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1684 Part XI: Malignant Lymphoid Diseases Chapter 103: Cutaneous T-Cell Lymphoma (Mycosis Fungoides and Sézary Syndrome) 1685

Figure 103–5. Transmission electron micro-
graphs of lymphocytes. A. Normal lymphocyte.
B. Two lymphocytes from a patient with Sézary
cells in the blood. The latter have the striking
cerebriform nuclear abnormalities characteris-
tic of Sézary cells. (Reproduced with permission
from Lichtman’s Atlas of Hematology, www.
accessmedicine.com.)

A B

the disease usually is indolent and localized, some patients present with THERAPY
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a disseminated form referred to as the Ketron-Goodman variant. The
histologic findings are similar to those found in Woringer-Kolopp dis- A variety of therapeutic modalities produce remissions in most patients
ease, with predominantly epidermal involvement by malignant cells and with MF. In general, MF therapy is divided into (1) skin-directed ther-
a poor prognosis. This variant is a disease of an activated T lymphocyte apy (SDT) and (2) systemic therapy (Table 103–5). SDT is the main-
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that only occasionally expresses the helper T-cell CD4 antigen. 77,78 Like stream therapy in early disease but also is used as an adjunct in systemic
MF, the neoplastic cells have TCR gene rearrangements. disease. Therapeutic decisions may be difficult and heavily depend on
Other mimics of CTCL, such as alopecia mucinosa, contact der- the stage at presentation. Revised practice guidelines are available on
matitis, lichen planus, parapsoriasis, pediatric atopic dermatitis, pem- National Comprehensive Cancer Network (NCCN) website (www.
phigus foliaceus, plaque psoriasis, pustular psoriasis, and tinea corporis, nccn.org). See Fig. 103–6 for an overview of the treatment algorithm
should be considered in the differential diagnosis. The diagnosis is made for MF/SS. In view of the protracted course of the disease and lack of
by appropriate investigation (e.g., potassium hydroxide skin prep) and curative therapies, therapeutic decisions should generally be focused
skin biopsy. CD30+ (Ki-1) and CD30− lymphomas can mimic tumors
of MF; they present as erythematous or violaceous nodules that ulcer-
ate. The critical issue is to differentiate primary cutaneous CD30+ lym-
phoproliferative disorder from CD30+ large cell transformation of MF TABLE 103–5. Therapeutic Option for Mycosis Fungoides
and from secondary cutaneous involvement caused by CD30+ nodal and Sézary Syndrome
lymphoma. The course of CD30+ lymphomas of the skin is indolent,
they carry a favorable prognosis and tend to regress spontaneously, Skin-Directed Therapy Systemic Therapy
whereas transformed MF and nodal lymphoma have poor prognoses. In Topical therapy Immunomodulators
rare instances, these lymphomas progress to systemic involvement and Topical glucocorticoids Interferon-α
have the same prognosis as nodal CD30+ lymphomas. 79,80 Lymphoma- Nitrogen mustard Extracorporeal photophore-
toid papulosis (LyP) is a benign counterpart of CD30+ lymphoprolifer- (mechlorethamine) sis (ECP)
ative disorders of the skin with excellent prognosis. It usually presents Carmustine (BCNU, nitrosourea) Antibodies/fusion proteins
as crops of recurrent pruritic or painful erythematous papules or nod-
ules, which ulcerate and heal spontaneously. LyP usually runs a chronic Retinoids (bexarotene, Denileukin diftitox
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course. LyP may be associated with other malignancies, particularly tretinoin) (ONTAK, DAB –IL-2)
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MF and other lymphomas, in up to 10 percent of cases. Therefore close Topical tacrolimus (Protopic) Alemtuzumab (Campath)
observation and followup are recommended for patients with LyP. Low- Imiquimod (Aldara) Retinoids
dose oral methotrexate is the drug of choice for treatment of LyP. Light therapy Oral bexarotene (Targretin)
UVB and PUVA Acitretin (Soriatane)
Photodynamic therapy Isotretinoin (Accutane)
Electron beam Histone deacetylase inhibitors
TABLE 103–4. Classification of Erythrodermic Cutaneous Localized Vorinostat (Zolinza)
T-Cell Lymphoma Total-skin Romidepsin (Istodax)
Erythrodermic Preexisting Blood Chemotherapy (alone or in
Subset (T4) MF combinations)
Sézary syndrome Rarely Leukemia: B2 Oral prednisone, metho-
Erythrodermic mycosis Always Normal or minimally trexate, doxorubicin, cyclo-
phosphamide; chlorambucil;
Fungoides abnormal: B0–B1 pentostatin, cladribine, flu-
Erythrodermic cutaneous Absent Normal or minimally darabine, pralatrexate, sev-
T-cell lymphoma, not abnormal: B0–B1 eral others
otherwise specified
PUVA, psoralen and ultraviolet radiation of the A spectrum; UVB,
MF, mycosis fungoides. ultraviolet radiation of the B spectrum.

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