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Neuroleukosis - 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

 

 
In a modern leykozologiya much attention is paid to a neuroleukosis problem. The neuroleukosis is a specific defeat of the central nervous system; it meets at all forms of an acute leukosis, however most often complicates the course of an acute lymphoblastoid leukosis at children. The analysis of cases of a neuroleukosis confirms increase of frequency of incidence. It is connected with a variety of reasons. First, with increase in longevity, especially children with an acute lymphoblastoid leukosis. If earlier the neuroleukosis at a rapid current of tumoral process did not manage to be shown, then now at considerable lengthening of life process of defeat of a nervous system reaches peak. Secondly, cytostatic drugs do not get through a blood-brain barrier and the tumoral center in a nervous system has an opportunity to a progression. According to different authors, neuroleukosis frequency at children makes more than 50%. It is noted that the neuroleukosis develops more often at those patients at whom in a primary and active phase of a disease were a high leukocytosis, the expressed organomegaliya. It causes the big frequency of innidiation of leukemic cells.
Neuroleukosis symptoms, as a rule, find during remission, they are more rare happen the first displays of an acute leukosis. However innidiation of process is observed during the early period of a disease. Confirmation of it are works of A. Mastrangelo (1970) which found identical anomalies of chromosomes in blast cells of marrow at statement of the diagnosis and in blast cells of cerebrospinal fluid at development of a neuroleukosis.
As a rule, the neuroleukosis develops against hematologic wellbeing and marrowy remission. However it is regarded as recurrence of a disease, the factor defining the further adverse forecast.
Histologically at a neuroleukosis find the tumor cells which are densely filling a subarachnoid space, perivascular cracks of Virkhov — Robena in marrow. As a result of obstruction of entrances to perivascular cracks normal circulation of cerebrospinal fluid between intracerebral and subarachnoidal spaces is broken that leads to development of a gipertenzionno-likvorny syndrome. Except a diffusion infiltration leukemic cells of covers of a brain, the focal perivascular infiltration (leukemic small knots) which is quite often found in brain substance also meets. Leukemic small knots are more often localized in intensively vaskulyarizovanny sites of a brain — a gipotalasuma, a subarachnoidal layer of white matter of big hemispheres. Also the leukemic infiltration on the course of roots of cranial and spinal nerves can be noted.
The clinical picture of a neuroleukosis develops gradually. Mainly it consists of symptoms of an encephalomeningitis, a gipertenzionny syndrome.
In the early period children have phenomena of the general adynamy. Children become sluggish, whimsical, sleepy. Fast fatigue, the general weakness are observed. The behavior of the child changes, he becomes closed, irritable. The further clinical picture depends on nature of leukemic defeat of the central nervous system. Allocate several forms of a neuroleukosis: defeat mainly a meninx (meningeal), brain substances (encephalitic), mixed form (meningoentsefalitichesky), defeat of peripheral trunks and roots of a nervous system. Some authors describe a large number of defeats of the central nervous system. So, N. A. Alekseev and I. M. Vorontsov (1979) distinguish the following forms of a neuroleukosis: meningeal, meningoentsefalitichesky, the diencephalic syndrome, meningomiyelitichesky, epidurit, a plexitis, a polyradiculoneuritis. The most frequent are the first four forms.
Except signs of the general adynamy, at defeat of covers of a brain a precursory symptom of leukemic meningitis is the headache. The morning headache without accurate localization is characteristic. Often after awakening the attack of a headache is followed by vomiting. Vomiting arises suddenly when moving the head, is not followed by nausea. At patients the photophobia can be noted. Meningeal signs (a stiff neck, Kernig's signs, Brudzinsky) are expressed slightly or can be even absent. At brain substance defeat at patients the focal symptomatology — deviation of language, a nystagmus is noted. Development of paresis and paralyzes, a loss of consciousness, a convulsive syndrome, dysfunction of cranial nerves are possible, is more often than others nerves of a mosto-cerebellar corner (VI, VII, VIII) and visual are surprised. Asymmetry of nasolabial folds, a diplopia, decrease in sight and hearing is observed, sometimes up to total loss, etc. At defeat of hypothalamic area at children the diencephalic syndrome develops. There are increased thirst, appetite, obesity develops. A hyperthermia, fluctuations of arterial pressure, a sleep disorder are noted. Development of the not diabetes mellitus which is shown a polydipsia and a polyuria is possible. Along with obesity cases of adiposogenital dystrophy are described. Seldom the leukemic infiltration of peripheral nervous trunks and roots which is shown polyneuritis symptoms develops.
