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1 F Definition: idiopathic thrombocytopenic purpura is a group of unknown cause immune-mediated destruction of platelets due to excessive bleeding disorders
Characteristic features: 1, 2, mucocutaneous and visceral bleeding, thrombocytopenia 3, megakaryocyte maturation disorder 4, 5, shortened platelet survival time, there anti-platelet glycoprotein autoantibodies
According to the clinical situation raised an army divided into acute type and chronic type, the former is common in children, often their own mitigation, which is more common in adult women, often recurrent
Excessive destruction of platelets macrophage is the direct cause of disease thrombocytopenia
Etiology and pathogenesis: 1, immune factors, 2, spleen factors (platelet antibody production sites and sites of platelet destruction)
Clinical manifestations: thrombocytopenia caused by performance of various types of bleeding
Laboratory tests: platelet-related checks, bone marrow, and other (such as with iron deficiency anemia)
Diagnosis: There is no "gold standard" test to establish the diagnosis
Diagnosis: multiple testing reduced platelet counts, the performance of various types of bleeding, not the spleen, bone marrow megakaryocyte maturation disorder, glucocorticoids or splenectomy treatment is effective, rule out other reasons for thrombocytopenia caused by
Treatment: General treatment: rest, avoid strenuous activities and trauma, bed rest, local bleedi


ng, hemostatic applications
ITP's first-line treatment: corticosteroids, IVIG
Second-line treatment of ITP: splenectomy, rituximab
Three-line treatment for ITP: TPO receptor agonists, anti-CD52 monoclonal antibody, a second-line drug combination therapy, combination chemotherapy, hematopoietic cell transplantation liver 12:59 reply
I hope my answer can be helpful to you, you said not specific enough, do not worry, take a look at the following information in control again, and wished speedy recovery of patients. Idiopathic thrombocytopenic purpura idiopathic thrombocytopenic purpura is a reduction in peripheral blood platelets causes bleeding disorders. The main clinical manifestations of the skin, mucous membranes and a significant (or) organ bleeding. Can be divided into acute type and chronic type. No gender difference in the incidence of children, in adults, male and female incidence rate ratio of about 1:4. (A) acute type 1. Symptoms: bleeding skin, ecchymosis, mucosal Xuepao lips, nasal bleeding, bleeding gums, tongue bleeding. Visceral bleeding may have blood in the urine, black stool, sputum and vomit mixed with bloodshot eyes, or blood clots. Women can have vaginal bleeding. In severe cases, intracranial hemorrhage, patients often can lead to death. 2. Signs: skin and mucous membrane shows extensive bleeding, ecchymosis. Injury and injection site hematoma or even visible ecchymosis. Intracranial hemorrhage may have severe headache, blurred vision, disturbance of consciousness, paralysis and convulsions. 3. Complication: bleeding a large quantity, may be complicated by blood loss, anemia, low blood pressure, or even hemorrhagic shock. (B) chronic type 1. Symptoms: bleeding lighter than the acute form, and limitations. Can be expressed as recurring skin, mucous membrane bleeding, ecchymosis, or nasal bleeding, gingival bleeding. Less severe visceral hemorrhage. Increased menstrual flow is more common in some patients can be the only symptoms. 2. Signs: skin scattered bleeding points, ecchymosis. Injection site ecchymosis or injury. A small number of patients may have mild swelling of the spleen. 3. Complication: chronic bleeding, increased menstrual flow, especially in patients with hemorrhagic anemia may occur. Laboratory 1. Blood: thrombocytopenia, WBC and RBC, hemoglobin levels were normal. Large amount of bleeding, could have reduced the level of red blood cells and hemoglobin. More acute platelet <20 109 / L, platelet levels often chronic 50 109 / L or so. Microscope, mean platelet volume is too large, easy to see that large platelets. 2. Bone marrow: acute type of bone marrow megakaryocytes were normal or slightly increased the number of chronic-type giant cells were significantly increased. Megakaryocyte developmental disorders, with acute type were significantly. Produced a significant reduction in board giant, often "30%. Normal myeloid and erythroid. 