Health Worlds

Epidemiology of pathological low incidence of TTP, 1:100 million per year. However, statistics show that China is increasing the number of cases, more than 500 cases have been reported; abroad only
Thrombotic thrombocytopenic purpura pathological picture of the incidence of the United States each year are 500 to 1,000 cases, if coupled with a certain number of patients do not typically have to increase. To female patients more than 20 to 60 years old, no geographical or racial differences. Cause of the cause of TTP has not been fully elucidated. Most patients had no incentive to be found, called the primary. Small number of patients may have genetic, immunological abnormalities, infection, pregnancy or medication history and other background, such as secondary to drug allergies (antibiotics such as penicillin, sulfa drugs, iodine, phenytoin, chloroquine, aspirin, phenacetin, procainamide, oral contraceptives, etc.); poisoning (CO, dyes, paint, bees and dogs, etc.); infection (bacteria, rickettsia, respiratory and intestinal viruses, influenza, herpes simplex Coxakie B, Mycoplasma pneumonia is, etc.); autoimmune diseases (such as rheumatoid arthritis, spondylitis, SLE nodosa Sjogren's syndrome); late pregnancy cancer chemotherapy (mitomycin common), cyclosporin A, taking estrogen hormones and progesterone, and other reported HIV infection in vaccine-related TTP, accompanied by severe anemia and thrombocytopenia, and mild renal insufficiency. Pathogenesis on the pathogen


esis of TTP is inconclusive, since the past suggested that endothelial cell damage may play to promote the aggregation of platelets in the formation of blood vessels
Pathological picture of platelet plug. Supporters of this idea gradually reduced. More popular theory is too strong for the ability of platelet aggregation, platelet plug formation, adhesion to the vascular endothelium, causing the secondary changes, possible mechanism are: 1. Small vessel disease, significant lesions in the capillaries, the electron microscope thrombosis can be seen under the endothelium before the pathological changes of the lesions occur in the microcirculation, it can cause microangiopathic red blood cell solution, which can also increase the local thrombosis, if the lesion is more extensive and may result in thrombocytopenia, TTP reported in the literature may be associated with disk-shaped or systemic lupus erythematosus, rheumatoid arthritis, rheumatoid spondylitis, multiple nodules of the arteries go far, the characteristics of these diseases have a certain degree of vasculitis lesions. 2. Disseminated intravascular coagulation (DIC) thrombotic thrombocytopenic purpura as the main pathological changes of microcirculation thrombosis, it was considered thrombotic thrombocytopenic purpura is the essence of DIC. Takahashi and other 10 cases were detected in patients with acute TTP plasma thrombin - antithrombin complex (TAT) and plasmin- 2-antiplasmin complex (PAP). TAT and PAP patients were higher than normal controls, but no correlation between the two. Remission in 5 patients, the PAP and TAT values decreased significantly, but no other indicator of hemostatic abnormalities. 3. Prostacyclin (PGI2) synthesis in the absence of some of the reduction or prevention of plasma PGI2 degradation factor. About 60% of TTP patients with whole blood or plasma can be alleviated, if treated with 5% albumin is disease progression. The study, patients with normal PGI2 generation, but its degradation rate accelerated, suggesting that there is a normal plasma PGI2 to prevent rapid degradation of the factor, it does not exist in the albumin. Half-life of this factor is 2 weeks, it can prolong the biological activity of PGI2 its deficiency can result in decreased PGI2, associated with microvascular thrombosis. Hensby et al (1979) reported that patients with TTP plasma 6 keto-PGF1a reduce further confirm this theory. 4. Immunity doctrine was that in thrombotic thrombocytopenic purpura seen in the immune system damage caused by vascular disease, endothelial cells labeled with 3H proline, incubated with TTP plasma of patients with 42% of anti-endothelial cells can control group, only 8.6% in addition TTP serum IgG with endothelial cells can kill rate of 70%, while the control group, only 16.8% of clinical onset is often abrupt, the typical cases of fever, fatigue, weakness, a small number of slow onset, a precursor of muscle and joint pain and other symptoms, after the rapid
