angioimmunoblastic T-cell lymphoma


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angioimmunoblastic T-cell lymphoma

rare subtype of lymphoma characterized by generalized lymphadenopathy, prominent systemic symptoms, fever, weight loss, skin rash, polyclonal gammopathy, circulating immune complexes and autoantibodies, and a tendency to infection complication. Previously referred to as angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). Diagnosis is often difficult because of the varying clinicopathologic picture. Fewer than one third of the patients can be expected to have long-term remissions even after multiagent chemotherapy. Complete remissions have been reported after the use of interferon-α and cyclosporine.

angioimmunoblastic T-cell lymphoma

A lymphoma affecting the elderly characterised by generalised lymphadenopathy and systemic symptoms.
 
DiffDx
Drug reaction (e.g., antibiotics, phenytoin), T-cell-rich diffuse large B cell lymphoma, classical Hodgkin lymphoma, angioimmunoblastic lymphadenopathy with dysproteinaemia, peripheral T-cell lymphoma (undifferentiated).
References in periodicals archive ?
(80) Recurrent mutations have also been identified and well characterized in some non-Hodgkin lymphomas, such as BRAF p.V600E in hairy cell leukemia,81 myeloid differentiation factor 88 (MYD88) p.L265P in lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia, (82) and RHOA p.G17V in angioimmunoblastic T-cell lymphoma. (83-85) These mutations are relatively specific, and their detection may thus aid in the diagnosis and differential diagnosis of non-Hodgkin lymphomas.
We presently report on a T-cell lymphoma, most consistent with angioimmunoblastic T-cell lymphoma (AITL) [6, 7], occurring in an 80-year-old woman's inguinal lymph node.
Soluble Fas in the serum of patients with non-Hodgkin's lymphoma: higher concentrations in angioimmunoblastic T-cell lymphoma. Am J Hematol 1998;58:334-6.
T-cell neoplasms are most commonly associated with eosinophilia, and significant eosinophilia is most frequently seen in the context of cutaneous T-cell lymphoma, adult T-cell leukemia/lymphoma, and angioimmunoblastic T-cell lymphoma. (42) The B-cell neoplasms most significantly associated with eosinophilia are classical Hodgkin lymphoma and B-lymphoblastic leukemia/lymphoma, especially B ALL with t(5; 14)(q31; q32); IGH-IL3.
In T-cell lymphomas, RS-like cells of B-cell lineage are characteristically seen in angioimmunoblastic T-cell lymphoma and, more rarely, in peripheral T-cell lymphoma, not otherwise specified.
(24) Of note, PD-1 is a marker of T-helper cells of follicle origin and is seen in other T-cell lymphomas such as angioimmunoblastic T-cell lymphoma and a subset of peripheral T-cell lymphoma, not otherwise specified.
Angioimmunoblastic T-cell lymphoma is a peripheral T-cell lymphoma of follicular helper T cells that is associated with Epstein-Barr virus and frequently includes cutaneous manifestations.
The main differential diagnosis of ATLL includes peripheral T-cell lymphoma not otherwise specified, ALCL, mycosis fungoides/Sezary syndrome, and angioimmunoblastic T-cell lymphoma. (5,29)
CD30+ large cells are present in angioimmunoblastic T-cell lymphoma (AITL) but are usually few in number and randomly scattered without perivascular accentuation or sinus involvement.
Once the T-cell phenotype has been determined, the differential diagnosis for a predominantly small, T-cell lymphoproliferative lesion with CD30 positivity includes the primary cutaneous [CD30.sup.+] lymphoproliferative disorders (primary cutaneous ALCL, lymphomatoid papulosis, and borderline lesions) (2); peripheral T-cell lymphoma, not otherwise specified; and angioimmunoblastic T-cell lymphoma. (2,3) If cutaneous lesions are not present, it is easy to rule out the primary cutaneous [CD30.sup.+] lymphoproliferative disorders.
Of the secondary or concurrent cutaneous lymphomas, angioimmunoblastic T-cell lymphoma (20%) was significantly more common in Taiwan, whereas adult T-cell leukemia/ lymphoma (29%) was more frequent in Japan (P = .02; SPSS; [chi square] test).