3. Platelet associated antibodies (PAIg) measured: 80% to 90% of patients PAIg rise to IgG is most prevalent, followed by IgM, IgA, a small number of patients may have two or more antibodies simultaneously; platelet complement (PAC3) positive, 4. hemostasis and coagulation tests: hemostatic abnormalities were associated with thrombocytopenia related to the vast majority of patients (90%) of platelet survival time was significantly shortened. Diagnosis is based . Extensive hemorrhage involving the skin, mucous membranes and internal organs 2. Many tests to reduce blood platelet count 3. Splenic little or mild increase 4. Megakaryocytes increase in the number of bone marrow or normal, accompanied by mature barrier 5. Have the following Among those five A: prednisone treatment is effective; splenectomy treatment is effective; PAIg positive; PAC3 positive; shortened platelet survival time 6. exclusion of secondary thrombocytopenia Treatment (1) General treatment: the incidence of acute cases are mainly 1 to 2 weeks in the heavy bleeding, so the early stages, should reduce activities to avoid injury, especially head injury, severe by bed rest. Should actively prevent and control infection, aspirin can cause bleeding, should also be avoided. Provide adequate liquids and digestible diet to avoid cavity mucosal injury. To reduce the bleeding tendency, often to a large number of vitamin C and P. Local bleeding hemostasis. Beijing Children's Hospital 811 cases only 226 cases of acute ITP in the general treatment, recovered within 6 months in 78.3%, and the other no significant difference between treatment group, indicating the general case do not need to give special treatment. If serious bleeding or suspected intracranial bleeding, should actively adopt various measures to stop bleeding. Bleeding is not heavy or chronic cases in remission were no special treatment, but should avoid injury, prevention of infection, and sometimes mild respiratory infection can cause severe relapse. Severe or long treatment of bleeding should be the following special treatment. (2) of fresh blood or platelets input: only when used as emergency treatment of severe bleeding. Because there is blood in patients with anti-platelet antibodies, platelets can quickly enter the destruction of short-lived (minutes to hours). Therefore, transfusion of blood or platelets can effectively improve the platelet count. But some believe that platelets can be rapidly reduced after the capillary fragility, and reduce the bleeding tendency. (3) adrenal cortex hormones: effect of hormones is generally believed that is due: reduce capillary permeability and reduce the bleeding tendency; reduce the immune response, and reduce the generation and inhibition of PAIgG spleen macrophages cells with antibodies platelet phagocytosis. Therefore, in ITP patients with early application of a large number of hormones, the bleeding can be quickly improved. Still advocates within 1 month of onset (especially 2 weeks) condition for the onset of moderate or long, but the disease is more severe patients should be given hormone treatment. Principle is that early treatment, a large, short-range. Generally with prednisone 60mg/m2 · d (2mg/kg · d) 2 ~ 3 times or early in the morning once orally. If severe bleeding, prednisone can be used to 120mg/m2 · d orally or hydrocortisone 400mg/m2 · d or dexamethasone 10 ~ 15mg/m2 · d intravenous drip until the blood is better to prednisone 60 . g/m2 · d. General medication 3 weeks, the longest no more than 4 weeks, gradually tapered to discontinuation of treatment. Even after stopping thrombocytopenia, bleeding is not obvious as long as you can continue to observe, not to use hormones. If extensive bleeding occurs again, still with hormone therapy, to be replaced by the next day after the bleeding better, or only a small dose of medication to maintain, so that bleeding can not occur. Beijing Children's Hospital for children with acute critical type, the use of dexamethasone 1mg/kg · d intravenous infusion pulse therapy, every 3 to 4 days to reduce 1 / 4 dose. Strong pine after three weeks to 40 ~ 60mg/m2 · d orally, to achieve better results. Avoid prolonged use of adrenal cortex hormones. Adequate treatment of chronic patients need 3 to 4 weeks after the bleeding to stop, you can reduce, when the platelets rose to 50 109 / L, to observe the withdrawal. (4) high-dose intravenous gamma globulin: the more severe hemorrhagic children, may also enter the high-dose intravenous gamma globulin purification (IgG), about 0.4g/kg · d, for 5 days. About 