Epidemiology : the second most common subtype of peripheral T-cell lymphomaref
Aetiology :
- EBV-associated B cell lymphoproliferative disorder, the pathogenesis of which was suggested to be closely associated with immunosuppressive treatment for progressive systemic sclerosisref.
-
reactive lymphoma due to diphenylidantoine
Symptoms & signs :
- diffuse lymphadenopathy
- rash
- fever
- hepatosplenomegaly
- polyclonal hypergammaglobulinemia
-
autoimmune
hemolytic anemia
Laboratory examinations :
-
morphologic features favoring AILT over PCTLu are prominent vascularization
by arborizing venules, expansion of CD21+ follicular dendritic
cell networks, and the recent identification of CD10 as phenotypic marker
of the neoplastic T cellsref.
An oligoclonal or monoclonal B-cell population due to the expansion of
B cells infected with EBV and secondary EBV+ B-cell lymphomas
has been described in some patients (Banks PM, Warnke RA. Mature T-cell
and NK-cell neoplasms. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, eds.
World Health Organization Classification of Tumours: Pathology and Genetics
of Tumours of Hematopoetic and lymphoid Tissues. IARC Press: Lyon; 2001:189–230).
The gene expression profile of nodal peripheral T-cell lymphoma demonstrates
a molecular link between AITL and follicular
helper T (TFH) cellsref1,
ref2
- immunophenotype : CD2+3+(sometimes CD3- but cyCD3+ref)4+10+ref, TcR+; effector or effector-memory cell phenotype (CD45RA-/CD45R0+/CD27-)ref
- CXCL13 expression is a distinctive feature of angioimmunoblastic T-cell lymphoma, providing further support for the germinal center T-helper cell as the cell of origin for this neoplasmref1, ref2, ref3
Therapy : anti-CD4 mAb
(HuMax-CD4 iv once weekly for 10 wkref)
- lethal midline granuloma / polymorphic reticulosis : a progressive, localized, destructive process occurring chiefly in males, predominantly involving the nose, paranasal sinuses, and palate, with erosion through contiguous structures such as the orbit and face and destruction of soft tissue, bone, and cartilage, and associated with nonspecific acute and chronic inflammation and necrosis with or without granuloma formation.
- idiopathic midline granuloma : no neoplastic cells detected
- lymphomatoid granulomatosis : an immunoproliferative disorder that is angiogenic and characterized by tissue infiltration and nodular granulomatous inflammation with atypical lymphocytes and plasmacytoid cells; the organs involved are usually the lungs, skin, central nervous system, or kidneys. Presenting symptoms include cough, shortness of breath, and chest pain; extrapulmonary manifestations are common, such as skin nodules => 50% develops malignant lymphoma
Laboratory examinations : Therapy : HHV-4 / EBV-specific effector Th1 lymphocytes and Tc1 CTLs
