What Is Mycosis Fungoides?
Mycosisfungoides Health Search (MF) is a malignant tumor of T cell origin.
Mycosis
- Mycosisfungoides Health Search (MF) is a malignant tumor of T cell origin. Also known as granulomafungoides, is an epithelial cutaneous lymphoma. It is characterized by helper T cell proliferation, Langerhasns cells, and interdigitated reticulum cells also participating in the lesion. The course of the disease is chronic and progressive, with initial forms of erythema and invasive lesions that later develop into advanced tumors that can involve lymphoid viscera.
Mycosis fungal disease
- Mycosisfungoides Health Search (MF) is a malignant tumor of T cell origin.
- Also known as granulomafungoides, Auspitz's dermatosis, Aliber's disease. It is an epithelial cutaneous lymphoma. It is characterized by helper T cell proliferation, Langerhasns cells, and interdigitated reticulum cells also participating in the lesion. The course of the disease is chronic and progressive, with initial forms of erythema and invasive lesions that later develop into advanced tumors that can involve lymphoid viscera.
Mycosis fungal epidemiology
- Alibert first described the disease in 1.1806, and in 1876 Bazin identified the typical clinical manifestations of the disease. In 1885 Vidal and Brocq proposed the burst type. In 1892 Besnier and Hallopeau proposed the erythrodermic type. In 1971, Lutzner reported that under electron microscope, the disease had cells with special brain gyrus nucleus similar to Sézary cells in Sézary syndrome. A clinically and pathologically unique malignant lymphoma. In 1975, Lutzner proposed that the disease was a T-cell lymphoma.
- Mycosis
Causes of mycosis fungal
- Most cases are memory-assisted T-cell tumors. The cause is unknown. In the past, MF was generally considered to be a malignant tumor from the beginning, but more and more people who searched for health regarded this disease as an immune disease at the beginning and later developed into lymphoma. The fact that vascular immunoblastic lymphadenopathy often progresses to immunoblastic lymphoma proves the possibility of this development. In addition, the following observations also support the immunological origin of MF: normal human lymphocytes and highland mitogens Or plant hemagglutinin culture caused 5% to 11% of the appearance of cells can not be distinguished from MF cells or seary cells seen under light microscope and electron microscope, indicating that the latter is the product of stimulated lymphocytes. Epidermotropism and formation of Pautrier microabscesses in MF also represent an immune phenomenon. Langerhans cells present antigens to T lymphocytes, which, like contact dermatitis, cause lymphocytes to align with Langerhans cells. T lymphocytes interact with Langerhans cells to form a healthy search of Pautrier microabscesses. The fact that this process is slower than contact dermatitis suggests that it is defective in handling antigens, allows an unproven antigen to persist, and stimulates lymphocytes to undergo malignant changes during a chronic response. Given the monoclonality of cell infiltration (momoclonalily) is a malignant marker, southern blotting analysis of T cell receptor gene clonal rearrangement (southernblotting analysis) shows that the monoclonality of T cells is not obvious in the early plaque phase, but in the induration Plaques can be identified as they progress to the tumor stage. In addition, viral infections have also been reported. Among 315 MF patients, 36 (11.4%) were found to have special antibodies against human T-cell lymphovirus-1 (HTLV-1).
Pathogenesis of mycosis fungoides
- The pathogenesis is not very clear, but the following hypotheses are proposed for the pathogenesis of the disease. The skin infiltration of the disease goes through 3 stages:
- 1. Atypical skin infiltration stage Although the initial histopathological changes of the disease are still controversial, it is believed that the atypical infiltration of T lymphocytes and monocytes interacted in the beginning, and it can subside locally or persist in the future or develop under different influences. Into the next stage.
- 2. The above-mentioned atypical infiltrating cells in the skin expansion stage can affect the recycling of T cells. When the latter enters the skin of other parts, the factors that cause the same atypical interactions or stimulate the initial infiltration directly affect the skin of the other parts of the skin. Polymorphic infiltration. With the development of lesions, when plaques or tumors form, the infiltration becomes simpler, mainly T-cell tumor cells, and even at this stage, there is already a very slight systemic immune abnormality.
