THROMBOCYTOPENIC PURPURA

Thrombocytopenic purpura (a disease Verlgo-fa) - a disease characterized by a tendency to

bleeding caused by thrombocytopenia (reduced platelet count in the blood to 150h10 9 / l) with
normal or increased number of megakaryocytes in the bone marrow.

Thrombocytopenic purpura - the most common disease of the groups of hemorrhagic

diathesis. The frequency of detection of new cases

thrombocytopenic purpura is from 10 to 125 per 1 million population per year. The disease
usually manifests in childhood. Until the age of 10 the disease occurs with equal frequency in
boys and girls, and after 10 years and adults - in 2-3 times more frequently in females.

Etiology and pathogenesis

When thrombocytopenic purpura thrombocytopenia develops because of the destruction of

platelets by immune mechanisms. AT to its own platelet may appear after 1.3 weeks after past
viral or bacterial infections, immunization, medication with their individual intolerance,
hypothermia, or insolation, after surgery, trauma. In some cases, any particular reason you can
not identify. Enters the organism Ar (eg, viruses, medicines, including vaccines) are deposited on
the platelets of the patient and induce an immune response. Antiplatelet AT are predominantly
IgG. Reaction "Ar-AT" occurs on the surface of platelets. The life span of platelets, loaded with
AT, with thrombocytopenic purpura is reduced to a few hours instead of 7-10 days is
normal. Premature death of platelets occurs in the spleen. Bleeding in thrombocytopenic purpura
due to decline in the number of platelets, the secondary damage of the vascular wall due to
deposition of angiotroficheskoy platelet function, a violation of the contractile capacity of the
vessels due to lower serotonin levels in the blood, the impossibility of retraction of blood clot.

Clinical picture

The disease begins gradually or sharply with the appearance of a hemorrhagic syndrome. Type
of bleeding in thrombocytopenic purpura ne-tehialno spotted (sinyachkovy). On clinical
manifestations distinguish two variants of thrombocytopenic purpura: "dry" - the patient occurs
only cutaneous hemorrhagic syndrome, "wet" - bleeding in conjunction with
bleeding. Pathognomonic symptoms of thrombocytopenic purpura - bleeding into the skin,
mucous membranes and bleeding. The absence of these signs is in doubt in the correctness of the
diagnosis.

Cutaneous hemorrhagic syndrome occurs in 100% of patients. Number ecchymosis varies from
single to multiple. The main characteristics of the cutaneous hemorrhagic syndrome in
thrombocytopenic purpura following.

-- Inconsistency in the degree of severity of hemorrhage, traumatic exposure, perhaps their

spontaneous appearance of (mostly at night).
-- Polymorphism of hemorrhagic rash (petechiae on to major hemorrhages).

-- Polychrome skin hemorrhages (color of purple to blue-greenish and yellow, depending on the
limitations of their appearance), which is associated with a gradual transformation but through
the intermediate stage of decomposition in bilirubin.

-- Asymmetry (not a favorite locale) hemorrhagic elements.

-- Painless.

Often there are hemorrhages in the mucous membranes, most often the tonsils, soft and hard
palate. Possible bleeding in the eardrum, sclera, vitreous, ocular fundus.

Hemorrhage in the sclera may indicate a threat of very severe and dangerous complications
thrombocytopenic purpura - bleeding in the brain. Typically, it occurs suddenly and rapidly
progressing. Clinically, bleeding into the brain appears headache, dizziness, cramps, vomiting,
focal neurologic symptoms. The outcome of a brain hemorrhage depends on the amount,
localization of the pathological process, timely diagnosis and adequate therapy.

To thrombocytopenic purpura is characterized by bleeding from the mucous

membranes. Often, they are profuse in nature, causing severe hemorrhagic anemia, life-
threatening patient. Children most often caused by bleeding from the mucous membrane of the
nasal cavity. Bleeding gums are usually less abundant, but they can become dangerous in the
extraction of teeth, especially in patients with nediag-nostirovannym disease. Bleeding after
tooth extraction with thrombocytopenic purpura occurs immediately after the intervention and
not renewed after its termination, in contrast to the later, delayed bleeding in hemophilia. In girls,
puberty may difficult and Meno-metrorrhagia. Less likely to be gastro-intestinal bleeding and
kidney.

