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Myxedema face

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[Intro]

myxedema hypothyroidism face is characteristic of the adult face. Chinese medicine is qi deficiency with deficiency syndrome; Doctors believe that common in hypothyroidism. To testosterone deficiency and hypopituitarism can also be found such a face.

[Cause]

adult t-cell leukemia is caused by what the?

(a) causes

ATL occurrence and human T-cell leukemia virus type Ⅰ (HTV-Ⅰ) infection related to serum HTLV-Ⅰ-positive. High incidence of the southern island of Japan Kyushi, where residents of 10% ~ 15% HTLV-Ⅰ antibody-positive, low incidence of other places. How the high incidence in Japan and other regions linked to disease is unclear.

research suggesting that host susceptibility and (or) a common environmental conditions, infection with HTLV-Ⅰ, family members of the HTLV-Ⅰ antibody-positive rate is independent of the normal population of 3 to 4 times. In the antibody-positive, patients with normal serum clinical separable from the virus HTLV-Ⅰ.

(B) the pathogenesis

HTLV-Ⅰ still need a long incubation period after infection, it may eventually lead to a few people suffering from This in itself shows the incidence of ATL ATL complexity. ATL has not yet been finally clarified the pathogenesis of many data indicate that, ATL incidence may be related to the following mechanisms.

1. regulatory protein Tax in the HTLV-Ⅰ end of the original virus, there is a long terminal repeat (LTRs), LTRs contain the regulatory part of the virus, including the promoter sequence from U3, R and U5 (unique 3 'end, repeat the 5' end) sequence of the composition. Tax is the main function of the sequence 5'-LTR trans-activation function of HTLV-Ⅰ to regulate the transcription of viral replication, activate transcription of viral and cellular genes by at least two different host transcription factor pathway, proteins are involved in CAMP response element, activating transcription factor (ATF) and the transcription factor NF / κB / C-Rel family. Tax incidence in ATL may be the following role: ① IL-2 promoter activation and IL-2Rα subunit to stimulate t cells secrete their own growth, or even start the formation of T-cell immortalization, and finally to the occurrence of ATL: including IL- 2 and IL-2Ra, c-fos, c-jun and parathyroid hormone-related protein (PTHrP). ATL cells release these cytokines can cause a range of pathology: IL-2 and IL-2Ra can lead to T cell activation and proliferation, access to self-regulation of growth; PTHrP can stimulate osteoclasts, so that patients showed calcium hyperlipidemia; c-fos gene may be involved in T cell proliferation. ② Tax can accelerate the cell cycle in G1 phase and promote their progress into the S phase of cell cycle expression of Tax shorter. Faster cell growth kinetics, which is an H Ⅱ Ⅳ Ⅰ related diseases may be relevant; ③ Tax-mediated nf-κb activity changes. In the tumor may have a role.

2.HTLV-Ⅰ infected immune function transforming growth factor Tax can activate β1, (TGFβ1) expression levels, which in human cells and humoral immune disincentive. HTLV-Ⅰ infected cells, may be encoded by the virus, a new HLA-Ⅰ and HLA-Ⅱ epitopes, leading to immune dysfunction, reduced ability to host defense, tumor occurrence and development to create the conditions.

3. oncogene activation and inactivation of anti-cancer gene does not encode HTLV-Ⅰ despite cancer genes, but the cis-activation mechanism still exists. Such as the Tax can activate c-fos gene, indicating that efficient trans-activator protein Tax may start the malignant transformation.

other secondary events of p53 mutations. p53 is a nuclear phosphoprotein, the role of a tumor suppressor gene. loss of p53 activity caused by mutations in many malignant diseases have been found, ATL is no exception. Many patients with acute ATL p53 mutations were detected, and chronic ATL patients this mutation is very rare phenomenon. Some data indicate that, p53 mutations associated with a certain incidence of ATL. However, other recent studies that Portis, p53 gene does not cause functional inactivation of tumor, but may promote the proliferation of advanced malignant tumor effect.

seen in peripheral blood or the nucleation of many petal-like lymphocytes (flower cells). Sizes of these cells, the nuclei were pleomorphic changes, distorted, deformed, or lobulated nucleus deep depression, was the second leaf or leaves, or was baseball-like, glove-like, folded petal-like, it is also known roseus cells. Cytochemical staining showed negative peroxidase, acid phosphatase β glucuronidase positive. Cellular immune markers confirmed flowers mature T lymphocytes.

skin lesions, mostly due to a large number of abnormal lymphocytes. 2 / 3 of the skin lesions in patients with focal epidermal infiltration and Pautrie small abscess. In addition, lymph nodes, liver, spleen, lungs, gastrointestinal tract can also be a large number of abnormal lymphocytes, the performance of the relevant organ enlargement and dysfunction.