- 3. In the stage of systemic lesions, when the skin lesions develop or involve other viscera in the blood, the infiltration of monomorphic T-cell tumor cells highlights the abnormal cellular immunity of patients. Abnormal immune response can occur in various ways, can be due to abnormal ways of swimming and recycling, transmission of skin stimuli (persistent antigens, environmental allergens), abnormal T lymphocyte responses or auxiliary or inhibiting component health Search for abnormal regulation while aggregating of lymphocytes and macrophages occurs in the tissue. The occurrence of MF and environmental factors (such as exposure to petroleum industrial products, industrial waste, waste gas, radioactive pollutants, pesticides, pesticides and other allergens, such as light or viruses), susceptibility to the body, abnormal lymphocytes & shy; Monocytes or lymphocytesLangerhans cell interactions, or persistent stimulation of abnormal antigens are related. Based on the above assumptions and reasoning, he believes that the disease is "reactive" in the early stages, and then develops into a malignant neobiosis.
Clinical manifestations of mycosis fungoides
Mycosis fungal disease
- MF is primary in the skin but is often affected by lymph nodes and viscera.
- 1. Clinical manifestations and histopathological staging Typical cases can be divided into 3 stages clinically and histopathologically, namely erythema stage, plaque stage and tumor stage. Phase 3 can overlap each other, so phase 3 damage can occur simultaneously.
Mycosis fungus erythema
- The rash is patchy, usually flat and not atrophic, but some patients show atrophy and flatness. Non-atrophic plaques often have scales attached, similar to psoriasis or eczema. Polycyclic or arched. The surface of atrophic plaque is bright, easy to shrink, normal gully disappears, capillaries dilate, hypopigmentation or pigmentation is clinically. It showed vasotrophic skin heterochromatism, large plaque parapsoriasis or mottled parapsoriatic disease. Flat non-atrophic plaques usually infiltrate after several months and years, and a health search for visceral damage can also occur. In contrast, only 12% of patients with flat atrophic plaques have evolved to aggressive MF.
Mycotic fungal plaque
- It progresses from the erythema stage, or occurs on normal skin. It is irregular, with well-defined and slightly higher plaques, ranging from yellow-red, dark red to purplish red. It can subside on its own, or it can be fused into a ring-shaped arch or limp on the edge of a large plaque. When the face is affected, the folds deepen to form a "lion face". The plaques can fuse into each other, but there is scattered normal skin. Further development of the lesion can occur with painful superficial ulcers. Lymphadenopathy often occurs during this period, without tenderness, solid in nature, and can be pushed freely.
Mycosis fungal stage
- It can occur on existing plaques or on normal skin. The skin lesions are maroons of various sizes and different shapes with high nodules, which tend to rupture early and form deep oval ulcers. The basal layer is covered with necrotic pale gray-white material, and the edges of the ulcers are curly, and they occur in the trunk. But it can also occur in any part of the mouth and even the upper respiratory tract. Once a tumor develops, patients usually die within a few years. In addition, MF can be seen as a erythrodermic subtype, with systemic exfoliation and flushing of the skin, scarce hair, malnutrition, palmar plantar keratosis, and sometimes systemic pigmentation. Occasionally, follicular mucinosis, hypopigmentation damage, and MF associated with health search.
Mycosis fungal examination
- 1. Hemoglobin is normal in the early stages, and mild anemia may occur in the later stages. Occasionally or hemolytic anemia. In some cases, white blood cells have increased. Eosinophils and monocytes increase lymphocytopenia, which is particularly common in patients with generalized plaques and tumors. It is suggested that the cases with poor prognosis are mostly in stage , 27.5% have increased eosinophils, 47.5% have monocytes and 76% have lymphocytes. It is reported in the literature that abnormal lymphocytes can be found in blood in about 20% of cases (our 70.8% of our cases), occupying 6% to 35% of the number of nuclear cells, and most of them are below 20%.
