Sezeri syndrome
Introduction
Introduction to Sezeri Syndrome First reported by Sézary and Bouvrain in 1938, Sézary syndrome is a leukemia, erythrodermic, mild malignant T-cell lymphoma characterized by systemic erythroderma, superficial lymphadenopathy and peripheral blood. There are atypical cells, although patients with typical MF can progress to Sezeri syndrome, but patients with Sézary syndrome usually show erythroderma from the beginning. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: pruritus
Cause
Cause of Sezeri syndrome
(1) Causes of the disease
The exact cause of the disease is still unknown, and the nature of the disease can be summarized into three kinds of understanding:
1. The disease is considered to be an independent disease. Unlike sputum granuloma, Sézary himself pointed out that although the disease has great similarities with MF in histopathology and cytology, the disease has an abnormal shape in the surrounding blood. Cells, therefore, should be distinguished from mycosis fungoides, which are considered to be a benign and reversible disease, mainly in the appearance of healthy elderly people, as a result of delayed hypersensitivity reactions, but can also be transformed by other diseases. For example, atopic dermatitis, contact dermatitis, drug reactions and skin grafting reactions, only a small number of patients converted to malignant lymphoma.
2. It is considered that this disease is a syndrome that can occur in various malignant lymphomas.
3. It is considered to be a subtype of mycosis fungoides - erythrodermic type, because Sézary cells and sputum-like granuloma cells in the skin cannot be distinguished under the light microscope or under electron microscope.
(two) pathogenesis
The pathogenesis is still unclear. Through immunological studies, Sézary cells have been shown to be T cells, which can be synthesized in vitro with PHA to form DNA, form petals with sheep red blood cells, and can be resistant to specific rabbit anti-human T cells. Serum is killed, so Sézary cells, like sputum granuloma cells, are derived from T cells, and the immunophenotype is mainly CD4.
Prevention
Sezeri syndrome prevention
1. Have a good attitude to cope with stress, work and rest, not excessive fatigue. Visible stress is an important cause of cancer, and Chinese medicine believes that stress causes cancer. Prevention of physical and mental weakness caused by decreased immune function, endocrine disorders, metabolic disorders in the body, deposition of acidic substances in the body; stress can also lead to mental stress caused by qi stagnation and blood stasis, poisonous fire invagination.
2. Strengthen physical exercise, enhance physical fitness, and exercise more in the sun. Excessive sweating can excrete acidic substances in the body with sweat, avoiding the formation of acidic constitution.
3. People who have regular life and irregular living habits, such as singing karaoke, playing mahjong, and not returning to the night, will have aggravated physical acidification and are prone to cancer. Good habits should be developed to maintain a weak alkaline constitution and keep various cancer diseases away from you.
4. Do not eat contaminated food, such as contaminated water, crops, poultry, eggs, moldy foods, etc., eat some green organic food (not green vegetables), especially to prevent the disease from entering the mouth.
5. Take an initiative to do an anti-cancer check every year. Everyone has cancerous genetic cells, but not everyone will get cancer. But when your immune function is low, normal cells are reduced and cancer cells are increased. People with a family history of cancer are advised to check the body twice a year; healthy people are advised to review it every year.
6. The onset of this disease is slow. Early patients with reasonable treatment can achieve years of remission, and a small number of patients can relieve themselves.
Complication
Complications of Sezeri syndrome Complications pruritus
Lymph nodes are swollen and itchy.
Symptom
Symptoms of Sezeri syndrome Common symptoms Squamous secondary infection Splenomegaly Leukocytosis Lymph node swelling Weak night sweats nodules Eczema A malnutrition
Slow onset, consciously itchy, early lesions are localized edema ring or scaly erythema, sometimes eczema-like, neurodermatitis-like, can temporarily subside, gradually become erythroderosis after several months or years The skin is infiltrated and thickened, the face is slightly edema, and the shape is like a lion's face. The eyelids can be everted. According to our observation, the erythroderma (Table 1) of this disease has the following different performances compared with the general erythroderma:
1 with brownish yellow;
2 nodules, ichthyosis or mossy lesions appear at the extinction;
3 dry skin, especially with the back of both hands, prone to chapped;
4The skin is swollen and easy to rub. The skin is often pigmented due to scratching or other physical stimuli. Occasionally, blisters may occur, hair may fall off, malnutrition or shedding, some patients have palm, hyperkeratosis, most cases are whole body or Local superficial lymphadenopathy, common in the groin, followed by the underarm or neck, can be resolved when the disease is relieved, late weight loss, fatigue, fever and night sweats, liver, splenomegaly, other organs such as heart, kidney, esophagus And dura mater can also be affected, the incidence of various symptoms can be seen in Table 2, the course of disease is generally longer, a small number of patients can relieve themselves, late patients mostly died of secondary infection due to low immune function, it has been reported that the disease can be developed T lymphoblastic or immunoblastic lymphoma has also been reported to occur after successful treatment of Hodgkin's disease.
