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Histiocytoses - 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

Chapter V histiocytoses
Under the general name "histiocytosis" integrate several diseases having the general pathogenetic mechanisms and morphological features. Are included in this group: Letterer's illness — Ziva, an eosinophilic granuloma and Hend's illness — Schueller — Krischena. The term "histiocytosis of X" is offered L. Lichtenstein in 1953 and now is standard. For almost centenary period which passed from the moment of the first description, Kliniko-morfologichesky features of histiocytoses are well studied, however the pathogeny is up to the end not found out. The etiology is unknown. Infectious agents consider as probable etiological factors. Adverse effects on a fruit matter during pregnancy and at childbirth. In emergence of an eosinophilic granuloma a certain part is assigned to traumatic injury of a bone. The description of family cases allows to think of hereditary character of a disease with probable autosomal and recessive type of transfer.
Due to the disturbances of a cholesteric exchange which are available at histiocytoses some authors consider this pathology as primary lipoidoses with a secondary infiltration of fabrics histiocytic elements. Now most of researchers adhere to other point of view. Histiocytoses are carried to primary tumoral processes which cornerstone are proliferation of macrophagic elements (according to the old nomenclature — reticuloendothelial cells), by formation of granulomas and an infiltration of fabrics. Disturbances of a cholesteric exchange are secondary, and at different forms of histiocytoses extent of these disturbances is various. Secondary disturbances of a lipometabolism distinguish this group of diseases from accumulation diseases (an illness to Gosha, Nimann's illness — Peak, etc.) which cornerstone primary enzymatic defect of utilization of lipids is. From modern positions Letterer's illness — Ziva, the eosinophilic granule and Hend's illness — Schueller — Krischena consider as the uniform disease reflecting various stages of pathological process. As confirmation to it serve cases of transition of one form in another. So, the eosinophilic granuloma can be considered as an initial stage of an illness of Hend — Schueller — Krischena. According to the international WHO classification (1976), histiocytoses are allocated in group of general tumoral diseases of the hemopoietic and adenoid tissue which cornerstone defect in system of the englobing mononuklear is. N. S. Kislyak and R. V. Lenskaya (1978) at the children suffering from a histiocytosis studied phagocytal function of monocytes of peripheral blood. It is established that the englobing activity of cells is reduced that is expressed in disturbance of the digesting ability and incomplete phagocytosis. According to authors, functional defect of monocytes has primary character and reflects essence of a disease — pathology of the englobing mononuklear. Similar researches at malignant limfoproliferativny diseases revealed normal functional activity of monocytes that serves as confirmation of primary defect at a histiocytosis. On the basis of these data authors formulated the original theory of a pathogeny of a histiocytosis. The pathological poliferation of histiocytes occurs in the presence of a defective pool of progenitors which monocytes are. Decrease in the digesting ability of monocytes of peripheral blood defines disturbance of normal inflammatory reaction in fabrics that in turn exponentiates tumoral transformation of macrophagic elements in places of an inflammation.
Depending on a disease form at a histiocytosis the local or generalized infiltration of fabrics is observed. The morphological picture of cellular substrate has similarity at different forms of a disease though certain peculiar features, of course, come to light. Proliferata contain histiocytic cells, neutrophylic and eosinophilic granulocytes, plasmocytes. N. S. Kislyak and R. V. Lenskaya (1978) on morphological and cytochemical features describe three types of the cells relating to histiocytic. This triad of cells is characteristic of all forms of a histiocytosis. Morphological distinctions in structure are caused by a stage of development of pathological process, on the origin these cells are uniform. Are a part of a triad: atypical histiocytes, multinuclear, or Touton-cells, and ksantomny cells.