- 2. Bone marrow is generally normal like health search, with occasional increase in plasma cells. We examined 9 cases, 6 cases had increased eosinophils and plasma cells, 2 cases had macrophage hyperplasia, and 6 cases saw abnormal lymphocytes. The number of nuclear cells occupied 2% to 3%.
- 3. Peripheral blood electrolytes were normal. We determined that 20 cases of blood calcium were normal. Uric acid can increase. Advanced serum albumin decreases, and alpha1 and alpha2 globulin increase. Reduced circulating helper T cells, reduced responsiveness to PHA stimulation, increased ineffective cells, decreased monocyte tropism, decreased monocyte inhibitory factors, and increased healthy search for serum IgG and IgE.
- 4. Seventy cases of erythrocyte sedimentation rate were measured, of which 60 cases (80.5%) had different degrees of increase (15-30mm / h).
- 5. Immunoassay The cellular immune response (including tuberculin test, DNCB test, streptavidin, and lymphocyte conversion rate measurement) is negative or lower than normal. Of the 40 DNCB trials, 22 (53.84%) were negative; 50 of the OT trials were healthy searches, of which 32 (64.28%) were negative; and 20 of the LTTs, of which 15 (78.58%) were lower than normal. Fluorescence examination showed the deposition of IgG, IgA, IgM and IgD in the vessel wall.
- 6. Other liver functions such as increased liver fatigue and increased serum alkaline phosphatase are also abnormal. When the lungs are affected, X-rays show tumor-like shadows, but they are not characteristic.
Mycosis fungoides treatment
- To enhance patient immunity. Many therapies can provide relief of the disease over time. Topical corticosteroids, topical nitrogen mustard therapy, or carmustine (carbazide) and PUVA can be used in stages IA, IB, and A. Whole body skin electron beam therapy can be used for stage A, B. Single-dose chemotherapy or photoimmunochemotherapy can be used for early treatment of patients with stage III health search systemic chemotherapy, vitamin A, photochemical immunotherapy and alpha interferon can be used for stage VI treatment.
- 1. In the early stage of local corticosteroid therapy (plaque stage T1 and T2), high-intensity corticosteroid emulsion for external application of T1 patients had a complete remission of 63% and a total effective rate of 94%. The complete response rate of T2 patients was only 25%, and the total effective rate was 82%. Easy to relapse after treatment is stopped
- 2. Local Nitrogen Mustard Therapy 100mg Nitrogen Mustard is dissolved in 60ml tap water for external application to the whole body (except the genital area), and it is applied once a day for several months. 80% of patients with IA, 68% of patients with IB, 61% of patients with A, 49% of patients with B and 60% of patients with stage III showed significant effects. About 10% of patients can obtain long-term relief for more than 8 years. The main side effect is skin irritation; the use of ointment base can reduce the response, but the effect is worse than that of water. At least 1/2 of the patients relapsed after treatment was stopped, and retreatment was still effective.
- 3. Topical Carmustine (Carnitine) Therapeutic preparation of ethanol solution containing 2mg of Carmustine per ml. Add 5ml of this stock solution to 60ml per day. Once a day for external application of the whole body (if there is no skin lesions on the folds, genitals, hands, feet, and feet), when the skin lesions are limited, they can only be used externally for an average of 8 to 12 weeks. If the treatment is not effective after 3 to 6 months, the drug concentration can be doubled and the treatment can be repeated for 12 weeks. Small pieces and refractory skin lesions can be directly treated with the stock solution for external application. Patients are less tolerant to carmustine (nitrocarb mustard) than nitrogen mustard. However, bone marrow suppression can occur in less than 10% of patients treated with low-level carmustine (carbazepine). Long-term application of carmustine (carnitine) treatment can cause long-term and severe capillary dilatation.
