Cesari syndrome

Sezary syndrome is an erythrodermic form of malignant T-cell lymphoma of the skin with an increased number of large atypical lymphocytes with cerebriform nuclei in the peripheral blood. It was described by A. Sezary and J. Bouvrain in 1938. Sezary syndrome may occur acutely (primary) or, less commonly, in patients with mycosis fungoides (secondary). In recent years, de novo erythrodermic T-cell malignant lymphoma of the skin without previous mycosis fungoides or specific blood changes characteristic of Sezary syndrome has also been identified.

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Pathogenesis

In the upper part of the dermis there is a dense infiltrate consisting of atypical lymphocytes, histiocytes, fibroblasts, neutrophilic and eosinophilic granulocytes. Potrier microabscesses containing atypical lymphocytic and histiocytic elements may also be observed. Electron microscopy revealed a significant number of Sezary cells in the infiltrate. They are cells with a diameter of 7 μm or more, with irregularly shaped nuclei that have deep invaginations of the nuclear membrane, near which there is compacted chromatin, which gives them a cerebriform appearance. The nucleus is surrounded by a narrow zone of cytoplasm with mitochondria, centrioles, and the Golgi complex. Histochemical examination revealed coarse PAS-positive granules and high beta-glucuronidase activity in the cytoplasm. Immunocytological examination showed that Sezary cells in most cases have T-lymphocyte markers, while surface immunoglobulins and the Fc fragment are absent.

In the lymph nodes in Sezary syndrome, changes are similar to those in mycosis fungoides. Blood changes can be detected at clinically early stages using flow immunophenotyping of peripheral blood lymphocytes or by a genetic method using PCR. The prognostic significance of such early blood changes is unclear. For staging, the minimum criteria for blood changes in erythrodermic forms of cutaneous T-cell lymphomas are the presence of more than 5% atypical lymphocytes or Sezary cells in the lymphocyte population and additional evidence of a circulating pathological population using PCR or other adequate methods.

Histogenesis

Sézary syndrome and mycosis fungoides have many common morphological, pathogenetic and immunological properties. Most authors consider Sézary syndrome to be a leukemic variant of mycosis fungoides based on the fact that Sézary cells are found not only in tumor processes. Scientists believe that there are varieties of these cells: reactive with characteristic properties of T-lymphocytes and malignant, not forming F-rosettes. Research by S. Broder et al. (1976) showed that Sézary cells are malignant T-helpers.

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Symptoms Cesari syndrome

Sezary syndrome is characterized by generalized erythroderma with intense itching, enlarged peripheral lymph nodes, and the presence of Sezary cells in the blood and skin proliferates. Elderly men are more often affected. Clinically, the process begins with the appearance of erythematous and infiltrative plaque rashes resembling contact dermatitis or drug rash. Gradually, the elements merge and the process becomes generalized in the form of erythroderma. The entire skin is a rich bluish-red color, edematous, covered with medium- and large-plate scales. Dystrophic changes in the hair and nails are observed. Some patients have plaque-nodular elements that are no different from those in mycosis fungoides. A leukemoid reaction of the blood with an increase in the number of lymphocytoid cells and Sezary cells with T-cell characteristics is added to the typical clinical picture.

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Differential diagnosis

To differentiate Sézary syndrome from other cutaneous T-cell lymphomas with erythrodermic manifestations, which may have a more favorable prognosis than Sézary syndrome, additional criteria are needed to demonstrate an increase in the population of abnormal T cells in the peripheral blood. At present, according to the consensus decision of the Conference of the International Society for the Study of Cutaneous Lymphoma on the Definitions and Terminology of Erythrodermic Forms of Cutaneous T-cell Lymphomas (1998), the following diagnostic criteria for Sézary syndrome have been adopted:

1. Sezary cell count greater than 1000/ ^mm3 or
2. CD4/CD8 greater than 10, which is due to an increase in CD4+ cells or an increase in CD4+ CD7- or Vb+ cell populations that make up at least 40% of the total lymphocyte pool by flow immunophenotyping, or
3. Southern blot confirmation of the presence of a T-cell clone, or
4. confirmation of chromosomal aberrations of clonal T cells in the form of the same pathological karyotype in 3 or more cells.