Characteristic changes of internal organs with thrombocytopenic purpura lacking. Body

temperature is usually normal. Sometimes reveal tachycardia, with heart auscultation - systolic
noise at the top and at the point Botkin, the weakening of tone I caused anemia. Increase of the
spleen is unusual and probably excludes the diagnosis thrombocytopenic purpura.

Adrift distinguish acute (lasting up to 6 months) and chronic (lasting more than 6 months old)
form of the disease. Upon initial examination to determine the nature of the disease
impossible. Depending on the degree of manifestation of hemorrhagic syndrome, and blood
parameters in the course of the disease are three periods: the bloody crisis, clinical remission and
clinical and hematologic remission.

Hemorrhagic crisis is characterized by distinct syndrome of bleeding, significant changes in

laboratory parameters.
 During clinical remission disappears hemorrhagic syndrome, reduced bleeding time, decrease
secondary changes in blood coagulation system, but thrombocytopenia persists, although it is
less pronounced than in hemorrhagic crisis.

Clinical and hematologic remission implies not only the absence of bleeding, but normalization
of laboratory parameters.

Laboratory studies

Characterized by reduced platelet count in blood up to the individual in the sample and the
increase in bleeding time. Duration of bleeding does not always correspond to the degree of
thrombocytopenia, since it depends not only on the number of platelets, but also on their
qualitative characteristics. Significantly reduced or not occur at all a retraction of a blood
clot. Secondary (due to thrombocytopenia), altered plasma coagulation properties of blood,
which is manifested in lack of education thromboplastin time due to lack of the 3rd platelet
factor. Violation of thromboplastin formation leads to a decrease in the consumption of
prothrombin during blood coagulation. In some cases, thrombotic purpura tsitopenicheskoy
during the crisis mark the activation fibrinoliti-agency system and increase the anticoagulant
activity (antithrombin, heparin). All patients with thrombocytopenia decreased concentration of
gray-fineness in the blood. Endothelial samples (tourniquet, pinch, Hammer, UCO-alkaline) in
the period of hematological crisis are positive. In the red blood cells and leykogramme (in the
absence of blood loss), changes were found. In the study of red bone marrow is usually reveal a
normal or high content of megakaryocytes.

Diagnosis and differential diagnosis

Diagnosis thrombocytopenic purpura based on a characteristic clinical picture and laboratory

data (Table 14.2).

Table 14-2, Basic diagnostic criteria for thrombocytopenic purpura

Clinical Hemorrhages on the skin and mucous membranes (petechiae on to large ecchymosis)

Bleeding from the mucous membranes of the nose, gums, uterus, etc. Positive endothelial samples

Laboratory Thrombocytopenia
Increased bleeding time
Reducing the blood clot retraction

Thrombocytopenic purpura should be differentiated from acute leukemia, hypo-or aplasia of red
bone marrow of SLE, trombotsitopaty.

In hypo-and aplastic states in the study of blood reveal pancytopenia. Punctate red bone
marrow impoverished cellular elements.
 Imperious metaplasia in the red bone marrow - the main criterion for acute leukemia.

Thrombocytopenic purpura may be a manifestation of diffuse connective tissue diseases, most

commonly SLE. In this case, you must rely on the results of immunological studies. A high titer
of antinuclear factor, the presence of LE-cells suggest SLE.

The main difference thrombocytopenic purpura from trombotsitopaty - reduced platelet count.

Treatment

During hemorrhagic crisis shows the child to bed with its gradual expansion in the fading of
hemorrhagic phenomena. Special diets are not prescribed, but the bleeding of the oral mucosa
children should receive the food is cooled.

Pathogenetic therapy of autoimmune thrombocytopenic purpura include the appointment of

glucocorticoids, the implementation of splenectomy and the use of immunosuppressants.

