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Treatment of an idiopathic Werlhof's disease - Practical hematology of children's age

Table of contents
Practical hematology of children's age
Embryonal hemopoiesis
Morfofunktsionalny characteristic of cells of marrow and peripheral blood
Marrow parenchyma cells
Peripheral blood of children of different age
The system of a hemostasis is normal
Etiology and pathogeny of leukoses
Acute leukoses
Acute leukoses - a preleukosis
Possibilities of a predictive assessment of a course of an acute lymphoblastoid leukosis at children
General principles of treatment of an acute leukosis
Chemotherapeutic drugs
Treatment of an acute lymphoblastoid leukosis
Treatment of myeloid forms of an acute leukosis
Infectious complications and symptomatic therapy of an acute leukosis
Consolidation and maintenance therapy of an acute leukosis
Immunotherapy
Remission and recurrence of an acute leukosis
Inborn leukosis
Neuroleukosis
Myelosis
Lymphogranulomatosis
Gematosarkoma
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Leukopenias
Histiocytoses
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Angiopathies
Hemorrhagic vasculitis (Shenleyn's illness — Genokh)
Mayokki's purpura
Ataxy teleangiectasia
Entsefalotrigeminalny angiomatosis
Kortiko-meningealny diffusion angiomatosis
Cerebroretinal angiomatosis
Hypertrophic gemangiektaziya
Multiple and huge hemangiomas
Elastic fibrodisplaziya
Coagulopathies
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Angiohemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Dysfibrinogenemias
Hereditary deficit of factors of VII, X, V and II
Deficit K-vitaminozavisimykh of factors of coagulation
Syndrome of the disseminated intravascular coagulation
Clinic and diagnosis of the IDCS
Treatment of the IDCS
Thrombocytopenia
Idiopathic Werlhof's disease
Clinic and diagnosis of an idiopathic Werlhof's disease
Treatment of an idiopathic Werlhof's disease
Isoimmune Werlhof's disease
Transimmune Werlhof's disease of newborns
Trombogemolitichesky Werlhof's disease (syndrome Moshkovich)
Hereditary Werlhof's diseases
Trobotsitopatiya
Anemias
The anemias connected with blood loss
Chronic posthemorrhagic anemia
Iron deficiency anemias
Clinic and diagnosis of an iron deficiency anemia
Treatment of iron deficiency anemias
Sideroakhrestichesky, sideroblastny anemias
Megaloblastny anemias
Foliyevodefitsitny anemia
Hereditary forms of megaloblastny anemias
Hereditary dizeritropoetichesky anemias
The anemias connected with oppression of proliferation of cells of marrow
Hereditary hypoplastic anemias
Hemolitic anemias
Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis
Acanthocytosis, piknotsitoz
The hereditary hemolitic anemias connected with disturbance of activity of enzymes of erythrocytes
The hereditary hemolitic anemias connected with disturbance of structure or synthesis of hemoglobin
The acquired immune hemolitic anemias
Isoimmune hemolitic anemias
Treatment of a hemolitic illness of newborns
Autoimmune hemolitic anemias
List of references

The complex of therapeutic actions at ITP is defined by weight, a current and the period of an illness and it consists of conservative and operational methods.
In the period of crisis hospitalization in a specialized hospital and purpose of a bed rest is shown. A physical activity can strengthen hemorrhagic manifestations. The diet has to be full, caloric and provide the child's organism with the main food ingredients. The food is recommended to be given in small amounts, fractionally, in the cooled state and a liquid consistence. The drinking mode, especially at children with bleedings to which appoint plentiful amount of liquid is important.
The special place in conservative therapy of ITP belongs to corticosteroid hormones which reduce vascular permeability, increase coagulability of blood and that is the most important, possess an immunodepressive effect. Despite high performance, in a row all children cannot appoint glucocorticoids with ITP. It is necessary to estimate a clinical picture, weight of a hemorrhagic syndrome and thrombocytopenia. In some cases the positive therapeutic effect can be gained also without use of corticosteroids. Proceeding from theoretical premises, at the ITP acute form it is possible to do without corticosteroid therapy. As it is almost difficult to decide what process — heteroimmune or autoimmune — is the cornerstone of ITP in each case, criteria for a question of purpose of corticosteroid hormones are clinical laboratory data. Glucocorticoids are shown at an extensive skin hemorrhagic syndrome, bleeding from mucous membranes, bleedings of various localization, hemorrhages in internals expressed to thrombocytopenia (less than 30 X 109/l). At the ITP easy forms when in a clinical picture the isolated skin hemorrhagic syndrome, in therapy enough symptomatic means is noted. Corticosteroid hormones (Prednisolonum) appoint 1,5 — 2 mg/kg of body weight in a daily dose. Glucocorticoids recommend to enter taking into account a day-night rhythm of secretion of hormones — 2/z a daily dose of drug in the morning, and 1/3 in the afternoon. The effect of therapy is shown already in 1 — 2 week when the hemorrhagic syndrome is stopped and further gradual increase of quantity of platelets begins. Despite rather fast effect, hormonal therapy it is recommended to carry out in an initial dose during 3 — 4 weeks then Prednisolonum dose is slowly reduced every 5 — 7 days on 5 mg to full cancellation. In recent years schemes of treatment are offered by discontinuous courses which are recommended in need of long therapy by corticosteroids. Pass to discontinuous therapy for maintenance of the reached effect, full cancellation of corticosteroids leads to recurrence of a disease. Hormones can be accepted bucketed every other day or in a row four days with a three-day interval. In the absence of desirable result within 1 month the dose of hormones should be lowered. Further repeatedly to appoint a course, however it often is inefficient. If hormonal therapy within 3 — 4 months gives incomplete and unstable effect, raise a question of a splenectomy or purpose of cytostatic drugs.
In complex therapy of ITP the important place belongs to the symptomatic funds allocated for increase of resistance of a vascular wall, improvement of functional properties of thrombocytes etc. As it was already noted, it is possible to be limited to this therapy at the ITP easy forms. Sick appoint a complex of vitamins. Tselesosobrazno administration of vitamins P and Page. Now it is proved that it is not necessary to resort to purpose of high doses of ascorbic acid as it worsens functional properties of thrombocytes.
A certain effect is rendered by calcium salts, and the best drug is calcium pantothenate. Aminocapronic acid which improves functional properties of thrombocytes possesses good haemo static properties, oppresses the fibrinolitic activity of blood increasing at many sick ITP. Amiyaokapronovy acid is appointed inside in a dose of 0,2 g/kg of body weight by 3 — 4 times a day or enter intravenously kapelno in the form of 5% of solution. Aminocapronic acid is also effective as a local hemostatic. At nasal bleedings use absorbable gelatin sponges, cryotherapy, Adroxonum. At treatment of a Werlhof's disease broad application was found by drug Dicynonum. It possesses action both on a vascular wall, and on functional capacity of thrombocytes, stimulating their adhesive properties. Dicynonum is appointed inside in tablets or entered intramusculary. The certain effect caused by impact on functional properties of thrombocytes ATP in combination with magnesium sulfate has. N. P. Shabalov (1982) in therapy of ITP recommends to 0,5% sodium chlorophyllin solution (water-soluble drug of needles) which is appointed inside on 2 drops of times a day during 2 — 6 weeks. As styptic means use the medicinal collecting including a nettle, a dogrose, corn stigmas, water pepper, a yarrow.