ATL large number of abnormal lymphocytes and plasma vascular endothelial growth factor (VEGF) levels increased. Data indicate that, ATL cell lines expressed vegf mRNA, and secreted into the extracellular environment. meanwhile, ATL cell lines also expressed the vegf receptor Flt-1 (fms-like tyrosine kinase-1) mrna and protein, and vegf and Flt-1 expression only effective combination of cells, leading to enhanced chemotactic activity of ATL cells, resulting in ATL cell infiltration of tissues and organs.

[Sign]

adult t-cell leukemia early symptoms?

ATL patients with a variety of clinical manifestations, can be expressed as a kind of acute type of leukemia, lymphoma-type lymphoproliferative better prognosis of chronic type and smoking status (insidious type).

almost all of the patients had lymph nodes. Many patients with extensive lymph node disease, a majority of retroperitoneal lymph nodes, but the mediastinal mass is rare. leukemic cell infiltration of bone marrow often. Other common sites affected are the lungs, liver, skin, gastrointestinal and central nervous system.

about 2 / 3 of patients with skin involvement can occur, most of the skin seen in patients with focal infiltration of ATL cell infiltration or abscess Potter micro Montpellier (Pautrier microabscesses).

main clinical manifestations of various types

1. Acute type: The median age was 40 years old. Typical performance: the incidence of very rapid, rapid progress was mainly skin lesions, hypercalcemia, or both. A variety of skin lesions, such as scattered tumor blocks, integration of small nodules, plaques, papules, erythema and other non-specific. Patients with hypercalcemia usually fatigue, apathy, confusion, polyuria, polydipsia.

2. chronic type: may have swollen lymph nodes, hepatosplenomegaly, skin and lung infiltration, no hypercalcemia, no central nervous system, bone, gastrointestinal tract infiltration, without ascites and pleural effusion.

3.'s lymphoma type: lymph nodes histologically proven lymph node disease, leukemia-cell infiltration.

4. smoke type: characterized by skin lesions, can be expressed as erythema, papules, nodules. Can have pulmonary infiltrates. Generally no hypercalcemia, lymphadenopathy, hepatosplenomegaly and bone marrow infiltration were mild; no central nervous system infiltration.

1. domestic diagnostic criteria (ATL 1984, some provinces and cities nationwide collaborative meetings)

(1) leukemia performance: ① disease in adults; ② a superficial lymph nodes, no mediastinal or thymic tumor.

(2) laboratory tests: white blood cells often increased, polymorphonuclear lymphocytes (flower cells) accounted for 10% or more; is a T-cell type, mature t cells surface markers; serum antibodies against HTLV-Ⅰ.

2.ATL foreign diagnostic criteria (Schimoyama metal, 1991)

(1) histology and (or) chemical evidence for the lymphocytic leukemia cells with T cell surface antigens (mainly CD2, CD3, CD4).

(2) must have abnormalities in peripheral blood T lymphocytes, including the typical adult T-lymphoid leukemia cells (also known as flower cells and small mature T cells, have cut into the nucleus leaf depression or nuclear).

(3) anti-human T cell leukemia virus type Ⅰ (HTLV-Ⅰ) antibodies.

3.ATL subtype diagnostic criteria (Gessainetal, 1992)

(1) smoke type:

abnormal peripheral blood t cells ≥ 5%.

② total lymphocyte count is normal.

③ no high blood calcium, LDH ≤ 1.5 × normal.

④ without lymph node disease; no liver, spleen, CNS, bone, gastrointestinal tract involvement.

⑤ no ascites or pleural effusion.

⑥ may have skin and lung damage.

⑦ If the abnormal t cells <5%, should be histologically confirmed skin and lung damage.