Atypical histiocytes have regular shape, a kernel rather small, roundish, with gentle structure of chromatin, contains a nukleola. Cytoplasm wide, is painted in bluish color, around a kernel there is a small amount of small vacuoles. Inclusions reveal seldom. Cells of the big size (20 — 40 microns). Determine by Tsitokhimicheski high activity of oxidation-reduction enzymes: succinatedehydrogenases, glyukozo-6-fosfatdegidrogenazy, etc. In cytoplasm of some cells find difficult lipids that is uncharacteristic for histiocytes and monocytes. Reaction to acid phosphatase and and-naftilatsetat esterase positive. The CHIC reaction gives both diffusion, and granular coloring. At elektronnomikroskopichesky research in cytoplasm of atypical histiocytes the inclusions which received names of "a little body of X" and being lipoprotein complexes were found.
Multinuclear, or Touton-cells, bigger size, than atypical histiocytes. They have regular shape, contain 3 — 6 kernels and more. Kernels of gentle structure, contain nukleola of rounded shape. Cytoplasm wide, with a violet shade, has a small amount of vacuoles. Cytochemical characteristics are similar to atypical histiocytes. However enzymatic activity is higher. There is an interesting pattern: in comparison with a one-nuclear cell activity increases by number, multiple to number of kernels (in 2, 3, 4 etc.).
Ksantomny cells are a final stage of development of histiocytes at accumulation of fats in them. These are huge elements (the size 50 — 100 microns) with very low nucleocytoplasmic relation. The kernel is small, with rough structure, it is located in a cell excentricly. Cytoplasm plentiful, blue-violet color, foamy because of existence of a large number of vacuoles. Determine by Tsitokhimicheski intensive reaction to lipids with Sudan black B. Other cytochemical changes in comparison with atypical histiocytes are lowered.
The general sign of proliferat at a histiocytosis is dominance of atypical histiocytes and multinucleate cells in the young centers and ksantomny cells — in the old centers.
Diagnosis of a histiocytosis has certain difficulties. The diagnosis is confirmed on the basis of set of a clinical symptom complex and morphological research - punctates of bodies. And at research of marrow of change it is not always possible to reveal. It is caused by the fact that at the height of a disease when inspection is in most cases undertaken, hyper proliferation of monocytes in marrow is replaced by hypoproliferation that causes accident of detection of the pathological centers. As more informative diagnostic methods serve researches of the struck bodies — spleens, skin, lymph nodes, bone defects.
The most severe form of a histiocytosis is the illness of Letterera-— Ziva. Made the first description in 1924 E. Letterer. In 1933 S. A. Siwe on the basis of own and literary data carried a disease in group which was designated at that time as reticuloendothelioses. In 1936 A. Abt made the detailed analysis of the being available cases and offered the name — "Letterer's illness — Ziva". The disease is generally noted at children of the first two years of life though cases of emergence and at more advanced age are described. Weight of process is defined by generalized defeat of fabrics and availability of infiltrates in many bodies. K. A. Moskacheva and coauthors (1967) suggested to allocate several periods during an illness: initial, the period of the expressed phenomena, remissions and terminal. Diagnosis of a disease in an initial stage which is characterized by a number of the general nonspecific symptoms is most difficult. Children become sluggish, appetite worsens, the sleep is interrupted, badly add in weight. At the same time, already in an initial stage there is a number of characteristic symptoms — damage of skin and mucous membranes. Perhaps localized or widespread hyperadenosis, damage of a bone tissue, pulmonary changes that is probably caused by primary localization of process in these bodies.
The developed picture of a disease is characterized by a variety of clinical symptoms. A condition of children heavy, the general symptoms of intoxication — lack of appetite, slackness, tearfulness, emaciation, temperature increase. The temperature curve has no pattern: periodic rises to high figures or long subfebrile condition are possible. A typical symptom of an illness of Letterer — Ziva are skin changes which are observed almost at all children. Skin rash has various character and has various localization. It is most often noted on pilar part of the head, behind ears, on a front surface of a trunk, is more rare — in axillary and inguinal folds, on extremities, a back, a face. Character of rash from small petekhialny to large papulo-makulezny, places of merging, has coloring from light pink to red. Afterwards elements become covered by the yellowish, easily removed crust. During the late period of a disease emergence of hemorrhagic rash is possible, hemorrhagic character is also accepted by the available papular elements. The mucous membrane of an oral cavity is edematous, hyperemic, yazvennonekrotichesky processes develop. Patients because of a pain syndrome refuse meal, a putrefactive smell from a mouth, shaking and dedentition are noted. Ulcer and necrotic damages of mucous membranes can also be revealed in the field of an anus, generative organs, a century. One of constant symptoms of an illness of Letterer — Ziva — a hyperadenosis. As a rule, several groups are involved in process: cervical, axillary, inguinal. The polyadenia has moderate character. Lymph nodes are elastic, are not soldered among themselves and to surrounding fabrics, at a palpation are painless. At some patients significant tumoral increase in separate groups of lymph nodes is noted. Increase in intra belly lymph nodes can give symptoms of an acute abdomen.