Prednisone is prescribed in a dose of 2 mg / kg / day for 2-3 weeks followed by dose reduction
and complete removal of the drug. Prednisolone at higher doses (3 mg / kg / day) prescribed
short courses of 7 days with an interval of 5 days (not more than three courses). In sharply
expressed hemorrhagic syndrome, risk of hemorrhage in the brain can "pulse therapy" methyl-
prednisolone (30 mg / kg / day intravenously for 3 days). In most cases, this therapy is quite
effective. Initially disappears hemorrhagic syndrome, and then begins to increase platelet
count. In some patients after discontinuation of hormone occurs relapse.

In recent years, in the treatment of thrombocytopenic purpura with good results using
intravenous administration of normal human Ig at a dose of 0.4 or 1 g / kg for 5 or 2 days,
respectively (course dose of 2 g / kg) as monotherapy or in combination with glucocorticoids.

Splenectomy or vessels of the spleen tromboembolizatsiyu conducted in the absence or

instability of the effect of conservative treatment, recurring prolonged heavy bleeding, resulting
in severe hemorrhagic anemia, heavy bleeding, life-threatening patient. The operation is usually
performed against a background of glucocorticoid-term therapy in children older than 5 years, as
at an earlier age is great risk of postsplenektomicheskogo sepsis. In 70-80% of patients the
operation leads to an almost complete recovery. Other children and after splenectomy in need of
continued treatment.

Immunosuppressants (cytostatics) for the treatment of thrombocytopenic purpura in children is

used only when there is no effect on other types of therapy, as well as the effectiveness of their
use is considerably less than splenectomy. Applied vincristine at a dose of 1,5-2 mg / m 2 body
surface inward, cyclophosphamide at a dose of 10 mg / kg - 5-10 injections, azathioprine at a
dose of 2-3 mg / kg / day in 2-3 doses for 1 -- 2 months.

Recently, for the treatment of thrombocytopenic purpura is also used danazol (a synthetic drug
androgen action), preparations of interferon (reaferon, Intron A, Roferon-A), anti-D-Ig (anti-
D). However, the positive effects of their use of non-persistent, possible side effects, which
makes it necessary to further study the mechanism of their action and determine their place in the
treatment of this disease.

To reduce the severity of hemorrhagic syndrome during the period of increased bleeding
prescribe intravenous or oral aminokap-Ronova acid at the rate of 0,1 g / kg (contraindicated
with hematuria). The drug belongs to inhibitors of fibrinolysis, but also enhances platelet
aggregation. Applied also haemostatic tool etamzilat a dose of 5 mg / kg / day by mouth or
intravenously. The drug has also an-gioprotektornym and proagregantnym action. To stop
nosebleeds use tampons with hydrogen peroxide, adrenaline ^; nokapronovoy amino acid;
hemostatic sponge, fibrin, gelatin films.

In the treatment of posthemorrhagic anemia in children with thrombocytopenic purpura employ

means to stimulate hematopoiesis, as the regenerative ability of hematopoietic system in this
disease are not violated. Transfusion of washed red cells, matched individually hold only when
expressed severe anemia.

Prevention

Primary prevention is not developed. Secondary prevention is to prevent the recurrence of the
disease. If vaccination of children with thrombocytopenic purpura requires an individual
approach and special care. Schoolchildren are exempt from physical education, avoiding
insolation. In order to prevent hemorrhagic syndrome patients should not be given drugs which
inhibit platelet aggregation (eg, salicylates, indomethacin, barbiturates, caffeine, carbo-nitsillin,
nitrofurans, and others). After discharge from the hospital dispensary observation of children to
be within 5 years. The following study of blood with the platelet count 1 time in 7 days, in the
future (while maintaining remission) per month. Be sure to study the blood after each of the
disease.

Forecast

The outcome thrombocytopenic purpura can be healing, clinical remission without normalization
of laboratory parameters, a chronic relapsing course with hemorrhagic crisis and, in rare cases -
death as a result of bleeding in the brain (1-2%). With modern methods of treatment the
prognosis for life in most cases, favorable.