(2) chronic type:

① the absolute number of lymphocytes increased (≥ 4 × 109 / L) with T cells> 3.5 × 109 / L, including abnormal t cells and occasional petal-shaped cells.

② no high blood calcium, LDH ≤ 2 × normal.

③ no CNS, bone, gastrointestinal tract involvement, no pleural effusion or ascites.

④ may have lymph nodes and spleen, liver, lung, skin involvement.

(3) lymphoma type:

① no increase in lymphocytes, accompanied by abnormal lymphocytes ≤ 1%.

② histologically positive lymph node lesions.

(4) acute type:

① except for the three types of ATL patients, often with the performance and lymphatic leukemia enlargement of the lesions.

② organized science, and (T-lymphocytes or cytologically confirmed tumor.

③ In addition to lymphoma outside the ATL , there should be abnormal peripheral blood T lymphocytes, including the typical "petals" cell and a small notch and leaf nuclear mature T lymphocytes.

④ LTHV-1 antibody positive.

[Aftertreat]

adult t-cell leukemia ate?

[Prevent]

myxedema face should be how to prevent?

(1) add the right amount of iodine, avoid using raw thyroid material.

1) add salt: general domestic use every 2 to 10 kg of salt plus 1 g of potassium iodide concentration for the control of goiter, the incidence rate decreased significantly for endemic goiter endemic area. In addition, women should pay more attention to the fertility supplement of iodized salt to prevent iodine deficiency caused by maternal offspring suffering from cretinism.

2) avoid using raw thyroid material: Avoid eating cabbage, Chinese cabbage, rape, cassava, walnuts, etc., in order to avoid application goiter.

(2) the supply of sufficient quantities of protein: protein mass per person per day at least more than 20G, in order to maintain the body balance of egg protein, amino acid composition of proteins is a basic ingredient, about a day 3% of the protein is continuously updated when the hypothyroidism slowed small intestinal updates, digestive gland body affected, decreased enzyme activity, decreased albumin general, it should be added essential amino acids, adequate protein supply, improve the condition.

(3) limit fat and cholesterol-rich diet: hypothyroidism patients often have hyperlipidemia, which is more evident in primary hypothyroidism, it is dietary fat should be limited . Daily fat supply about 20% of total calories, and limit cholesterol-rich diet.

(4) to correct anemia, a rich supply of vitamins: anemia should supplement a diet rich in iron, vitamin B12, such as animal liver, if necessary, the supply of folic acid liver preparations.

(5) diet allocation:

1) appropriate selection of food: hypothyroidism due to iodine deficiency, the need to use amount of kelp, seaweed, used iodized salt, iodine, soy sauce, eggs and bread, iodized iodine. Cooking should note that iodized salt should be put into boiling oil in the wood, so as not to lower iodine concentration of volatile iodine. protein supplement can be used eggs, dairy, meat, fish; vegetable protein can complement each other, such as various soy products, soybeans and so on. Supply of animal liver can correct anemia, but also to ensure the supply of vegetables and fresh fruit.

2) avoid selection of food: avoid goiter of various raw materials, such as cabbage, Chinese cabbage, rape, cassava, walnuts, etc.; avoid cholesterol-rich foods such as butter, animal brain and other organs. Restricting the use of high-fat foods, such as oil, peanuts, walnuts, almonds, sesame paste, ham, pork, sweet cheese, etc.

[Treat]

adult t-cell leukemia before treatment considerations?

(a) treatment

this disease based on clinical classification of different treatment strategies and decisions, chronic type, or smoke type to use more symptomatic and supportive treatment of patients with a positive control infection and improve organ function mainly when there is disease progression or acute changes in time, be considered an active treatment. Acute-or lymphoma-type ATL, although the use of chemical, biological and other active treatment measures, but the effects are poor, with a median survival of 2-6 months.

