02:19,12,Jul,2005 | (436/0/0) | Original

for idiopathic thrombocytopenic purpura


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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 bleeding, 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


2010-10-16 12:59 reply
for idiopathic thrombocytopenic purpura

Although the hormone can increase blood platelet, but the hormone reduced or stopped, the platelet will decline; frequent use of hormones, adverse effects on the human body is very large, while the treatment can also cause such as central obesity, hypertension, diabetes and other diseases. Other treatments such as platelet transfusions, splenectomy and other treatments, although can play a certain effect, but consumption of fast, high cost, side effects, most patients with unbearable.
Thrombocytopenic purpura is a common bleeding disorder, occurs in children and young people, particularly young women is more common. Clinical manifestations and skin petechia petechiae, mucocutaneous bleeding, fraudulent claims epistaxis, hematemesis, hematuria, blood in the stool, visceral bleeding. The disease belongs to Chinese medicine, "fat spot", "Blood", the cause: Since toxic heat flaming, gas is not taken blood, causing blood Wang Xing; or may be solid liv
er spleen, liver restricting Ling soil, blood and spleen does not cause the system disease. The disease can cause long-term healing of the spleen or liver and kidney deficiency. TCM treatment is roughly: Liver Fu spleen, Yin Jiang Huo, nourishing Qi.
U.S. security rather blood blood-dimensional, strictly in accordance with requirements by the Ministry of Health for basic research and modern medicine Xiyuan Hospital of China Academy of Traditional Chinese Medicine, Union Hospital, Tongji Medical University, and a number of clinical studies confirm its authority significantly better than the control group. It can be used to increase platelets for idiopathic thrombocytopenic purpura, skin and mucous hemorrhagic disease, and contribute to the proliferation of blood cell differentiation, maturation, release, thereby increasing white blood cells, improving immune function.
Clinical symptoms of idiopathic thrombocytopenic purpura, skin and mucous membranes or internal organs to clinical bleeding as the main performance, serious bleeding may have other parts such as nose bleeding, bleeding gums, excessive menstruation or severe hematemesis, hemoptysis, hematochezia, hematuria and other symptoms, intracranial hemorrhage is the cause of death in this disease. Clinically divided into acute type and chronic type, the former is more common in children, which occur in women under 40 years of age. Female: Male is about 4:1. Period of the disease found in children of all ages, sub-acute (≤ 6 months) and chronic (> 6 months) of two types. Children mostly during the acute ITP, more common in infants and young children during the 7 years of age decreased significantly. Higher incidence in spring. No previous history of bleeding, the incidence of sudden, severe bleeding, bleeding or bleeding at the same time not long ago often suffer from respiratory infections. Chronic cases, but not significantly more common in school-age age of the peak period, the majority of latent disease, less bleeding, about 10% of patients from acute to chronic. Can also be divided into four degrees according to the disease: â‘  mild: Platelet <100 × 109 / L (10   / mm3) and> 50 × 109 / L, only in post-traumatic bleeding; â‘¡ Moderate: platelet ≤ 50 × 109 / L and> 25 × 109 / L, no extensive bleeding; â‘¢ severe: platelets <25 × 109 / L and> 10 × 109 / L, see the extensive bleeding, bleeding at the injury. â‘£ very severe: platelets <10 × 109 / L, spontaneous bleeding, life-threatening (including intracranial hemorrhage). ITP is characterized by bleeding in the skin, mucous membranes, extensive bleeding, mostly scattered in the size of the needle intradermal or subcutaneous bleeding, formation of petechiae or ecchymoses; limbs more, but spots for the systemic bleeding or hematoma; some patients to a large number of epistaxis (about 20% to 30%) or chief complaint of bleeding gums. Common hematemesis or melena, bleeding nose and mouth to swallow mostly caused by gastrointestinal bleeding are of real and rare. Subconjunctival hemorrhage are also common symptoms. Occasionally gross hematuria. About 1% of patients with intracranial hemorrhage, a major cause of death ITP. Adolescent girls can be seen menorrhagia. Other parts of the bleeding, such as the chest, abdomen, joints, etc., are extremely rare. In addition to skin and mucous membrane bleeding, but only 10% to 20% of patients had mild splenomegaly. The incidence of acute exposure is often accompanied by fever. In severe cases, bleeding may be beneath bloody anemia, hemorrhagic shock can occur side. Often accompanied by the corresponding local hematoma symptoms, intracranial hemorrhage were headache, drowsiness, coma, convulsions, paralysis and other symptoms. In addition to patients with acute fulminant thrombocytopenia, but often accompanied by damage to the vessel wall, it is heavy bleeding. About 80% of the etiology and pathology of the sick child in about 3 weeks before the onset of a virus infection, mostly upper respiratory tract infection, about 20% of the patient's disease is a pioneer in rubella, measles, chicken pox, mumps, infectious mononucleosis syndrome , hepatitis, giant cell inclusion disease and other diseases. About 1% of cases due to injection of live vaccine after the onset. Therefore, the ITP is not a viral infection that the virus directly but rather because the immune mechanisms involved; because infection often 2 to 3 weeks after onset, and the majority of patients in the presence of platelet surface coating antibody (PAIgG) increased, causing platelets are destroyed by phagocytes. Antibodies in acute than chronic volume-based higher