Protocols - Hemorio

11 – PERIPHERAL T-LYMPHOMA
- fundamental the differentiation among the non-specified peripheral T-lymphoma, systemic big cells
anaplastic lymphoma and angioimmunoblastic T-lymphoma;
- non-specified peripheral T-lymphoma: heterogeneous group with variable clinical presentation. Usually
diagnosed on the most advanced stages. Bone marrow and skin infiltration is common.
Immunohistochemistry: CD2+/-, CD3+/-, CD4 +/-, CD7+/-, CD8+/-, TdT-, CD30- and ALK-;
- angioimmunoblastic T-lymphoma: general lymphadenopathy, hepatosplenomegaly and rash. Polyclonal
hypergammaglobulinemia, eosinophilia and self-immune hemolytic anemia are common. The proliferation
of follicular dendritic cells and endothelial cells is frequent at the histopathology. Immunohistochemistry:
CD2+/-, CD3+/-, CD4 +/-, CD7+/-, CD8+/-, TdT-, CD 21+ (dendritic cells close to the venulas), CD30- and
ALK-;
- systemic big cells anaplastic T-lymphoma: the attack located is frequent and usually with a good
response to the QT. Immunohistochemistry: CD2+, CD3+, TdT-, CD15-, EMA+, CD30+ and ALK+. The
sub-group with ALK- presents the worst prognosis. The ALK protein is resultant from t (2;5) (it can be
evaluated by FISH or cytogenetics);
- angioimmunoblastic T-lymphoma: initial treatment with prednisone 1 mg/kg/day. At the absence of
response after 10 days, consider QT (see protocol below);
- non-specified peripheral T-lymphoma and big cells anaplastic T-lymphoma staging I and II: CHOP of 6 to
8 cycles and additional RT if initial bulky or located persistence of disease after the conclusion of the
treatment. Re-staging during the treatment must include all the tests initially positive;
- non-specified peripheral T-lymphoma and big cells anaplastic T-lymphoma staging III and IV: CHOP of 6
to 8 cycles and additional RT if initial bulky or located persistence of disease after the conclusion of the
treatment. Re-staging during the treatment must include all tests that were initially positive. Subjects with
positive ALK-1 anaplastic lymphoma with full response must be followed. Subjects with negative ALK-1
anaplastic lymphoma, peripheral T-lymphoma or angioimmunoblastic T-lymphoma must be taken into
account for the chemotherapy in high doses and autologous transplant in the first remission (mainly if IPI
intermediate or high). In the cases of partial response or absence of response to the initial protocol of QT,
use the rescue protocol for the high-grade LNH and send to the autologous transplant the cases with
chemosensitivity to rescue;
- consider prophylaxis of tumoral lysis syndrome and prophylaxis of CNS in specific cases.
139