The disease is characterized by exfoliative erythroderma, itchy, superficial lymph nodes and liver, splenomegaly, and increased leukocytosis and blood S cells (more than 15%), generally not difficult to diagnose, but sometimes with other partners Identification of diseases characterized by erythroderma (such as chronic lymphocytic leukemia, Hodgkin's disease, erythrodermic MF, and adult T-cell leukemia). The peripheral blood of patients with chronic lymphocytic leukemia is mainly mature lymphocytes, and the morphology of a few naive lymphocytes. Unlike S cells, patients with Hodgkin's disease have no S cells in their peripheral blood and are differentiated from erythrodermic MF and adult T-cell leukemia (ATL).
Examine
Examination of Sezeri syndrome
1. Blood picture: White blood cells are generally (15 ~ 20) × 10 9 / L, the highest is 238 × 10 9 / L, and typical S cells appear, S cells > 10% ~ 15% have diagnostic significance, often with the disease worsening And increase, up to 80%, or even more than 90%.
2. Bone marrow: Early normal, late S-cell visible, we see 29% of cases.
3. Others: Liver, kidney function, serum protein electrophoresis and electrolyte determination are generally normal. E Huahuan test of the cases seen in the dermatology department of Shanghai Huashan Hospital: Et and Ea and lymphocyte transformation rate are lower than normal, indicating patient cell immunity The function is low, not only that, B lymphocytes are only 50% of normal, indicating that the patient's humoral immune function is also reduced.
Common leukocytosis up to 30000/mm3, peripheral blood, skin infiltration and lymph nodes can be seen in deep brain-like helper T cells (Sézary cells), the absolute number is greater than 1000/mm3 or more than 10% of the circulation is the diagnosis of Sezeri syndrome standard.
Pathological examination: unequal amount of S cells appear around the upper part of the dermis, and S cells can also invade.
In the epidermis, Pautrier microabscess is formed. Different from MF, the other infiltrating cells are relatively simple. Only a few macrophages, rare neutrophils, plasma cells or eosinophils can be seen. The late cell infiltration can be extended to the deep or subcutaneous tissue of the dermis. Can be converted to a height ML.
The diameter of S cells is generally about 10m, and the nucleus is large, accounting for more than 80% of the whole cell. It is often irregularly concave and leafy, sometimes typical brain-like or disc-like, with deep nuclear chromatin and some nucleus. Kernels, less cytoplasm, basophilic, sometimes a lot of uniform small vacuoles arranged in a circle shape, vacuole inclusions positive for PAS staining, amylase resistant, neutral mucopolysaccharide, S Cytochemistry of cells.
Immunohistochemistry: Lymphoid cells express CD2, CD, CD5, CD45RO, CD8- and CD3O-, similar to other low-grade cutaneous T-cell lymphomas, such as MF, in most cases, skin infiltration, peripheral blood and affected tables There is a rearrangement of TCR gene clones in the superficial lymph nodes.
Diagnosis
Diagnostic identification of Sezeri syndrome
The disease needs to be combined with clinical and pathological methods to determine, especially in the surrounding blood can find more Sézary cells with relative characteristics (generally should be more than 10%), which has certain significance for diagnosis, in some other skin diseases patients. Similar cells can be found in the surrounding blood, and its significance needs to be further explored. Therefore, it is not possible to confirm the diagnosis by simply checking the cells in the surrounding blood.
In the differential diagnosis, the disease is easily misdiagnosed as chronic lymphocytic leukemia and erythroderma. However, the former is hyperplasia of B lymphocytes from bone marrow. More than 80% of bone marrow is lymphocytes, especially in peripheral blood. More than 100 × 109 / L, must be differentiated from chronic lymphocytic leukemia, psoriasis, atopic dermatitis, solar dermatitis, seborrheic dermatitis, contact dermatitis, drug eruption and red pityriasis.