1. chemical treatment of the most commonly used treatment options for VEPA program (vincristine 1mg / week, once every 6 weeks; cyclophosphamide 300mg / d, No. 8,22, 29 days; prednisone 40 ~ 60mg / d, three days per week; doxorubicin (adriamycin) 40 ~ 60mg / d, the first 1,22 days) application of this treatment of 322 cases of patients, patients with complete remission 7l (22%). Classic CHOF program efficacy is not satisfactory, 59 cases in 10 patients (17%) complete remission. Other available options include the CVP program, MACOP-B program, ProMACE-MOPP programs, treatment is not ideal. Recently, japanese scholars use LSG15 program (7 cycles VCAF, AMP and VECP program) plus granulocyte growth factor (G-CSF) treatment of 96 cases of advanced ATL patients, complete remission in 33 cases (35.5%), partial remission in 42 cases ( 45.2%), median survival time of 13 months, 2-year disease-free survival rate was 31.3%, significantly higher than other regimens. Currently, chemotherapy is still the treatment of advanced primary means of ATL.

2. vitamin a acid (all-trans retinoic acid) vitamin a acid (ATRA) may affect or block the ATL cells Tax / NF-KB signaling pathway, has been used ATL in patients with clinical resistance to chemotherapy treatment, clinical efficacy remains to be verified.

3. Interferon-2b interferon for treatment of ATL, but poor efficacy alone. Recently, there have been several stories interferon α-2b with the antiviral drug zidovudine (AZT) in combination therapy in patients with ATL and get a certain effect. white and the use of interferon α-2b 250 million to 10 million U, subcutaneously, 1 / d and zidovudine (AZT) 50 ~ 200mg, orally, 5 times / d, treatment of 18 patients with ATL, in addition to 6 patients unable to evaluate the efficacy, 1 case of continuous complete remission 21.6 months, 2 patients sustained partial remission were 3.7 months and 26.5 months. Matutes and other methods of treatment of 15 cases with the previous ATL patients have received various treatments, including eight cases of complete remission or partial remission, the other seven cases were ineffective.

4. immunotherapy IL-2R (Tac) monoclonal antibody therapy for ATL. Clinical data show that anti-Tac treatment of 20 cases of ATL were, 1 patient had transient relief is not sure. Four cases of partial remission, 2 patients achieved complete remission. With immune anti-Tac also radionuclides (90Y) cross-linking for the treatment of ATL patients, in 15 patients receiving treatment, eight had a partial response, 2 cases of complete remission.

5. Utsunomiya and other hematopoietic stem cell transplantation with allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with ATL treatment of 10 cases, 9 cases for those of kinship, one case of unrelated donor marrow, with a median disease-free period of 17.5 months generated, indicating that treatment of allo-HSCT for ATL may be some effects.

6. osteolytic lesions and hypercalcemia, available pamidronate 90mg, intravenously, once a month.

(b) the prognosis

poor prognosis of this disease. lymphoma Study group of Japan reported 854 cases of ATL patients, median follow-up time (calculated from the time of diagnosis) was 14 months, 585 patients (68.5%) had died, 269 patients (31.5%) still alive, with a median survival of only 6 months, 2 and 4 years expected survival of 28% and 12%. poor prognosis-related factors: ① generally in poor condition; ② lactic dehydrogenase hyperlipidemia; ③ Age> 40 years of age; ④ multi-site involvement; ⑤ hypercalcemia; ⑥ CD4-, CD8-; cd4 +, cd8 or CD4-, CD8; ⑦ Ki-67> 18%.

adult t-cell leukemia Chinese medicine treatment methods

No information

western adult t-cell leukemia treatment

No information

[Examine]

myxedema face should be how?

mainly as follows: apathy, dull, cheek and eyelid edema, the pressure of the depression, pale greenish yellow, dry facial skin rough and dull and flexibility, nose, lips, tongue thickening, hair dry, sparse eyebrows and sometimes from the outside off.

[Diff]

myxedema face with which the symptoms easily confused?

myxedema face of the differential diagnosis:

1. acute face: the performance of flushing, shortness of breath, nasal flap, face pain, irritability and so on. Common in lobar pneumonia, malaria and so on.

2. chronic diseases face: the performance of gaunt, pale gray or pale, his eyes dim, Shenpi lack such power. Chinese medicine is a weakness of righteousness xu Zheng; western view prevalent in the chronic wasting disease. Such as cancer, chronic hepatitis, cirrhosis, tuberculosis, etc.

3. anemia face: the performance of pale, pale tongue, Shenpi fatigue, palpitation and shortness of breath. Chinese medicine is a blood deficiency xu Zheng; Doctors believe that common in anemia.