Important issue in treatment of ITP at the expressed bleeding is the possibility of use of hemotransfusions. Now the point of view is standard that hemotransfusions are ineffective. Moreover, under the influence of the last adhesive and aggregation properties of thrombocytes decrease that aggravates a Werlhof's disease and, perhaps, strengthens bleeding (3. S. Barkagan, 1980). As practice showed, the haemo static effect of the transfused blood is expressed poorly and short-term. It is necessary to resort to hemotransfusions only at the heavy acute anemia posing threat for life or for a stop of the massive, not stopped other means bleeding. In the presence of heavy anemia it is better to use the washed erythrocytes.
At inefficiency of conservative therapy the second stage of treatment of ITP is the splenectomy. Resort to an operational method at a chronic form of the Werlhof's disease which is not giving in to the hormonal therapy which is characterized by frequent recurrence and plentiful bleedings. The splenectomy is also shown in critical situations when it is impossible to stop heavy bleedings, life-threatening patients or the hematencephalons interfaced to threat. In usual situations the issue of a splenectomy is resolved after 4 — 6 months of inefficient conservative therapy. As show numerous supervision, efficiency of a splenectomy at ITP very high. Good results after operation receive more than at 90% of patients. In several hours after a splenectomy increase in number of thrombocytes is noted. The percent of operational and postoperative complications is low. The splenectomy is recommended to be carried out to the period of relative wellbeing, with an obligatory preoperative and postoperative course of hormonal therapy (4 — 5 days). It is necessary to specify that the splenectomy not always leads to normalization of level of thrombocytes, but even in these situations reduction or even a total disappearance of signs of bleeding is noted.
According to hematologic clinic of scientific research institute of pediatrics of the USSR Academy of Medical Sciences, at 15% of children the splenectomy, judging by the long-term results, did not bring the expected results. One of the reasons of inefficiency of a splenectomy at ITP is existence of a spleniculus, unextracted during operation. The inefficient splenectomy nevertheless to some extent softens a clinical picture of a disease — recurrence urezhatsya, bleeding symptoms decrease, sensitivity to hormonal therapy appears.
The third stage in treatment of ITP is purpose of cytostatic immunodepressants. Indications to cytostatic therapy have to be strictly limited, resort to it only after inefficiency of hormonal means and a splenectomy. Especially careful approach in pediatric practice is dictated to those by circumstance that cytostatic drugs lead not only to development of a pancytopenia and the related infectious complications, but also oppress function of gonads, damage healthy fabrics that adversely affects the growing child's organism. Thus, cytostatic drugs are appointed only in extreme situations when all available means are tested. Immunodepressive therapy by cytostatic drugs is carried usually out in combination with glucocorticoids. At treatment of ITP use the following drugs: imuran (Azathioprinum), Cyclophosphanum, 6 Mercaptopurinum, Vincristinum, vinblastine. It is established that the effect of chemotherapy is connected with suppression of proliferation of V-lymphocytes, however at the same time the oppressing action extends also to T-cells that aggravates the imbalance which is available at ITP in lymphoid subpopulations.
Sick ITP are subject to dispensary accounting. During remission to children carry out sanitation of the centers of persistent infection. School students are exempted from exercises, preventive inoculations. It is necessary to avoid purpose of physiotherapeutic and thermal procedures. Stay on the sun as it can lead to an exacerbation of a disease is not recommended. For prevention of bleeding appoint courses of haemo static therapy — aminocapronic acid, vitamins P, C and A. Special preparation is required in need of operative measures.



 
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