platelet destruction more. Some patients also thrombocytopenia purpura and autoimmune hemolytic; newborn mother suffers from the same half of the patients were disease; these phenomena support the ITP is the autoimmune disease. Diagnosis of idiopathic thrombocytopenic purpura, the bleeding as the main clinical symptoms, no liver, spleen and lymph nodes, blood platelet count <100 × 109 / L, mainly bone marrow cells, the total number of megakaryocytes increased or normal serum anti-platelet antibodies were detected (PAIgG, M, A), platelet life span shortened, and the exclusion of other diseases can cause thrombocytopenia diagnosis. Auxiliary examination 1. Blood and more severe bleeding is not no red, white blood cell changes, and occasionally abnormal lymphocytes, suggesting that the virus infection. Acute hemorrhage during or after repeated bleeding, decreased red blood cell and hemoglobin Constant, leukocyte, reticulocyte increase after the bleeding. Peripheral blood is the most important change is reduced to 100 × 109L platelets below the level of bleeding is proportional to the severity and platelet, platelet <50 × 109L visible when spontaneous bleeding, <20 × 109L when the bleeding obvious, <10 × 109L serious bleeding . Platelet morphology seen in patients with chronic large and loose, stained lighter; prolonged bleeding time, coagulation time of normal time, poor clot contraction or contraction; reduced prothrombin consumption, thromboplastin generation bad. Extreme reduction of platelets, due to the lack of platelet factor 3, can cause coagulation time and platelet life span is very short. 2. In severe cases, bleeding bone marrow hematopoietic function shows strong reaction. The total number of cases of acute megakaryocytic normal or slightly higher; chronic diseases increased megakaryocyte number, and more in 0.2 × 109L (200/mm3) or more, even as high as 0.9 × 109L 〔normal values (0.025 ~ 0.075) × 109L〕. Megakaryocytes Category: The original giant cells and immature megakaryocytes normal or slightly higher percentage; mature release of platelets is not significantly increased megakaryocytes, up 80%; and mature megakaryocytes release platelets rare. In order to diagnose the disease to the exclusion of leukemia or aplastic anemia bone marrow examination to be carried out. 3. Platelet antibody test was mainly on the platelet surface IgG (PA IgG) increased, the positive rate of 66% to 100%. Made simultaneous detection of PAIgG, PAIgM, PAIgA positive rate can increase. PAIgG specific changes of this disease is not increased in other autoimmune diseases can also be increased. But the non-immune thrombocytopenic purpura PAIgG not increased. In addition the system changes observed PAIgG the prognosis of ITP guide. General decline in the platelet PAIgG only increased the amount was reported for each platelet PAIgG> 1.1 × 10-12g of cases with hormone therapy is not valid, and each platelet PAIgG amount (0.5 ~ 1.0) × 10-12g of corticosteroid treatment in cases of good . Before splenectomy also indicates that if the high PAIgG surgery is not effective. Such as hormone therapy or splenectomy PAIgG return to normal after surgery the prognosis is good. PAIgG continued to increase as the prompt treatment is invalid. In addition platelet antibodies in serum can be measured, about 54% ~ 57% addition of the serum antibodies. However, platelet antibodies in serum free and platelet surface IgG positive rate was not parallel. 4. Platelets shorten the life of 51Cr or 111In isotope labeled ITP patients with platelet loss: the determination of platelet life span was shorter, or even only a few hours (1 to 6 hours, normally 8 to 10 days). Applications can also peer factors measured surface platelet count retention and destruction of parts (spleen, liver, lung, bone marrow). Platelet adhesion and aggregation applications, such as testing for chronic ITP patients can sometimes be measured by platelet function abnormalities. With the National Diagnostic Criteria 1. Several laboratory tests to reduce platelet count 2. Spleen does not increase or only slightly increased 3. Bone marrow megakaryocytes increased or normal, mature barrier 4. What should have five points at any point: (1) prednisone treatment is effective; (2) splenectomy treatment is effective; (3) PAIg increased; (4) PAC3 increased; (5) determination of platelet life span shortened. 5. Exclusion of secondary thrombocytopenia in the clinical differential diagnosis of diseases often need to identify the following: (1), aplastic anemia: fever, anemia, bleeding the three major symptoms, liver, spleen, lymph nodes small, with idiopathic thrombocytopenic purpura associated with anemia were similar, but generally severe anemia, total leukocyte and neutrophil number decreased reticulocyte not high. Red bone marrow, and blood particle system functio
nality to reduce, reduce or difficult megakaryocyte check see. (2) acute leukemia: ITP special needs and identification of white blood cells of leukemia is not increased, visible through the blood smears and bone marrow examination of immature white blood cells can be confirmed. (3) allergic purpura: haemorrhagic rash is symmetrical, the lower limbs is more common, many platelets, generally easy to identify. (4) Lupus Erythematosus: early can be expressed as thrombocytopenic purpura, when in doubt check the anti-nuclear antibodies and LE cells (LEC) can help identify. (5) Wiskortt-Aldrich syndrome: in addition to bleeding and thrombocytopenia, the merged body a wide range of eczema and is susceptible to infection, reduce platelet adhesion, and ADP, epinephrine and collagen aggregation does not occur. Recessive genetic disease Property care, baby disease, more than 1 year old died. (6) Evans syndrome: is characterized by concurrent autoimmune thrombocytopenia and hemolytic anemia, Coomb
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