4. hyperthyroidism face: the performance of the eye protruding, split large eyes, eyes Yuan Deng, frightened eyes, pale and thin, excitement, anxiety, irritability and so on. Chinese medicine is liver qi stagnation, lack of fire, liver and lack of hyperactivity; western medicine is seen as typical of hyperthyroidism face.

5. hypothyroidism face: also known as myxedema face, manifested as pale or yellow, dull eyes, facial swelling, thick lips, large tongue, eyelid thickening , dry skin rash inelastic, a small but deep wrinkles, dry thinning hair, eyebrows off the most obvious. Chinese medicine is qi deficiency with deficiency syndrome; Doctors believe that common in hypothyroidism. To testosterone deficiency and hypopituitarism can also be found such a face.

6. acromegaly face: head larger, longer face, large and protruding lower jaw, malar prominence, eyebrow uplift, increased ear, nose, tongue hypertrophy, dental and other rare and dislocation. The face more common in acromegaly.

7. mitral valve face: face, also known as rheumatic heart disease, manifested as pale yellow and swollen, dark red cheeks, lips cyanosis, tongue color dull, palpitation and shortness of breath. Chinese medicine is a blood stasis, qi and yang deficiency is caused by heart; western medicine is considered more common in rheumatic heart disease, is an important feature of mitral stenosis, or pulmonary heart disease is also seen in some patients with congenital heart disease .

8. typhoid face: the performance of slow, indifferent expression, tongue, little coating, shortness of breath, disinclination to talk, and even consciousness. Chinese medicine is qi and yin Deficiency, is a febrile disease areas; Doctors believe that common in typhoid fever, cerebrospinal meningitis, encephalitis and other diseases.

9. tuberculosis face: the performance of pale, cheeks red as rouge, weight loss. The face more common in active tuberculosis.

10. moon face: the performance of face red fat, round like the full moon, or even invisible from the front, ears, nose visible from the side. As the accumulation of fat cheeks fall, pushed the nose down, mouth fission small mouth and the emergence of deep groove between the cheek, looking Hongnen, vellus hair increase, acne often raw, lips can be seen a small beard. Doctors believe that the long-term use due to cushing syndrome, or adrenocorticotropic hormone skin caused by excessive corticosteroids's characteristic face.

11. nephrotic edema face: the performance of pale edema, skin tight and dry, especially in early morning eyelid edema is most remarkable, and there are more than mean pressure frontal depressions . In addition to this face seen in a variety of kidney disease, but also seen in heart failure, malnutrition, facial angioedema, severe asthma, whooping cough and so on.

12. proliferator-face: the performance of mouth breathing, nose shape dysplasia, wide flat nose bridge, small nose wilt, short thick lips, turned on the lips, nasolabial shallow, underdeveloped jaw, slurred speech, hard of hearing, dull, dull expression. The face seen in children adenoid hypertrophy.

13. dying face: the performance of face haggard, pale or lead-gray, indifferent, present state of absence, sunken eyes, limbs Jueleng and so on. Chinese medicine considers death or death yin yang areas; Doctors believe that common in bleeding, severe shock, dehydration and acute critical illness.

14. lion: also known as lepromatous leprosy face, showing uneven end face covered with plaques, eyebrows, eyelashes, hair, beard off some or all of the , the shape of "lion." face seen in this tumor type leprosy. Lion is found in bone fibrous dysplasia of bone, skull area periostitis, osteitis deformity or trauma.

15. myopathy face: the performance of the eye can not open nor closed, in a state of half a sheet, or the amount can not be wrinkled, lips prominent hypertrophy, under the lower lip hanging, not closed. The face seen in muscular dystrophy or myasthenia gravis.

16. Down's syndrome face: high performance external border, tilt out the top of the eye fissure, exophthalmos, or strabismus, or tremor; nasion Department of flat, the nose toward the on.

mainly as follows: apathy, dull, cheek and eyelid edema, the pressure of the depression, pale greenish yellow, dry facial skin rough and dull and flexibility, nose, lips, tongue thick, dry hair, sparse eyebrows and sometimes from the outside off.

[Disease]

myxedema face-related diseases

elderly hypothyroidism during pregnancy subclinical hypothyroidism hypothyroidism thyroid dysfunction, hypothyroidism cardiomyopathy elderly hypothyroidism hypothyroidism caused anemia chibai the wind, edema

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