T-prolymphocytic leukemia (T-PLL)
Variants: small cell and cerebriform cell.

TdT-, CD1a-,
CD4+ CD8-
CD4- CD8+
CD4+ CD8+ , CD3+, CD2+, CD8+ CD4-, CD57+, CD16+/-Cytotoxic or suppressor activity. , CD2+, CD56+, CD16+, CD7+/-CD3-, CD5-, TCR-Natural killer Activity.

Aggressive course
Splenomegaly, high WBC with prolymphocytes. , Cytopenias, splenomegaly, lymphocytosis with granular lymphocytes. , Lymphocytosis, splenomegaly, hepatomegaly , Hypercalcaemia, lymphadenopathy, flower cells, HTLV-1 Positive. , Hepato splenomegaly , Mediastinal mass, high WBC.

inv(14)(q11q32), t(14;14)(q11;q32)
Xq28 abnormalities
idic(8)(p11), t(8;8)(p11;q1-2)
11q22-23 abnormalities
12p abnormalities
13q14.3 deletions , Abnormal.2p,
 , Abnormal.6q,
 , i(17q),
 , del(13)(q14) , Numerical abnormalities: 3, 7, X.
 , Structural abnormalities: 1q, 3q, 6q, 14q. , +3 or i(3q), +5, del(6q).
 , Progression from normal karyotype to abnormal clone observed during transition from hyperplasia to neoplasia. , t(11;14)(p13;q11)
 , t(1;14)(p34;q11); 1p34: tal-1gene; 14q11: TCR alpha

ATM gene (11q22-23) mutated.
TCL1 (14q32.1) or
MTCP1 (Xq28) activated. , Few cases express MDM2 , Abnormalities of p53, p16 and p15 genes.

Large granular lymphocyte leukemia (LGL) - T-cell Type

TdT-, CD1a

Indolent

Clonal abnormalities. In some cases, but no consistent specific abnormalities.

Clonality established by TCR rearrangements.

Large granular lymphocyte leukemia (LGL) - NK type

TdT-, CD1a

Aggressive or indolent

TCR chain genes in germ line.

TdT-, CD1a-, CD3+, CD4+, CD8-, Helper or no functional activity.

Variable clinical course with skin involvement and cells with cerebriform nuclei.

Complex, clonal, oligoclonal or nonclonal with variable ploidy.

P53 gene deletion and protein expression in the absence of gene mutation.

Adult T-cell leukemia lymphoma (ATLL)

TdT-, CD1a-, CD7- CD4+ CD8- CD25+, Suppressor activity.

Aggressive,

Complex and often oligoclonal.

Oligoclonal/mono clonal integration of HTLV-1in host DNA.

TdT-, CD1a-, CD3+/- CD56+, CD7+, granzyme A+, TCR g/d+

Aggressive,

Abnormal.7q, i(7q)

TCR genes gamma/delta rearranged but alpha/beta not rearranged.

Peripheral/post-thymic T-cell lymphoma (pleomorphic and immunoblastic subtypes)

TdT-, CD1a-, Variable expression of CD4 or CD8

Aggressive; advanced stages.

Variable

TdT-, CD1a-, CD2+, CD5+, CD3+ CD4+ CD8-

Disproteinemia, lymphadenopathy, immune abnormalities.

Complex with multiple related or unrelated clones.

Integrated EBV sequences present in both B-and T-cells and is unlikely to be the etiological agent.

TdT-, CD1a-, T-cell or NK phenotype.

Prevalent in Asia and south America; extra nodal involvement.

i(1q), del(6q), i(6p)

Majority have no TCR rearrangement; EBV clonally integrated and plays a role in the etiology of the disease.

TdT-, CD1a-, CD3+/- CD30+ (Ki 1+), CD15-, CD25+, HLA-Dr+, CD71+

Aggressive with skin nodes and extranodal involvement.

Fusion gene NPM-ALK; 2p23 -Nucleolar phosphoprotein- NPM; 5q35 -Anaplastic lymphoma kinase- ALK.

TdT, CD1a-, CD3+, CD8+, CD103+, CD4-, CD8-

Bone pain, coeliac disease, mesenteric nodes.

EBV genome present in mexican population but not in the europeans.

T-lymphoblastic Lymphoma/leukaemia (T-Lbly/T-ALL)

TDT+, CD1a+, CD7+, cytCD3+ or +/-, other T-cell antigens.Thymic uncommitted T-cell.

Aggressive; course similar to ALL.

TCR chain genes rearranged.

T-cell lymphoid disorders include a variety of disease entities which result from the clonal neoplastic expansion of an uncommitted (thymic) or a committed (post thymic) T-cell. Some of these diseases have distinct cytogenetic/molecular genetic features which allow to better define the various entities and understand their pathogenesis.