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Sézary syndrome and Shakespeare: What’s in a name?

DII small banner By Warren R. Heymann, MD
March 24, 2017

Sézary syndrome
Initial clinical presentations of Sézary syndrome without erythroderma. Normal-appearing skin with moderate pigmentation on back of patient 2 (A), several seborrheic keratoses and a hematoma secondary to skin biopsy for patient 3 (B), no apparent lesion on back of patient 5 (C). Specific lesions: a 2-cm patch on right buttock of patient 5 (D) and palmar keratoderma of patient 6 (E).
Credit: JAAD
O Sézary, Sézary! Wherefore art thou Sézary?

Deny thy erythema and refuse thy name

Or, if thou wilt not, but sworn by biome,

And you’ll no longer be a syndrome.

Dermatologists have contended with entities such as zoster sine herpete and aleukemic leukemia cutis, but now reports of Sézary syndrome (SS) without erythroderma challenge long-held concepts.

SS is a leukemic subtype of cutaneous T-cell lymphoma (CTCL) defined by the presence of erythroderma and significant peripheral blood involvement by malignant T cells. Stages of blood involvement are recognized by the revised ISCL/EORTC classification system. Significant blood involvement (B2) is associated with an aggressive course, similar to visceral involvement (Stage IV). The prognosis of cutaneous T lymphoma is determined in part by the TNMB stage, which is reviewed by Junkins-Hopkins (1).

Henn et al sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma. Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with SS. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred. The authors concluded that SS without erythroderma is a rare entity that may have a better prognosis than classical SS (2).
In contrast, Thompson et al, in their series of 16 patients of SS without erythroderma, found that leukemic involvement may confer shortened survival as in classical SS. The authors retrospectively identified patients with SS, but without erythroderma, at the Mayo Clinic. All patients met criteria for high blood tumor burden. Their clinical characteristics, disease course, and prognosis were reviewed and compared with a previously described cohort of 176 patients with SS. Among the 16 patients identified, all had cutaneous findings consistent with CTCL, most commonly erythematous patches (n = 6) and plaques (n = 12). Erythroderma eventually developed in 6 of the 16 patients. All 16 patients were deceased at the time of the study. The median time from the diagnosis of SS without erythroderma to the date of death was 3.6 years. Survival was not different between patients with SS with and without erythroderma. The authors proposed that using the TNMB staging may more accurately describe patients with CTCL including classical and atypical forms of SS (3).
While I understand the authors’ sentiments and desires to use the TNMB classification to accurately portray such cases, I am not enamored with the idea. For example, if I told you that my professional life centers at 390 55’ 32” latitude and 750 7’ 12” longitude, would you recognize that as Camden, New Jersey? Just as the term mycosis fungoides has remained, falling under the umbrella of CTCL, the diagnosis of SS will continue as well. Every dermatologist knows what classical SS represents. Let’s not obfuscate the picture with terms such as “SS without erythroderma,” “Atypical SS,” or “Incomplete SS.” The important concept is that there are rare leukemic cases of CTCL presenting as pruritus without cutaneous lesions, or appearing as more classical lesions of mycosis fungoides. When staging CTCL patients, regardless of their clinical presentation, check the blood for Sézary cells. I implore the arbiters of the lymphoma world to reserve the diagnosis of SS for patients with blood involvement and erythroderma.

1. Junkins-Hopkins JM. Aggressive cutaneous T-cell lymphomas. Semin Diagn Pathol 2017; 34: 44-59.
2. Henn A, et al. Sézary syndrome without erythroderma. J Am Acad Dermatol 2015; 72: 1003-9.
3. Thompson AK, et al. Sézary syndrome without erythroderma: A review of 16 cases at Mayo Clinic 2017; 76: 683-8.

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