— Ziva the moderate gepatosplenomegaliya is typical for Letterer's illness, sometimes the liver and a spleen can reach the considerable sizes. At a palpation bodies dense, painless. At fast increase the pain syndrome is noted. Damage of a liver can cause jaundice of both parenchymatous type, and mechanical as a result of a prelum of zhelchevyvodyashchy channels the increased lymph nodes. During the late period development of biliary cirrhosis and ascites is possible.
In most cases Letterer's diseases — Ziva the infiltration of pulmonary fabric which can proceed asimptomno is observed and be defined only at pathoanatomical research. At some patients pulmonary process accepts an acute malignant current and is followed by respiratory insufficiency. More often dysfunctions of lungs develop gradually, short wind, cyanosis of a nasolabial triangle, inflating of wings of a nose, dry cough develop. The X-ray pattern is characterized by strengthening of pulmonary drawing, emergence of gentle netting and focal shadows in both lungs. Increase in intrathoracic lymph nodes can lead to a compression of bronchial tubes and development of atelectases. At Letterer's illness — Ziva, especially at babies, cases of formation of pulmonary cavities, and afterwards — developing of spontaneous pheumothorax are described. Sometimes there is exudative pleurisy which is followed by considerable accumulation of liquid. At pulmonary defeats rattles are not listened, nature of breath changes slightly (weakened or rigid), perkutorno define easy shortening in places of infiltrates with tympanites zones around.
One of important symptoms — damage of a bone tissue. At Letterer's illness — Ziva, as well as at other forms of a histiocytosis, the most frequent changes find in flat bones of a calvaria. As a rule, destruction multiple. In the beginning the dense elastic swelling, painful is noted at a palpation. At destruction of bone plates the finger at a palpation "fails" in defect. Skin over the centers of defeat is not changed, signs of an inflammation are not noted. Fistulas can sometimes be formed, at the same time the discharge carries is purulent - hemorrhagic character, skin ulcerations over defeat sites are possible. Radiological define the destruction centers with accurate contours. At damage of a temporal bone the picture of otitis and a mastoiditis is created. In acoustical pass it is possible to see granulyatsionny fabric. In separated from an ear find atypical histiocytes.
Infiltration of fabrics of the alimentary system causes typical frustration: there is an abdominal pain, the unstable chair appears — the lock replaces a diarrhea, in Calais there is an impurity of slime and blood. The specified disturbances are caused by the general intoxication. The terrible complications which are followed by a necrosis of a wall of a gut and development of peritonitis are described.
Cardiovascular changes have nonspecific character more often and are connected with intoxication and anemia. At patients tachycardia, dullness of cordial tones, systolic noise over a heart top, lowering of arterial pressure are noted. On an ECG — symptoms of a hypoxia of a myocardium.
The current and the forecast of an illness of Letterer — Ziva in many respects depend on extent of generalization of process and time of developing of a disease. It is long and rather softly process proceeds at defeat no more than four types of fabrics and at children of advanced age. The forecast adverse at defeat of five and more types of fabrics, and also at a disease at chest age, especially in the first half of the year.
Modern therapy allows to receive long remissions at a number of patients. Sometimes improvement or remission can spontaneously arise without treatment. The period of remission is characterized by improvement of the general condition of the child and obsolescence of clinical signs of a disease. First of all there take place changes on skin and mucous membranes. The liver and a spleen, lymph nodes decrease. Most longer bone defects which are filled during 1 year — 2 years keep.
