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Mayokki's purpura - 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

Mayokki's purpura — the rare, low-studied disease. Some authors consider it as option of an illness of Shenleyn — Genokh. The disease is for the first time described by the Italian dermatologist of A. Majocchi in 1895 under the name "purpura anularis teleangiectoides". The illness meets at children's age, and boys are ill more often than girls. The hereditary nature of a disease it is not established. The etiology and a pathogeny are not clear. Assume neurogenic character of a disease. Now this syndrome is carried to group of the acquired immunological states connected with long allergenic action of infectious factors. The disease has only skin manifestations and proceed at a satisfactory condition of patients. The clinical picture is characterized by emergence on skin of symmetrically located spots having the ring form. Elements are mainly exudative and inflammatory, dark red color, act over a skin surface. Component of spots are the smallest teleangiectasias. When pressing on spots glass they accept a brown shade, at the same time expanded small vessels are looked through. From affected areas there can sometimes be insignificant bleedings. Preferential localization of purpura — on skin of the lower extremities, buttocks, hips, is more rare — forearms and a trunk. Unlike Shenleyn's illness — Genokh at Mayokki's purpura there are an atrophy of skin, a teleangiectasia and a local alopecia. The forecast is favorable. The disease proceeds is benign and is independently allowed. Skin becomes smooth, accepts normal coloring, changes disappear completely. In rare instances the illness accepts a long current with recurrence of purpura. In the majority of situations it is possible to appoint antihistaminic and sosudoukreplyayushchy drugs, especially at tendency to bleedings.

Dysplastic hemorrhagic angiopathies

Dysplastic hemorrhagic angiopathies are caused by inborn and hereditary defects of development of connecting fabric. Dysplastic disturbances are the cornerstone not only malformations of vascular system, but also malformations of internals, bone system. In these cases the hemorrhagic angiopathy is one of disease symptoms. Bleeding pathogeny at dysplastic angiopathies is difficult and caused not only anatomic defect of a vascular wall, but also disturbance of platelet and coagulative links of system of a hemostasis.

Hereditary hemorrhagic teleangiectasia (an illness of Randyu — Oslera, an inborn hemorrhagic angiomatosis)