Platelet pathological picture have other symptoms. Have to pleurisy, Raynaud's phenomenon, women complained of vaginal bleeding for the first. 1. The typical clinical manifestations are the following characteristics (1) bleeding caused by thrombocytopenia: the skin and mucous membrane mainly manifested as petechia, ecchymosis or purpura, epistaxis, retinal hemorrhage, genital urinary tract and gastrointestinal bleeding, severe by intracranial hemorrhage, the extent depending on the degree of platelet reduction without one. (2), microangiopathic hemolytic anemia: different degrees of anemia. About 1 / 2 of the cases of jaundice, 20% had hepatosplenomegaly, few cases have Raynaud phenomenon. (3) neuropsychiatric symptoms: the first clinical manifestation of typical cases found in the nervous system, which often determine the severity of thrombotic thrombocytopenic purpura Silverstein prognosis of 168 cases reported in 151 patients with neurological symptoms (90%), and its characterized by fluctuating symptoms, a transient initial period, 50% can be improved, and repeated attacks. Patients had varying degrees of consciousness disorder, 30% had headache and (or) aphasia, speech is unclear, vertigo, convulsions, spasm, paresthesia, visual disturbances, perceptual disturbances, disorientation, mental confusion, delirium, drowsiness, coma, cranial nerve palsy. 45% had paresis, sometimes paralysis, can be resumed within a few hours. Variability in the performance of the nervous system as thrombotic thrombocytopenic purpura characteristics. These performance and cerebral circulation disorders. (4) kidney damage: gross hematuria is not common. Eventually severe acute renal failure. (5) fever: 90% of patients have fever, fever can be in a different stage of disease, mostly moderate. The reason is unknown, may be related to the following factors: secondary infection, but blood cultures were negative; hypothalamic thermoregulatory dysfunction; necrosis; hemolysate release; antigen-antibody reaction that macrophages and granulocytes damaged, and the release of endogenous pyrogen. (6) Other: multifocal hemorrhagic myocardial necrosis, cardiac muscle micro-thrombosis, may be complicated by heart failure or sudden death, ECG showed repolarization abnormalities or a variety of arrhythmias, autopsies for acute myocardial infarction. The performance of pulmonary insufficiency also reported that due to the pulmonary vascular involvement. 2. Sub-type (1) acute type: more common in the rapid progress of the explosion, 7 to 14 days the onset of symptoms. About 75% of patients within 3 months after the onset of death. Common cause of death was hemorrhage, cerebral vascular accident, or heart lung and kidney failure. chronic form: rare, mitigation and aggravation of one after another, the course for several months or years. relapse type: As advances in treatment, may relapse 1 to 5 times, survived an average of 9 months to 12 years, median survival of 5.1 years. Congenital Type: monozygotic twins have occurred TTP. (2) secondary type: Pregnancy complicated by TTP, most occur in eclampsia, pre-eclampsia or eclampsia may also occur prior to 1 week after production, pathogenesis may be related to increased circulating immune complexes. A group of 151 patients reported 23 cases of TTP which exist SLE. Another report of autoimmune hemolytic anemia eventually occurs TTP, pancytopenia, autoimmune disorders occur simultaneously TTP, ITP also interval of 4 months after the tumor can cause TTP TTP, such as lymphoma, can be 2 to 6 months after TTP. Diagnosis 1, diagnostic criteria and the basis for Zhang Nan editor of the "standard of diagnosis and treatment of blood diseases," a book based on the development of China's foreign diagnostic criteria for the literature as the main diagnosis was based on
Nucleated red blood cell pathological picture, according to: (1), microangiopathic hemolytic anemia: positive cell anemia is more pigmented and severe anemia. microangiopathic hemolytic. A. jaundice, dark urine, urine bilirubin negative, with occasional high hemoglobin hyperlipidemia, high nocturnal hemoglobinuria with hemosiderin urine disease. B. red blood film br 2%, occasionally nucleated red blood cells. C. reticulocyte count increased. D. highly proliferative bone marrow erythroid, granulocyte / red ratio decreased. E. High-bile pigment of red blood viremia, the main indirect bilirubin. F. Plasma haptoglobin (haptoglobin), heme binding proteins (hemopexin) reduction, lactate dehydrogenase increased. (2), thrombocytopenia and bleeding tendency: often significantly lower platelet count and blood film shows large platelets. skin and (or) other parts of the bleeding. the number of megakaryocytes in the bone marrow is normal or increased, may be associated with mature obstacles. platelets shortened life expectancy. (3) neuropsychiatric disorders: headache and personality changes can occur, mental confusion, abnormal consciousness, language, sensory and movement disorders, convulsions, stupor, positive pathological reflex, etc., and there is often a transient, repeated, diversity and variability characteristics. Exist as more than three triad. (4) kidney damage: the performance of laboratory abnormalities such as proteinuria, urine appear red blood cells, white blood cells and the tube, blood urea nitrogen, creatinine level rise, severe cases can be seen with nephrotic syndrome or renal failure. (5) fever: mostly low, moderate. 