Devita, Hellman, and Rosenberg's Cancer

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

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

Question 30.30. Which statement about CTCL is FALSE?

A. The skin is the most common extranodal site of lymphomas.

B. The World Health Organization European Organization for Research

and Treatment of Cancer (WHO-EORTC) classification identifies

three groups as CTCL: cutaneous T/NK cell lymphoma (MF) and

its Sezary subtype, now viewed as a distinct entity; the cutaneous ́

B-cell lymphomas; and the precursor hematologic neoplasms.

C. B-cell CTCL includes marginal zone (mucosa-associated lymphoid

tissue) lymphoma, primary cutaneous follicular cell lymphoma, dif-

fuse cutaneous B-cell lymphoma, intravascular large B-cell lym-

phoma, lymphomatoid granulomatosis, and cutaneous involvement

of MCL, chronic lymphocytic leukemia (CLL), and Burkitt’s lym-

phoma.

D. Precursor hematologic neoplasms include the blastic NK/cell der-

mal neoplasms, precursor lymphoblastic leukemia/lymphomas, and

myeloid leukemia.

Question 30.31. All is true about S ́ezary syndrome (SS), EXCEPT:

A. Sezary cells are peripheral blood atypical lymphocytes with hypercon- ́

voluted nuclei. SS is defined as diffuse erythroderma with circulating

neoplastic SS cells at>1000 cells/mm^3.

B. IL-11 is an autocrine and paracrine growth factor for Sezary cells. ́

C. Loss of antigens CD2, CD7, and CD4 is seen in MF/SS.

D. CTCL express cutaneous T-cell antigen (CLA) that binds E selectin

on activated endothelial venules and facilitates homing to the skin

via CCL17/CCR4 chemokine receptor interactions.

Question 30.32. All are true about MF, EXCEPT:

A. Outcome of MF/SS is not correlated with clinical stage.

B. Features include patches, plaques, erythroderma, and cutaneous

tumors or ulcers. T-cell receptor rearrangement is used in the differ-

ential diagnosis (psoriasis, eczema, large plaque parapsoriasis, and

drug eruptions).

C. Therapy for localized disease includes topical carmustine or

mechlorethamine, bexarotene, ultraviolet B, plus near ultraviolet

light±interferon alpha, and electron beam radiation.

D. Systemic therapy may include chemotherapy (EPOCH, pentostatin,

fludarabine+interferon alpha, gemcitabine, and doxorubicin), extra-

corporeal photochemotherapy, denileukin diftitox ± bexarotene,

histone acetylase inhibition (vorinostat), and the monoclonal anti-

bodies alemtuzumab and zanolimumab.