The disease is for the first time described by D. R. Sutton (1864) and G. Babington (1865) in the form of hereditary, observed at persons in several generations of nasal bleedings. In 1896 L. The m of Rendu established that the change of blood vessels (teleangiectasia) which is descended is the cornerstone of pathology. L. The m of Rendu called an illness a pseudohemophilia. In 1901 W. Osier studied clinicopathologic essence of this disease and otdifferentsirovat it from true hemophilia. Now the etiology and a pathogeny of an illness of Randyu — Oslera are up to the end not found out, but its clinical aspects are well studied. It is established that anatomic substrate of a disease is inborn inferiority of mesenchymal fabric. It leads to developing of the teleangiectasias possessing the increased vulnerability and tendency to bleeding. On histologic structure of a teleangiectasia differ from hemangiomas. In typical cases vasodilatation which walls consist mainly of an endothelium is observed and are surrounded with friable connecting fabric, elastic and muscular layers are atrophied. The mechanisms causing deficiency of mezenkhialny fabric are not clear. According to Yu. A. Livandovsky (1980), change of activity of a number of the enzymes participating in development of vascular system is the cornerstone of a course of a disease. There are some more hypotheses which consider: 1) value of a hyperserotoninemia as the factor leading to long dilatation of capillaries; 2) an abnormal liver function in connection with existence of teleangiectasias at chronic diseases of a liver; 3) genetically caused endothelium dysplasias.
Yu. A. Livandovsky (1980) notes that in bleeding pathogeny at an illness of Randyu — Oslera three factors work: deficiency of a vascular wall, functional disturbances of a local fibrinolysis and change of function of thrombocytes. There are disturbances in the I phase of a blood coagulation, decrease in level of a prothrombin, fibrinogen, plasminogen that is regarded as consumption coagulopathy signs, passing thrombocytopenia. The illness of Randyu — Oslera is inherited on autosomnodominantny type from various penetrantnostyo of a pathological gene. Homozygous forms of a disease meet seldom and often give a lethal outcome. Still births in families where both parents suffered from a hemorrhagic angiomatosis are described. An illness of Randyu — Oslera — rather rare disease. Frequency in population 1 — 2:100 000. The clinical picture of a disease is characterized by existence of teleangiectasias on skin, mucous membranes and frequent bleedings, especially nasal. Teleangiectasias form at early children's age, however the disease a long time proceeds asimptomno or with insignificant hemorrhagic manifestations. As a rule, the expressed bleeding arises during puberty or at youthful age that allows to speak about an etiological role of endocrine system (the increased content of estrogen). After the puberty period the number of teleangiectasias increases and, respectively, bleeding frequency increases. At children of the first years of life at external survey it is very difficult, and sometimes and it is impossible to find vascular changes. At an illness of Randyu — Osler is distinguished by three types of the teleangiectasias reflecting various extents of forming of elements: early — teleangiectasias have an appearance of points or specks, transitional — because of a star-shaped form elements are reminded by vascular spiders and the knotty mature type is provided by the small knots of the correct rounded shape, saturated-red color which are a little towering over a surface of skin and mucous membranes. When pressing a teleangiectasia turn pale, after the terminations — quickly recover the color. At a diaskopiya reveal the central papulovidny eminence with radial star-shaped branchings.
Most often teleangiectasias are located on a mucous membrane of a nose, an oral cavity, lips, language, on skin of wings of a nose, cheeks less often on other sites of skin (a breast, a stomach, extremities). Hemorrhagic manifestations in most cases begin with nasal bleedings. Quite often they are the only symptom of a disease appearing at children's age. At a rinoskopiya of a teleangiectasia of a mucous membrane of a nose come to light badly in connection with the developed vascular network that can complicate diagnosis of a disease. In the presence of teleangiectasias of other superficial localization diagnosis of an illness of Randyu — Oslera comes easy. Certain help in diagnosis is given by the family anamnesis and survey of parents of the child at which skin teleangiectasias were already usually created. Nasal bleedings arise spontaneously or as a result of small injuries, overheating, the infectious diseases which are especially followed by fever and toxicosis. Bleedings happen frequent and intensive that causes the child's anemization. Now, despite plentiful profuse bleedings of various localization, fatal cases at an illness of Randyu — Oslera are rare, however, according to various authors, make up to 4%.
Teleangiectasias can be located in bronchopulmonary system and cause a pneumorrhagia, pulmonary bleedings. Pulmonary bleedings differ in extremely heavy current. At an arrangement of teleangiectasias on a mucous membrane of a digestive tract gastrointestinal bleedings are observed. Similar changes are observed on a mucous membrane of urinary tract in this connection there is a hamaturia. In these cases as criteria serve results of endoscopic methods of research. At an illness of Randyu — Oslera often is involved in pathological process a liver. Objectively define a hepatomegalia, change of functional trials. Multiple angiomas of a liver can be complicated by bleeding in an abdominal cavity a lethal outcome. or development of cirrhosis with symptoms of portal hypertensia.
Cases of an illness of Randyu — Oslera with localization telean the iektaziya in the central nervous system giving hemorrhage in brain substance are described.
The hemorrhagic angiomatosis can be combined with arteriovenous aneurisms of internals — lungs, a liver, kidneys, a spleen. Arteriovenous shunts in vessels of lungs are most often noted. At the same time the clinical picture caused by the hypoxia phenomena as a result of mixing of an arterial and venous blood is observed. Cyanosis, short wind are noted, pulmonary insufficiency progresses, fingers get a form of "drum sticks", there is a "compensatory" secondary hyperglobulia. The correct diagnosis is promoted by radiological and angiographic methods of research.
In blood at frequent bleedings it is noted normo-, and afterwards — hypochromia posthemorrhagic anemia of various degree.
Treatment of a disease consists of several stages: prevention of bleedings, during the acute period — a stop of bleeding and elimination of its effect — anemia.
Preventive actions provide the guarding mode which is not allowing heavy exercise and intellectual stresses, emotional and mental pressure, the prevention of injuries and infectious diseases. The food and medicinal substances containing vinegar, salicylates have to be excluded from use.
At a stop of bleeding use local and general influence. Enter Calcii chloridum, Dicynonum, Vikasolum, aminocapronic, ascorbic acid. During the acute period these means play a part at a stop of profuse bleedings. From local means the hard tamponade at nasal bleedings as it leads to traumatization of a mucous membrane and strengthening of bleeding after removal of a tampon is not recommended. Now the stop of nasal bleeding by means of rubber "finger" in which air from a pear is forced through a catheter (3 is recommended. S. Barkagan, 1980). Also carry out a local irrigation of the bleeding surfaces by hydrogen peroxide, thromboplastin, thrombin, 5 — 8% solution of aminocapronic acid. In urgentny situations it is possible to resort to cauterization of a mucous membrane of a nose, its amotio with bandaging of the bringing arteries. Good effect at a stop of bleeding cryoinfluence has. At profuse bleedings from internals sometimes it is necessary to resort to surgical intervention. At superficial localization attempts of radical removal of teleangiectasias by means of a cryolysis, electrocoagulations, rentgeno-and radiological influence become. However recurrence of a disease is possible and these methods can cause various complications.
At acute posthemorrhagic anemia fill the volume of the circulating blood, carry out replaceable hemotransfusions. As a result of frequent blood losses chronic posthemorrhagic anemia forms. Treatment is based by the general principles of therapy of iron deficiency anemias.



 
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