2, the diagnosis based on histopathological diagnosis of TTP can be used as auxiliary conditions. Drawn parts, including the skin, gums, bone marrow, lymph nodes, muscle, kidney, spleen, lung and so on. Differences usually presents a small arteries, capillaries, there is uniformity in the "transparent-like" platelet thrombus, PAS staining. In addition, there are vascular endothelial cells, subendothelial "transparent-like" material deposited around the fibrosis of small arteries. Partial embolization may have necrosis but no inflammatory cell infiltration or inflammatory reaction. Typing the following. (1) According to the course type: Acute: rapid onset after cure at least 6 months without recurrence. Chronic: not completely cured, a long protracted course of disease. recurrent: cured within 6 months after relapse. Relapsed within 1 month for the recent recurrence, relapse after 1 month and late recurrence. Chronic and recurrent cases of about 7.5% of total number of cases. (2) According to the etiology of type: Idiopathic: no special cause to be found, the majority of cases are this type. secondary: a specific cause could be found, such as pregnancy, infection, cancer, drugs and so on. 3, diagnostic assessment (1) typical symptoms associated TTP has five: But many scholars believe that as long as with microangiopathic hemolytic anemia, thrombocytopenia, and neuropsychiatric disorders can be diagnosed triad of TTP, even scholars in the light of thrombotic thrombocytopenic purpura, prognosis dangerous, such as with microangiopathic hemolytic anemia and thrombocytopenia, the exclusion of the DIC and other thrombotic microangiopathy after that should be considered thrombotic thrombocytopenic purpura, and begin treatment as soon as possible. (2) TTP diagnostic criteria lack specificity: only comprehensive clinical and laboratory findings, and other thrombotic microangiopathy than after, can make the diagnosis. In clinical manifestations, diagnosis of most neuropsychiatric disorders, but the performance varied, and can be a transient need to be carefully understood. (3) when the diagnosis of TTP: Find a variety of predisposing factors should be noted, such as infection, drugs, immune dysfunction, etc., with all kinds of new drugs continue to come out, reports of drug-induced TTP gradually increased, and some theory can be used to treat TTP drugs, such as ticlopidine (ticlopidine), has also been shown to induce TTP, should be extra vigilant. 4, the differential diagnosis 1. Disseminated intravascular coagulation (DIC) is not a serious disease in patients with hemolytic anemia and transient variability neuropsychiatric symptoms, there is severe bleeding, thrombocytopenia, decreased clotting factors, secondary fiber evidence for dissolved proteins, protein C measured significantly decreased tissue factor antigen were significantly higher TTP thrombocytopenia, there are broken red blood cells, clotting factor generally does not reduce determination of the normal protein C, FDP did not increase or slightly elevated, 3P negative, tissue factor antigen slightly decreased 1 month after treatment, no significant increase, and its inhibitor (TFPI) was significantly higher, but sometimes TTP and the more difficult the identification of DIC (Table 1) 2.Evans syndrome, autoimmune hemolytic anemia with immune thrombocytopenic purpura, renal impairment may have performance Coombs test was positive, no deformity and broken red blood cells, without neurological symptoms. 3. Systemic lupus erythematosus (SLE) the relevant section of the symptoms, kidney damage, neurological symptoms, and hemolytic anemia, skin lesions, LE cells positive, no deformity and fragmentation in peripheral blood erythrocytes. 4. Hemolytic uremic syndrome (HUS) tend to the same disease TTP and HUS are two different clinical presentations, is a polygenic disease, and is thrombotic microangiopathy (TMA). HUS lesions mainly to kidney damage, most of them children under 4 years old, adults occasionally, disease often have symptoms of upper respiratory tract infection and gastrointestinal symptoms, acute renal failure was the most prominent, in addition to microangiopathic hemolytic and platelet reduction, in general, non-psychiatric symptoms (Table 2). 