INTRODUCTION

As a result of our changing lifestyles, we are witnessing a marked increase in the incidence of tumor pathologies, especially in young people. Sezary syndrome is a leukemic form of cutaneous T-cell lymphoma, with an average age of onset of sixty years. However, rare misleading clinical forms are possible, particularly, in younger subjects.

Herein, we describe two cases of Sezary syndrome in young subjects in their thirties, with an opposite course of good improvement in one patient and therapeutic resistance leading to rapid death in the other.

CASE REPORT

The two patients were aged 30 and 29 years respectively, chronic smokers, with no personal or familial history of atopy, both were hospitalized for Sezary syndrome with several months of evolution. The diagnosis was made on the basis of:

	Figure 1: (a and b) Initial clinical images of the first patient.
	Figure 2: Initial clinical images of the second patient with (a) erythroderma and (b and c) palmoplantar keratoderma.
	Figure 3: (a and b) Histological images of the skin of the two patients showing lymphoid proliferation of T phenotype.
	Figure 4: Diffuse immunohistochemical expression of (a) CD3, (b) CD4, (c) CD8.

As part of the extension work-up, a thoraco-abdomino-pelvic CT scan was performed in both patients, showing no secondary localizations.

The two patients were initially treated with dermocorticoids, weekly injections of methotrexate (25 mg/week), and extracorporeal photochemotherapy.

The evolution after two months of treatment was marked by clinical improvement in the first patient, in contrast to the second patient, who presented a rapid clinical worsening, with deterioration of general condition, accentuation of pruritus, worsening of the erythroderma taking on a purplish appearance, generalized edematous infiltration and the appearance of papulo-nodular lesions (Figs. 5a–5c).

Figure 5: Clinical worsening of the second patient with the appearance of (a) facial masses, (b) diffuse papulo-nodular lesions, (c) and the accentuation of erythroderma taking on a purplish appearance.

Histology of the nodular lesions revealed lymphoid proliferation of T phenotype expressing anti-CD3, CD4, and CD8 antibodies, and moderate membrane expression (≤ 5%) of tumor cells of anti-CD30 antibody.

Biological worsening was also noted, with an increase in leukocytes (41120/mm3), lymphocytes (11320/mm3), eosinophils (1910/mm3), and LDH (450 U/L), as well as in the percentage of Sezary cells, estimated at 42% (6207 cells/mm3).

Gemcitabine mono-chemotherapy was, then, added to the previous treatments. The clinical course was marked by a sudden worsening of the disease, with weight loss and cachexia, leading to the patient’s rapid death after one month (Figs. 6a and 6b).

Figure 6: (a and b) Rapid progression to cachexia leading to death.

DISCUSSION

The particularity of our observation lay in the opposite evolution of the two patients, with a rapidly progressive and fatal evolution leading to death in our patient, contrary to the results of the literature where the evolution may, in certain cases, be fatal due to the resistance of the cells to chemotherapy, yet generally remains slow [1,2].

Young-onset Sezary syndrome is rare and is often misdiagnosed and delayed due to clinical polymorphism, in some cases mimicking benign skin eruptions. Nevertheless, early manifestations in patients with severe atopic dermatitis have been described [3].

One of the first large series to study the incidence and mortality of cutaneous T-cell lymphoma was an American study conducted in 1999, which found an especially low incidence in young subjects, estimated at 0.09%, correlated with a very low mortality rate [4].

A more recent study, based on the results of the California Cancer Registry and the SEER, showed that the incidence was higher than in the previous series, yet remained much lower than in the elderly [1].

The low incidence of Sezary syndrome in young subjects makes the prognosis difficult to anticipate, yet it would appear to be better than in older subjects [5,6], with a ten-year survival rate estimated at 94.3% in a study of 240 patients under 30 with mycosis fungoides or Sezary syndrome, where only nine patients died [1].

In addition, the main causes of mortality are recurrent infections and an association with other neoplasias, in particular, a statistically significant increase in the risk of developing melanoma, Merkel cell carcinoma, or other lymphomas [7–12].

To date, there has been no specific consensus on the treatment of aggressive Sezary syndrome in young people. The management depends more on the stage of the disease than on age [1,2,6].

CONCLUSION

The poor prognosis of Sezary syndrome illustrates the importance of early diagnosis. It should, therefore, be considered in the case of any atypical dermatosis that does not respond to standard treatments, regardless of age.

REFERENCES

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