idiopathic thrombocytopenic purpura (idiopathic thrombocytopenic purpura) - a disease that belongs to the group of hemorrhagic diathesis and is characterized by bleeding disorders due to lower levels of platelets in the blood. Content
etiology of the disease is not fully understood.Doctors distinguish between two forms of idiopathic thrombocytopenic purpura, depending on its cause:
main cause of idiopathic thrombocytopenic purpura is considered inadequate platelet production of megakaryocytes - the bone marrow cells - because of a violation of their functioning.
The basis of the disease is the destruction of platelet antibodies raised in the body.They can o
But sometimes the cause of trigger mechanism can not figure out at all.
After occurrence of autoantibodies in the blood increased agglutination ("gluing") of platelets, thereby forming microthrombuses, bridging the small blood vessels.
vessel wall due to increased permeability arising from a lack of serotonin (a substance produced by platelets vasoconstrictor) was passed out blood;hematomas occur in tissues or external bleeding begins.
addition, due to blood clots that partially or completely blocks a blood vessel to organs and tissues receives less oxygen and nutrients.Developed ischemia in many organs, including the brain, kidney, liver and heart.
When idiopathic thrombocytopenic purpura, in addition to the quantitative and qualitative changes in platelets, there are irregularities in the blood coagulation system, resulting in a blood clot at the bleeding is too loose.It has no retraction (spontaneous contraction, contraction of the wound edges), leading to recurrent bleeding.
In addition, because of the constant consumption of platelets stop bleeding when their blood levels are significantly reduced, which increases the thrombocytopenia.Blood clotting is deteriorating, bleeding becomes long, trudnoostanavlivaemymi.As a result, it may develop severe anemia.
The disease is classified according to different criteria: on the forms, trends and severity and periods.
forms of the disease:
course of the disease:
- with frequent relapses;
- with occasional relapses;
- with continuous relapses.
period of illness:
- Clinical (no bleeding with persistent blood thrombocytopenia);
- clinical and hematological (complete absence of bleeding and thrombocytopenia in the blood).
degrees of severity of idiopathic thrombocytopenic purpura:
Clinically, there are two types of thrombocytopenic purpura:
This classification of the severity of a little arbitrary, as there is always a degree of bleeding corresponds to thrombocytopenia in the blood.
acute form of the disease occurs spontaneously and rapidly progressing.In the initial stage of the disease manifest general symptoms of malaise:
then to other diagnostic joins hemorrhagic syndrome:
main characteristics of hemorrhagic syndrome in idiopathic thrombocytopenic purpura:
hemorrhages and bleeding from the mucous membranes are the main clinical symptoms.However, they may occur spontaneously or as a result of minor injuries and minor medical procedures (injections, collection of blood from a finger).
hemorrhages usually occur on the front surface of the body and limbs, they may be of different sizes and tend to merge.
In the future, the general condition is aggravated by prolonged fever and abnormal disorders of the central nervous system:
in blood in laboratory studies there is a pronounced thrombocytopenia and decreased hemoglobin levels.Because anemia may cause tachycardia.But after the normalization of the blood it usually disappears.
For the chronic form of the disease is characterized by acute onset did not.Common symptoms are mild, almost imperceptible, and appear well before the major symptoms of the disease.Hemorrhagic Syndrome begins with a small nasal bleeding, minor bleeding and prolonged bleeding after surgery.
The diagnosis of idiopathic thrombocytopenic purpura is carried out in several stages:
- thrombocytopenia - below 140 * 109 / L;
- increasing the duration of bleeding by Duke to 4 minutes or more;
- a blood clot retraction decrease to 60-75%;
- an increased number of megakaryocytes in the bone marrow (more than 54-114 cells / mm) - can be morphologically changed;
- the emergence of anti-platelet antibodies;
- functional disorders of platelets, increasing their size and shape change;
- Detection of elevated levels of antibody titers against certain infectious agents (eg, rubella, measles, chicken pox, cytomegalovirus, and others.);
- increased levels of bilirubin in the blood;
- at renal lesions in the blood level of urea and residual nitrogen.
Despite the fact that the main diagnostic feature is thrombocytopenia, its single detection in the blood is not sufficient for definitive diagnosis.Therefore, after 2-3 days it is carried out with a careful reanalysis of study leukocyte blood.
Methods and idiopathic thrombocytopenic purpura treatment regimens are completely dependent on the stage of the disease, the severity and course.They are divided into conservative and radical.
In the acute period needed urgent hospitalization and bed rest.Drinking plenty of fluids, liquid cooled food fractional portions.
treatment is carried out in four directions:
After hormonal therapy of the patients recover, but some dose reduction or withdrawal of the drug can cause a relapse of the disease.
When the "dry" form of purpura hormones are not administered.
When failure splenectomy (when the collapse platelets occurs primarily in the liver, rather than vselezenke supposed) designate immunosuppressants (cytostatics) together with hormones - cyclophosphamide, azathioprine, etc.
addition to these four directions, treatment of the various forms of the disease include:
All patients subject to compulsory medical examination and must be under constant supervision of a doctor-hematologist.It should be remembered that they are counter-chlorpromazine, salicylates, UFO, UHF and treatment of platelet inhibitors - drugs that inhibits the function of platelets.
In remission is necessary to make every effort to prevent exacerbations of the disease and complications.
with timely treatment the patient assistance and rapid diagnosis of the disease prognosis is quite favorable.There are cases of acute onset and rapid recovery after a course of treatment necessary.
poor prognosis may be the development of complications, as well as in cases where removal of the spleen has been ineffective.
pregnancy in idiopathic thrombocytopenic purpura occurs rarely.Still, you should know how to behave during gestation.
pregnant women with a diagnosis of "idiopathic thrombocytopenic purpura" should be constant monitoring and regular inspections haematologist.It is he who appoints the treatment and adjust the dose.
treatment with hormonal agents is not recommended because of the high risk of fetal malnutrition adrenal cortex.
splenectomy during pregnancy is carried out only by vital indications.
In most cases, pregnancy has a favorable outcome.Her interruption as splenectomy, shown only in the most extreme cases.
During treatment, idiopathic thrombocytopenic purpura can not breastfeed, as the milk may contain antibodies.Therefore, the baby should be placed temporarily on artificial feeding.
The disease can develop in both adults and children of all ages.
Newborn disease is hereditary, but not genetically caused because the mother passes child-specific antibodies.
older children the disease manifests itself in 1-3 weeks after suffering an infectious disease of viral or bacterial origin, as well as after preventive vaccinations.In children more often than adults, found chronic form of idiopathic thrombocytopenic purpura.
Boys and girls suffer equally often, but at an older age (after fourteen years), girls often suffer from this disease.
reasons mechanism of the disease and symptoms are the same for children and adults.But unlike adults, for the disease in children characterized by an inflammation of the lymph nodes, and 30% of the cases shown enlarged spleen (the spleen in adult idiopathic thrombocytopenic purpura increases when rare).
themselves no complaints in children up to the point of anemia.
Methods and treatment regimens are the same as in adults.Only children still prescribe drugs that stimulate hematopoiesis.