Devita, Hellman, and Rosenberg's Cancer

LWBK1006-30 LWW-Govindan-Review December 12, 2011 19:35

432 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology Review

factor to augment antibody-mediated cellular cytotoxicity may be used

to purge residual circulating DLCBL cells before peripheral blood stem

cell collection. Radioimmunotherapy is active in large-cell lymphoma,

but its role as a single agent in this disease has not been studied prospec-

tively. Reduced-intensity allogeneic stem cell transplantation may be used

in patients with less optimal responses or in whom autologous stem cell

collection is not possible.

Answer 30.27. The answer is C.

The GEP in PMBCL shows a distinct signature from nodal DLCBL,

whereas MAL and FIG1 genes were observed; similarities to HL were

apparent with loss of B-cell receptor signaling genes and activation of the

JAK-2 and NF-kb pathways. Ongoing studies are evaluating bortezomib

in combination with R-CHOP therapy in PMBCL.

Answer 30.28. The answer is D.

Patients with localized NK/T-cell lymphoma in the nasal pharynx can

be cured with a combination of chemotherapy and local RT. With RT

alone, treatment failure is frequent. Genetic abnormality includes del(6)

(q21;q25); T-receptor genes are germ line. Patients with disseminated

NK/T-cell lymphoma have an extremely poor outlook and should be

enrolled in clinical trials. Early application of high-dose chemotherapy

and autologous stem cell transplantation may be curative in some patients

after relapse from standard therapy.

Answer 30.29. The answer is C.

Subcutaneous panniculitis T-cell lymphoma is associated with atypical

lymphoid cells and often with necrosis and karyorrhexis. Responses to

RT and chemotherapy are transient, and the long-term outlook is very

poor. Early consideration of aggressive approaches, such as autologous

or allogeneic stem cell transplantation, is recommended.

Answer 30.30. The answer is A.

CTCLs are a heterogeneous group of both T and B lymphocytes that

localize to the skin, which is the second most common site of extranodal

lymphoma estimated at 1:100,000.

Answer 30.31. The answer is B.

Sezary cells are clonal CD4 ́ +CD45RO+T cells; some CTCL variants are

CD8+. Antigen loss is characteristic of the disease (loss of CD7, CD5,

or CD2 and dim CD3) and display a Th2 phenotype secreting IL-4, 5,

6, 10, and 13. The pruritus characteristic of this disease is related to IL-

5 and other chemokines. IL-7, not IL-11, is an autocrine and paracrine

growth factor for these cells. Clinical features of SS include extensive

skin involvement with erythroderma that may progress to lichenification,

palmoplantar hyperkeratosis, and diffuse exfoliation.