70% to 80% of patients can improve platelet counts, particularly for chronic patients temporarily splenectomy surgery trend. However, the high cost of such refined products, sometimes difficult to promote. (5) immunosuppressive drugs: hormone therapy with no response time can still try vincristine 1.5 ~ 2mg/m2 (maximum dose of 2mg / time) intravenously once a week; or every 0.5 ~ 1mg/m2 slow intravenous infusion of 250ml normal saline, once every 4 to 6 weeks for a course of treatment. Platelet increase seen after treatment, but most of the patients decreased after drug withdrawal, only a small number of long-term remission. Due to short-term effect, it is more suitable for surgery preparation. cyclophosphamide 2 ~ 3mg/kg · d orally or intravenously every time 300 ~ 600mg/m2, weekly, 1. More valid in the 2 to 6 weeks, such as ineffective can be discontinued for 8 weeks. Effective treatment may continue to 4 to 6 weeks. azathioprine 1 ~ 3mg/kg · d, a month before the general markedly. These immunosuppressive agents may be combined with corticosteroids. (6) Other drugs: In recent years domestic and international trial alkyne hydroxyl Androstene isoxazole (Danazol Danazol, DNZ), the non-masculine synthetic androgen treatment of refractory chronic ITP patients, immediate results were good, maintenance effect short time, so the need for splenectomy surgery being an increase of platelet a certain value. The role of T cells is considered to adjust the immune regulatory function, thereby reducing the production of antibodies and reduce macrophage elimination of platelets. Some people think that its efficacy and regulation of Fc receptor. More trials in adults and older children, oral administration of 200 ~ 400mg / d, treatment for at least 2 months. Side effects may have abnormal liver function, mild edema, rash, acne, skin bleeding occasionally fibrin solubility. However, due to lack of efficacy in children accurate and more expensive drug prices, side effects and more, the present treatment of ITP DNZ only as a three-line drugs. In addition, many units have tried ammonia domestic peptide (Zhao Jia extracted from the trotters, with more than 10 kinds of amino acids and trace elements) treatment. Also try cyclosporin A (Cy-A), plasma exchange therapy and recombinant -interferon therapy. (7), splenectomy and treatment: splenectomy in chronic ITP a response rate of 70% to 75%. However, indications for surgery should be strictly controlled, as the time delay splenectomy. More than splenectomy indications that there is a severe long-term or intermittent bleeding, application of long-term medical therapy or need a large number of hormones necessary to maintain hemostasis, duration of more than 1 year; or moderate bleeding more than 3 years duration; age of 5 later; the bone marrow megakaryocytes increased; PAIgG were moderately higher. However, severe bleeding or life-threatening surgical emergency operations are not subject to disease and age, can do an emergency splenectomy. In the use of hormone therapy before splenectomy patients before surgery and surgery should be intravenous infusion of hydrocortisone in the liquid, after the phasing out. Splenectomy, the splenic pedicle ligation to reduce bleeding after that, see, you can see within a few hours after platelet increase of up to 24 hours of platelet 200 109 / L, bleeding time is normal. Platelets of about 4 to 10 days reached a peak at about (300 ~ 900) 109 / L, up to 1000 109 / L or more. And then decreased gradually after several weeks in February is becoming normal. Generally believed that the higher the rise in platelets after long-term efficacy as possible. PAIgG drop of platelets after rise in parallel with platelets. Some patients with increased platelets after splenectomy is not high, bleeding and more significantly improved. When the platelets increased rapidly, should ensure adequate fluid intake, increased platelets after splenectomy 1000 109 / L or more should be given aspirin or dipyridamole and other drug treatment to prevent thrombosis. Most patients recovered after surgical treatment or to obtain longer-term mitigation. In order to reduce recurrence, surgery spleen must be removed. Splenectomy 5 years of age of the sick child, after splenectomy long-acting penicillin should be given once a month, or gamma globulin injections to prevent infection (especially pneumococcal infection) until 5 years of age.


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