During the terminal period a condition of children very heavy — intoxication is expressed, temperature reaches high figures. Irreversible changes in vitals, accession of consecutive infection define a lethal outcome.
In blood SOE, a moderate leukocytosis is increased. Usually there is normokhromny anemia progressing with a disease. It is caused by oppression of an erythroidal sprout of a hemopoiesis. Cases of an illness of Letterer — Ziva at which anemia resulted from vascular hemolysis are described. Quantity of thrombocytes normal. The pancytopenia connected both with a total infiltration of marrow, and with the hypersplenism phenomena can develop in the late period. At research of marrow histiocytic cells find less than at H \patients though in process of progressing of a disease the infiltration of marrow accrues.
At a biochemical blood analysis the amount of cholesterol corresponds to the upper bounds of norm or is slightly increased. During remission the level of cholesterol decreases, in comparison with initial, and again increases at an exacerbation of a disease. Changes in the ratio of fractions of lipoproteids are noted: contents and - lipoproteids is reduced and considerably increased — R-lipoproteids. At remission approach the ratio of fractions is normalized. Content of crude protein within age norm, but a ratio of protein fractions is broken. Level of albumine is considerably reduced, the hyperglobulinemia is expressed, the albumino-globulinovy coefficient is less than unit.
Differential diagnosis of an illness of Letterer — Ziva is carried out with an acute leukosis. Clinically both diseases have some similarity, especially at chest age therefore the main criterion is the analysis of hematologic data. The hyperleukocytosis, thrombocytopenia, heavy hemorrhagic manifestations are characteristic of an acute leukosis, find blast cells in peripheral blood and marrow. Also the return picture when the rare monoblast form of an acute leukosis at babies is carried to Letterer's illness — Ziva is sometimes observed. The disease should be differentiated with accumulation diseases at which, except the general symptoms, there is a similarity of a morphological picture of punctates of bodies. At infantile forms of an illness to Gosha, Nimanna — Peak the neurologic disturbances which are absent at Letterer's illness — Ziva always reveal.
There are no standard programs of treatment of a generalized form of a histiocytosis. The main place belongs to hormonal therapy by corticosteroids. Prolonged treatment, before receiving the expressed clinical effect. Average daily doses of Prednisolonum make 1,5 — 2 mg/kg of body weight. When receiving effect drug is cancelled gradually. Now in complex therapy of an illness of Letterer — Ziva use cytostatic means — Vincristinum, Cyclophosphanum, 6 Mercaptopurinum, a methotrexate. Himiopreparata apply surely against hormonal therapy. In some cases the acute form of a disease manages to be transferred to chronic or to receive remission. Full cancellation of therapy conducts to an aggravation therefore it is necessary to conduct the short supporting courses of treatment. In the presence of bone defeats the roentgenotherapy is shown. In the period of the expressed clinical picture, and also in a terminal phase of a disease use means of symptomatic therapy for maintenance of the vital functions of an organism. Antibiotics apply both to prevention, and to treatment of secondary infectious complications. To the senior children bring a certain correction in a diet, limiting lipids and replacing animal fats with vegetable.
In group of histiocytoses Hend's illness — Schueller — Krischena is carried. The disease usually begins at children at the age of 10 flyings, is less often more senior, occurs also at adults. Males are ill twice more often. A classical form of an illness of Hend — Schueller — Krischena harakterizustsya a triad of symptoms: defects of a bone tissue, exophthalmos and not diabetes mellitus. The clinical picture of a disease is not limited to a classical triad and, of course, is more various. At Hend's illness — Schueller — Krischena as well as at Letterer's illness — Ziva, process has generalized character, however a current softer, the tumoral progression occurs slowly. The disease of a natural way proceeds is long, chronically (for several years) with the improvement periods, spontaneous remissions though without treatment has a lethal outcome. At the same time existence of a triad of symptoms is not obligatory in a clinical picture. It can be noted mono - or a bisimptomatika that, according to many authors, meets more often than a classical form.
