Chapter 37 T-Cell Lymphomas
Treatment of Peripheral T-Cell Lymphoma
Given that the mature T-cell lymphomas are relatively rare diseases, there is a lack of large randomized clinical trials comparing the “standard” CHOP and CHOP-like regimens to other more-intensified chemotherapy strategies, or even other novel drug platforms. Our approach involves tailoring the treatment to the specific histologic subtype and age of the patient. From the outset, we send HLA typing for possible allogeneic stem cell transplant, assuming the patient is eligible, and request immunohistochemical staining of the primary tissue for CD30. Where possible, we preferentially try to put all patients on a clinical trial. For the majority of peripheral T-cell lymphomas, our standard initial approach is to use an etoposide-based regimen (CHOEP or EPOCH), especially for young patients or older adult patients with an excellent performance status. As alluded to earlier, there are some data to suggest a reduced failure-free interval and higher complete response rate. For patients with a poor performance status or older adult patients, standard CHOP administered on an every-21-day basis for six to eight cycles would be our preferred recommendation. In select cases, where an older adult patient is not a candidate for combination chemotherapy and requires palliative care, we have successfully used single-agent gemcitabine to obtain disease control and improve performance status. Those patients who attain a complete remission are usually referred for an autologous stem cell transplant. Those patients who do not attain a complete remission with standard frontline chemotherapy are typically referred for treatment with one of the drugs recently approved by the FDA for patients with relapsed or refractory disease, including pralatrexate, romidepsin, or brentuximab vedotin (if CD30+ positive). For patients who relapse or are refractory to frontline and beyond therapy, we typically consider allogeneic stem cell transplant, assuming they are transplant eligible and have an appropriate HLA match.
CHOEP, Cyclophosphamide, hydroxydaunomycin, vincristine (Oncovin), etoposide, and prednisone; CHOP, cyclophosphamide, hydroxydaunomycin, vincristine (Oncovin), and prednisone; EPOCH, etoposide, prednisone, vincristine (Oncovin), cyclophosphamide, and hydroxydaunomycin; FDA, Food and Drug Administration; HLA, human leukocyte antigen.
Figure 37-1 WHO CLASSIFICATION OF THE MATURE T-CELL NEOPLASMS.
NK, Natural killer; NOS, not otherwise specified; TCL, T-cell lymphoma.
Figure 37-2 ANAPLASTIC LARGE CELL LYMPHOMA.
Anaplastic large cell lymphoma has variable morphologic features but is typically composed of large, highly irregular “anaplastic” cells, which include giant cells sometimes with a wreath-like or horseshoe-shape nuclei, and “hallmark” cells, which are cells with a folded-up nucleus with an embryo shape (A). The cells are typically brightly CD30 positive (B). ALK1 staining with cytoplasmic and nuclear localization (C) is associated with the t(2;5)(p23;q35) translocation, whereas other patterns are associated with the variant translocations (C). Whether ALK1-negative cases should be considered as a separate group or classified together with peripheral T-cell lymphoma not otherwise specified is somewhat debatable.
Figure 37-3 PERIPHERAL T-CELL LYMPHOMA NOT OTHERWISE SPECIFIED (PTCL-NOS).
PTCL-NOS is morphologically heterogeneous. Typically, cases have a spectrum of small to large lymphoma cells, frequently with irregular nuclear borders and sometimes with clear cytoplasm. Other cases can have more of a predominance of small or large cells. Features of the other defined types of T-cell lymphoma should be lacking.