|
|
| Line 17: |
Line 17: |
| {{SK}} T-cell chronic lymphocytic leukemia, "Knobby" type of T-cell leukemia, T-prolymphocytic leukemia/T-cell lymphocytic leukemia | | {{SK}} T-cell chronic lymphocytic leukemia, "Knobby" type of T-cell leukemia, T-prolymphocytic leukemia/T-cell lymphocytic leukemia |
|
| |
|
| ==Overview==
| |
| ' T-PLL is a rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults.<ref name="mat1">{{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=}}</ref> Other names include: <ref name="who1"/>
| |
|
| |
|
| ==Clinical Features==
| |
| Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, [[lymph nodes]], [[bone marrow]], [[spleen]], [[liver]], [[skin]].<ref name="who1"/>
| |
|
| |
|
| ===Etiology===
| |
| It is postulated that the originating cell line for this disease is a mature (post-[[thymus|thymic]]) T-cell.<ref name="who1"/>
| |
|
| |
| ===Clinical Presentation===
| |
| Patients typically have systemic disease at presentation, including [[hepatosplenomegaly]], generalized [[lymphadenopathy]], and skin infiltrates.<ref name="who1"/>
| |
|
| |
| ===Laboratory Findings===
| |
| A high lymphocyte count (> 100 x 10<sup>9</sup>/L)along with [[anemia]] and [[thrombocytopenia]] are common findings. [[HTLV-1]] serologies are negative, and serum [[immunoglobin]]s are within normal limits with no [[paraprotein]]s present.<ref name="who1"/>
| |
|
| |
| ==Morphology==
| |
|
| |
| In the peripheral blood, T-PLL consists of medium-sized [[lymphocytes]] with single [[nucleoli]] and [[basophilic]] [[cytoplasm]] with occasional blebs or projections. The [[Cell nucleus|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]].<ref name="mat2">{{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=}}</ref> A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases.<ref name="mat2"/>
| |
|
| |
| Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood.<ref name="who1"/> In the [[spleen]], the leukemic cell infiltrate both the [[red pulp]] and [[white pulp]], and [[lymph node]] involvement is typically diffuse through the [[paracortex]].<ref name="who1"/>. Skin infiltrates are seen in 20% of patients, and the infiltrates are usually dense and confined to the [[dermis]] and around the skin appendages.<ref name="mat1"/>
| |
|
| |
| ==Molecular Findings==
| |
| ===Immunophenotype===
| |
| T-PLL has the [[immunophenotype]] of a mature (post-thymic) T-lymphocyte, and the [[neoplastic]] cells are typically positive for pan-T antigens [[CD2]], [[CD3]], and [[CD7]] and negative for TdT and [[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.<ref name="mat1"/>
| |
|
| |
| ===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, specifcally 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)). <ref name="bri1">{{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=}}</ref>
| |
|
| |
| <ref name="mal1">{{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=}}</ref> Also, abnormalities of [[chromosome 8]] are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and [[trisomy 8]].
| |
| <ref name="sor1">{{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=}}</ref>
| |
|
| |
| ==References==
| |
| {{Reflist}}
| |
|
| |
|
| {{Hematological malignancy histology}} | | {{Hematological malignancy histology}} |