 |doi=] Also, abnormalities of
chromosome 8are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and trisomy 8.cite journal |author=Sorour A, Brito-Babapulle V, Smedley D, Yuille M, Catovsky D |title=Unusual breakpoint distribution of 8p abnormalities in T-prolymphocytic leukemia: a study with YACS mapping to 8p11-p12 |journal=Cancer Genet. Cytogenet. |volume=121 |issue=2 |pages=128–32 |year=2000 |pmid=11063795 |doi=]
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