- 4. About 75% of patients with plaque stage in UV therapy can be completely relieved by UVB treatment. PUVA is more widely used due to its deeper penetration and is more suitable for intradermal damage. In patients with localized plaque or plaque damage, 88% of patients had complete remission, and extensive damage, 52% of patients had complete remission. Patients with ineffective tumours have poor tolerance to PUVA. PUVA treatment has a short remission period, with an average of about 1 year, so regular maintenance treatment is required. Can also be combined with vitamin A and interferon and PUVA.
- 5. Patients were treated with psoralen before in vitro photochemotherapy, and then blood cells were removed and treated with PUVA to treat about 20% of MF skin lesions completely in vitro, and 20% of patients were partially relieved. The total effective rate of erythrodermic patients was 80%. This treatment is expensive and other more economical treatments should be considered first.
- 6. Whole body electron beam therapy with radiation therapy, the dose exceeding 3000Gy is very effective for the treatment of MF. 98% of patients with stage T1 could achieve complete remission; T27l%; T336%; T464%. 50% of T1 patients can achieve long-term remission; 20% of T2 patients can achieve long-term remission. Erythrodermic type is poorly tolerated. Common side effects are erythema, increased edema damage, baldness and nail loss.
- 7. The biological response modifier interferon is about 60% effective for MF treatment, and 19% can be completely relieved. May have toxic and side effects such as fever, chills, and depression. Interferon therapy can be combined with retinoid therapy.
- 8. Retinoic acid Retinoic acid (isoretinoic acid) and avermic A ester (etretinate) treatment of MF44% effective starting dose of 1mg / (kg · d) health search, which can be increased to 3mg according to patient tolerated dose / (kg · d). Retinoids are effective in patients with IB (T2) and stage III patients. Symptoms can be reduced in patients with stage . Targretin is a synthetic retinoid that preferentially binds to the RXR receptor, causing apoptosis in many tumor cells and is also effective for MF.
- 9. Systemic chemotherapy Systemic chemotherapy is used for advanced MF treatment. Methotrexate 5 125mg / week, effective for T3 patients. Complete response was achieved in 41% of patients, partial response was achieved in 17% of patients, and the total effective rate was 58%. The average survival rate was 8.4 years and 69% of patients could survive for 5 years. Cyclophosphamide, doxorubicin (doxorubicin), vincristine, and prednisolone (CHOP) combined chemotherapy can be used in advanced patients.
- 10. Melt therapy DAB389IL-2 is a recombinant fusion of diphtheria toxin and interleukin-2. This formulation can selectively bind to cells expressing the interleukin-2 receptor causing the cells to die. This preparation treats MF cases expressing interleukin-2 receptor with an effective rate of 37%, of which the complete response is 14%. Side effects are fever, chills, hypotension, nausea, and vomiting. Large doses can cause vascular-leaksyndrome.
What diseases can mycorrhizal fungus have?
- 1. Ulcerative skin lesions prone to infection and secondary sepsis are the most common causes of acute death in MF.
- 2. About 8% of MF disease course is transformed into large cell lymphoma. The median time from diagnosis of MF to the appearance of transformation is 21.5 months. After transformation, the condition is accelerated and the prognosis is poor. 15% to 20% of MF courses are skin viscera. Organ invasion, including lymph nodes and internal organs, skin and external organ involvement rarely occur in localized plaque and plaque stage, and the incidence of generalized plaque stage is about 8%, but it occurs in tumor stage and systemic erythroderma The rate is as high as 30% to 42%. In the early stage of skin lesions, superficial lymph node enlargement is often dermatologically reactive, and tumor invasion only occurs. Superficial lymph nodes in the drainage area of the lesion are often involved first, and deep regions such as the mediastinum and abdominal aorta Invasion of paralymph nodes is usually late. Invasion of internal organs often occurs after invasion of lymph nodes. It most often involves the lungs, spleen, liver, central nervous system and gastrointestinal tract. Bone marrow invasion rarely occurs in early localized skin lesions, but when When there are Sezary cells in peripheral blood, the incidence of bone marrow invasion is significantly increased. Autopsy data show that advanced disease tumors can invade any organ.