Cesary Syndrome

Cesari syndrome is an erythrodermic form of T-cell malignant skin lymphoma with an increased number of large atypical lymphocytes with cerebriform nuclei in the peripheral blood. Described by A. Sezary and J. Bouvrain in 1938. Cesary syndrome can occur acutely (primary) or, less commonly, in patients with fungal mycosis (secondary). In recent years, de novo erythrodermal T-cell malignant skin lymphoma without prior fungal mycosis or specific blood changes characteristic of the Cesary syndrome has also been isolated.

[1], [2], [3]

Pathogenesis

In the upper part of the dermis there is a dense infiltrate, consisting of atypical lymphocytes, histiocytes, fibroblasts, neutrophilic and eosinophilic granulocytes. There may also be microabscesses of Sodium, containing atypical lymphocytic and histiocytic elements. With electron microscopy, a significant number of Sesari cells were found in the infiltrate. They are cells with a diameter of 7 μm or more, with irregularly shaped nuclei having deep invaginations of the nuclear envelope, near which there is condensed chromatin, which gives them a cerebral form. The nucleus is surrounded by a narrow cytoplasmic zone with mitochondria, centrioles, the Golgi complex. Histochemical examination in the cytoplasm reveals coarse NIR-positive granules and high activity of beta-glucuronidase. An immunocytological study showed that Cesari cells in most cases have T-lymphocyte markers, while surface immunoglobulins and Fc-fragment are absent.

In lymph nodes in Cesary's syndrome, the changes are similar to those in mushroom mycosis. Blood changes can be detected at clinically early stages with the help of flowing immunophenotyping of peripheral blood lymphocytes or by genetic method using PCR. The prognostic significance of such early blood changes is unclear. To determine the stage, the minimum criteria for blood changes in erythrodermic forms of T-cell skin lymphomas is the presence of more than 5% of atypical lymphocytes or Cesari cells in the lymphocyte population and additional evidence of the circulating pathological population by PCR or other appropriate methods.

Histogenesis

Cesary's syndrome and fungal mycosis have many common morphological, pathogenetic and immunological properties. Most authors consider Cesary's syndrome a leukemic variant of fungal mycosis on the basis that Cesari cells are found not only in tumor processes. Scientists believe that there are varieties of these cells: reactive with the characteristic properties of T-lymphocytes and malignant, not forming F-sockets. Studies by S. Broder et al. (1976) showed that Sesari cells are malignant T-helpers.

[4], [5], [6], [7], [8], [9]

Symptoms of the cesary Syndrome

Cesary syndrome is characterized by generalized erythrodermia with intense itching, an increase in peripheral lymph nodes and the presence of Cesary cells in the blood and proliferates of the skin. Older men are more often ill. Clinically, the process begins with the appearance of erythematous and infiltrative-plaque eruptions resembling contact dermatitis or a drug rash. Gradually, the elements merge and the process takes a generalized character in the form of erythroderma. The entire skin is full of cyanotic-red color, edematous, covered with medium- and large-plate scales. Dystrophic changes in hair and nails are observed. Some patients have plaque-nodular elements that do not differ from those in mushroom mycosis. A typical clinical picture is attached to the leukemoid reaction of blood with an increase in the number of lymphocytic cells and Cesary cells with T-cell characteristics.

[10], [11]

Differential diagnosis

To differentiate Cesary's syndrome from other T-cell lymphomas with erythrodermic manifestations, which may have a more favorable prognosis than Cesary's syndrome, additional criteria are needed to demonstrate an increase in the population of pathological T cells in peripheral blood. Currently, according to the agreed decision of the Conference of the International Society for the Study of Skin Lymphoma on the Definitions and Terminology of the Symptomatic Forms of T-Cell Lymphomas of the Skin (1998), the following criteria for the diagnosis of Cesary syndrome are adopted:

1. The number of Cesar cells is more than 1000 / mm ³  or
2. CD4 / CD8 is more than 10, due to an increase in CD4 + cells or an increase in CD4 + CD7- or Vb + cell populations, representing at least 40% of the total lymphocyte pool in flowing immunophenotyping, or
3. confirmation by the Southern blot method 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 cells or more.