K. A. Moskacheva and coauthors (1967) during Hend's illness — Schueller — Krischena allocate several periods: initial, the expressed phenomena, remission and terminal. Diagnosis of a disease is most difficult in an initial stage which can last several years. A number of authors consider that the illness arises at babyhood, but the slow progression leads to the expressed manifestations already at the senior children. Initial symptoms, as a rule, are registered a post factum. The general satisfactory condition, working capacity, game activity remain. However the periods of an aggravation of symptoms with subfebrile condition, the general weakness, drowsiness, headache attacks are noted. In an initial stage it is possible to note skin rash. The period of the expressed phenomena lasts several years. The condition of children worsens — appetite decreases, the general weakness, slackness, irritability, tearfulness appear. Physical development is late (especially growth) that is most expressed at not diabetes mellitus. In some cases emaciation, at some children is noted, on the contrary, pathological obesity develops.
The most characteristic symptom — the destruction of a bone tissue which is observed almost at all patients. On defeat frequency calvaria bones are on the first place. At Hend's illness — Schueller — Krischen is found by multiple defects of bones of a skull. At a long current one centers of destruction are filled and new defects of other localization appear. There is an opinion that at heavy process destruction of a bone tissue is insignificant, and, on the contrary, at improvement of a clinical state multiple bone changes appear. It is probably caused by features of histiocytic proliferation, mainly in visceral bodies or a bone tissue that defines weight of clinical manifestations. At X-ray inspection of bones of a skull reveal the defects of a bone tissue having accurate contours. Size varies them from the several tenth shares of centimeter to the large, sometimes merging among themselves centers the sizes of 10 cm and more in the diameter. Damage of bones of a skull can be followed by a headache, ear pain. Severe pain, especially at night, is noted at patients with damage of long tubular bones. At the same time children considerably limit a physical activity, refuse to rise, go, take objects in hand. Radiological periosteal reaction, in some cases — endosteal is almost always observed.
The centers of destruction have the oblong shape extended on a bone longitudinal axis. Damage of a backbone results in immobilization. Because of severe pain children refuse to sit, rise in a bed, adopt the forced provision. The X-ray pattern is characterized by existence of the isolated defects without the phenomena of diffusion osteoporosis and defeat of intervertebral disks. Defeat of pelvic bones at Hend's illness — Schueller — Krischena is observed very often and, according to a number of authors, takes the second place after defects of bones of a skull. Clinically it is also characterized by ossalgiya. Radiological define the destruction centers, small or average by the sizes, sometimes passing through all thickness of a bone. In defect there are bone crossing points giving it a cellular look. At the multiple centers of destruction haunch bones, on a trope, look as if corroded a moth. Find changes in edges and a clavicle much less often.
One of characteristic symptoms of an illness of Hend — Schueller — Krischena is not diabetes mellitus though this syndrome is noted not in all cases. Usually not diabetes mellitus develops several years later from the beginning of a disease. A number of authors regard its emergence as a favorable predictive sign testimonial of a long chronic current. A pathogeny of not diabetes mellitus connect with defeat of system a supraoptic kernel — a supraoptiko-pituitary path — a neurohypophysis. Localization of pathological process on any of these levels leads to reduction of products of the antidiuretic hormone responsible for a water reabsorption from distal tubules of nephron. It leads to the increased liquid losses. The reason of localization of process in gipotalamo-pituitary area is up to the end not studied. The direct infiltration of a hypophysis is a little probable. Defeat of hypothalamic area is possible. More real consider a hypophysis prelum as the histiocytic tumor proceeding from a firm meninx and bones of a base of skull. The main clinical symptoms of not diabetes mellitus — a polydipsia and a polyuria. Patients complain of constant painful thirst, amount of the drunk liquid in days can exceed 10 — 15 l. The daily amount of urine corresponds to the drunk liquid. Thirst and frequent urinations also disturb at night what leads to a sleep disorder, bed wetting. At children of younger age enuresis quite often is a precursory symptom of not diabetes mellitus. Overflow of a stomach liquid leads to braking of a food reflex. Children demand only water. Patients cannot be limited in liquid as the increased loss leads to fast dehydration. At not diabetes mellitus urine is emitted in large one-time numbers. It is transparent, colourless, has a scanty deposit, does not contain protein, sugar, its relative density low. Existence at patients of monosymptomatology in the form of not diabetes mellitus forces to carry out the differential diagnosis with a diabetes mellitus. At the last sugar level in blood is increased, urine has high relative density, the glycosuria is typical. — fosfatdiabt other forms, gluco-aminophosphate-diabetes (an illness to Debra Tony — Fankoni), a night glycosuria, a renal not diabetes mellitus — exclude on the basis of lack of characteristic clinical symptoms and excretion of substances with urine, typical for these diseases.