5, the laboratory normal red blood cells labeled with 51Cr loop in the half-life in patients with TTP, only 3 days (normally 25 to 26 days). Indirect bilirubin, manifested as proteinuria, microscopic hematuria and tubular urine, 40% to 80% had mild azotemia, creatinine clearance rate. 1. The performance of peripheral blood of patients have anemia, positive cells are pigmented, 1 / 3 of patients with hemoglobin 30%), also reported first and then decreased by a median value of 6.6% to 19%. Persistent thrombocytopenia 92%, median (8 ~ 40.4) 109 / L. 60% of white blood cell levels were leukemoid reaction rare, but can be significantly shifted to the left and immature granulocytes. 2. Bone marrow red blood cell system, a significant proliferation of the normal number of megakaryocytes or increased, the majority of immature megakaryocytes, were mature obstacles. 3. Check out the bleeding time normal coagulation, blood clot contraction poor beam arm test positive, prothrombin time prolonged, accounting for 20%, partial thromboplastin time prolonged, accounting for 8% of fibrinogen can be reduced, less than 1.5g / L, 7%, fibrinogen survival and convert most normal, a few mildly shortened. FDP-positive 70%, prothrombin time prolonged, 48%, but generally no change in the typical laboratory DIC factor , normal. PGI2 reduced. TM, PAIgG increased and decreased with the improvement of the disease. HIV-1 infection, endothelial cell injury PAI, W factor increased, PS reduced. Treatment 1. Plasma exchange therapy is the preferred method of treatment. Since 1976, the TTP of the effects after treatment with this Law, the rapid increase of 67% to 84%, so much TTP prognosis
Megakaryocyte pathological picture changed. That it can remove the body to promote platelet aggregates, supplementary normal anti-aggregates, shall be done. Daily dosage is 40 ~ 80ml/kg of fresh frozen plasma, at least 5 to 7 days. Treatment is effective (usually in 1 to 2 weeks) decreased the serum LDH concentration, increased nervous system symptoms of platelet recovery. Serum LDH concentration is usually decreased to 400U / L, they can stop the plasma exchange. Plasma exchange therapy should not use the cold sediment, so as not to trigger a large number of factor W intravascular platelet aggregation, platelet transfusion should be considered taboo. 2. Adrenocorticotropic hormone used alone these drugs are less effective for the treatment of TTP. Generally started with prednisone 60 ~ 80mg / d, if necessary, increased to 100 ~ 200mg / d. Those who can not also be appropriate oral dose of hydrocortisone or dexamethasone. Primary treatment of acute TTP are not unified, hormones may increase the platelet thrombus formation. Also believe that hormones alone only 11% effective, should be combined with other methods have a higher efficacy. 3. Immunosuppressive drugs commonly used by particular PAIgG increased vincristine (VCR). Schreeder et al (1983) with vincristine (VCR) treatment of 2 cases of TTP are in remission, 1 cases of the latter part of effective treatment with plasma exchange, splenectomy again temporarily effective, but by vincristine (VCR) therapy achieved complete ease. Joel et al (1985) reported 1 case of 57-year-old women with chronic TTP complicated W factor deficiency, the prednisone, azathioprine and plasma exchange treatment, 21 days after TTP remission, W factor disappears. 4. Anti-platelet aggregation agent such as indomethacin (indomethacin), aspirin (600 ~ 2400mg / d), dipyridamole (dipyridamole) (200 ~ 600mg / d), dextran -40 (500ml, 2 times / d, 14 days). Myers and so that at least some patients with TTP, anti-platelet drugs on the initial remission and maintaining remission TTP plays an important role. Therefore, in the comprehensive treatment of anti-platelet drugs play a secondary role, and achieved remission as maintenance therapy. Duration of treatment up to 6 to 18 months, premature withdrawal easy to relapse, one with anti-platelet drugs less effective, often combined with adrenal cortex hormones. Prognosis and prevention of prognosis in the past thrombocytopenic purpura thrombotic poor prognosis, short course, case fatality rate was 80% to 90%. Pregnancy, particularly high fatality rate, 69% of perinatal mortality, causes of death in the central nervous system hemorrhage or thrombosis, followed by renal failure, there are partial remission in a few months, several years recurrence after complete remission minority 12-year recurrence, a high degree of activation of the immune system, poor prognosis. Aggressive treatment of primary disease prevention, to avoid or reduce sulfonamides, oral contraceptives, to prevent poisoning and infection.


Friends Links:Automation Control Blog
Automation Products Order Numbers