Abstract

BackgroundThe Sézary syndrome is an aggressive leukemic form of cutaneous T cell lymphoma and there is no cure of this disease. Until now there is no true animal model for Sézary syndrome, by which new drugs against the disease could be tested.MethodsImmune deficient CB-17 SCID beige mice were injected subcutaneously with HUT78 cells, a cell line, derived from a Sézary syndrome patient. Developing tumors were analyzed by immunohistochemistry.ResultsInjected HUT78 cells formed tumors at the site of injection. In contrast to the Sézary syndrome in man, no malignant cells were observed in the blood of tumor bearing CB-17 SCID beige mice. The tumors appeared 44-62 days after injection and tumor bearing mice survived further 25 - 62 days until they had to be euthanized according to the guidelines of the Swiss animal protection law, since the tumors had reached the maximal allowed size.ConclusionAlthough the mouse model does not exactly match the human disease, it will be suited for tests of new substances for the treatment of the Sézary syndrome. The formation of an isolated tumor on the skin has the advantage that the effect of a potential drug can be directly monitored without the use of invasive methods.

Highlights

  • The Sézary syndrome is an aggressive leukemic form of cutaneous T cell lymphoma and there is no cure of this disease

  • Sézary Syndrome patients show generalized erythroderma, leukemic T cells in the blood and a reduced life expectancy compared to Mycosis fungoides (MF) patients with only approximately 30% of patients surviving beyond 5 years after diagnosis

  • The cell lines were grown in HEPES buffered RPMI 1640 medium supplemented with 2 mM glutamine, 10% fetal calf serum (FCS), 0.25 mg/ml amphotericin B, 100 U penicillin G, 100 U streptomycin and 1 mM pyruvate

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Summary

Introduction

The Sézary syndrome is an aggressive leukemic form of cutaneous T cell lymphoma and there is no cure of this disease. The two main forms are Mycosis fungoides (MF) and its leukemic counterpart, the Sézary syndrome (SzS). Sézary Syndrome patients show generalized erythroderma, leukemic T cells in the blood and a reduced life expectancy compared to MF patients with only approximately 30% of patients surviving beyond 5 years after diagnosis. This is probably due to the circulating malignant T cells producing various immunosuppressant molecules such as IL-10, which can lead to down regulation of the immunological tumor surveillance

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