At not diabetes mellitus combination to other symptoms diagnosis of an illness of Hend — Schueller — Krischena does not represent difficulties. At a half of children the exophthalmos is noted. Some authors an exophthalmos as well as not diabetes mellitus, carry to the signs testimonial of the long chronic course of process. Protrusion of an eyeglobe can be unilateral and bilateral. At considerable pucheglaziya are possible disturbances of an axis of an eyeglobe, injury of a retina, an atrophy of an optic nerve and loss of sight. Development of an exophthalmos is caused by histiocytic proliferata in eye-socket bones, frontal and temporal bones. Tumoral process in a skull can lead to increase of intracranial pressure and hydrocephaly.
Involvement in process of lymph nodes is characteristic of Hend's illness — Schueller — Krischena. Increase in a spleen — a non-constant symptom (it is noted less than in half of cases), the hepatomegalia is stabler. In late stages of a disease development of cirrhosis and ascites is possible. Very often find the skin rash of petekhialno-papular character which is localized on a breast and a back. Damages of mucous membranes are generally limited to an oral cavity. Changes in lungs, as a rule, join during the late period and are an adverse predictive sign. Sometimes there is a specific defeat of cardiovascular system. In late stages of a disease the infiltration of a digestive tract which is followed by the dispepsichesky phenomena, emergence of blood in fecal masses is possible. The specific damage of kidneys which is shown an uric syndrome — a moderate hamaturia is occasionally observed.
At most of patients the remission lasting several years is caused by treatment. Involution of symptoms has a certain pattern. In the beginning changes of skin and mucous membranes disappear, lymph nodes, a liver, a spleen, an exophthalmos decrease. Most long bone defects keep, the reparation occurs during 1 year — 2 years. Becomes softer than the phenomenon of not diabetes mellitus, but without special therapy completely do not pass. In rare instances remission can spontaneously arise, without treatment. During the terminal period damage of lungs, cirrhosis, consecutive infections lead to a lethal outcome.
In peripheral blood increase in SOE is noted. The quantity of leukocytes are normal or is a little increased. In a leykogramma — a moderate eosinophilia. In the period of the expressed clinical picture of a disease normokhromny anemia is noted. The quantity of thrombocytes remains within age norm. At research of punctates of marrow it is possible to reveal xanthochromatic cells though this sign is optional. In a miyelogramma — a picture of irritation of myeloid and platelet sprouts of a hemopoiesis, in a late stage — oppression of an erythroidal sprout. Biochemical changes are similar to that at Letterer's illness — Ziva, but are expressed to a lesser extent.
At treatment use a combination therapy — hormones, cytostatic drugs, X-ray radiation. In the period of the expressed phenomena carry out long continuous or course discontinuous therapy by corticosteroids, supporting it with cytostatic drugs. X-ray radiation is used carefully, influencing the large centers of destruction. At not sugar mocheiznureniye use of an adiurskrin in the form of powder for inhalation through a nose or ointments is effective. At rhinitis enter subcutaneously Pituitrinum. Antibiotics, symptomatic therapy, a diet with restriction of fats enter complex treatment.



 
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