The important place, especially in early diagnosis of a neuroleukosis, additional methods of research borrow. The most valuable information is given by the analysis of cerebrospinal fluid. In liquor the cytosis — from cells, single to several thousand, is observed. Absolute criterion is detection of blast cells. Increase of pressure of cerebrospinal fluid, increase in level of protein, decrease in level of sugar is characteristic. However in some cases, especially at the isolated defeat of substance of a brain, changes of liquor can not be. Existence of clinical signs of a neuroleukosis demonstrates the developed process. Therefore for the purpose of earlier diagnosis and treatment it is important to carry out a spinal puncture timely.
Certain information is given by research of an eyeground which helps to reveal congestive disks of optic nerves. The kraniografiya is of great importance. Refer discrepancy of seams, strengthening of drawing of manual impressions to radiological symptoms of a neuroleukosis.
V. I. Kurmashov (1980) attaches significance to change of density of a bone tissue of the Turkish saddle in the form of osteoporosis, especially dorsums sellae. On kraniogramma porosity of flat bones of a skull against osteoporosis is also noticeable. On the basis of the analysis of results of a kraniografiya at the children having a neuroleukosis, the author allocated a preclinical form. At such children the kliniko-neurologic status does not differ from healthy, but there are already kraniografichesky and ophthalmologic changes. It, undoubtedly, has practical value for timely diagnosis of a neuroleukosis. As it was already specified, development of a neuroleukosis demonstrates the end of remission even at the kept marrowy hemopoiesis. Its emergence considerably worsens the further forecast. Therefore for the purpose of achievement of long remissions at an acute leukosis the big place is allocated to prevention of a neuroleukosis.
For prevention use various schemes of therapy. Treat them: 1) introduction of tsitostatik to the spinal channel; 2) cranial or craniospinal radiation; 3) combination therapy: endolumbar administration of cytostatic drugs and cranial radiation.
It is established that the most effective remedy is the combination therapy. Endolumbar introduction of a methotrexate allows to carry out sanitation of liquor back and a brain. Cranial radiation causes death of the tumoral infiltrates located in brain substance (Page R. Pinkerton, S. M of Chessells, 1984). A number of authors specify that early administration of cytostatic drugs to the spinal canal prevents development of metastasises in a nervous system. Therefore prevention of a neuroleukosis can be begun during induction therapy, on 1 — the 2nd week of treatment. For this purpose endolyumbalno enter metotraksat in a dose 12 mg/sq.m once a week. The course consists of 4 injections. If during induction of remission prevention was not carried out, then it needs to be carried out during remission consolidation. Co-administration of a methotrexate (parenterally and endolyumbalno) is inadmissible as it leads to development of a tsitopenichesky syndrome. Further prevention of a neuroleukosis includes cranial radiation. Radiation therapy is carried out to the remission period. The general dose of gamma irradiation makes 24 Gr. Radiation is carried out from four fields (frontal, occipital and temporal shares) during 3 — 4 weeks on 0,15 — 0,2 Gr on one session. At the same time children receive the supporting chemotherapy. In view of danger of development of a tsitopenichesky syndrome during this period careful hematologic control is necessary. In some cases even when carrying out all preventive measures recurrence of a neuroleukosis is possible. From these positions preventive actions are recommended to be continued throughout the entire period of remission. Single endolumbar introduction of a methotrexate is carried out to time of courses of reinduktsionny therapy. At the same time for the analysis take cerebrospinal fluid, that is the carried-out puncture has both medical, and diagnostic value.
At development of a neuroleukosis also use endolumbar administrations of drugs (a methotrexate, tsitozin-arabinozid, Prednisolonum) and radiation. The method of therapy depends on a form of defeat of the central nervous system though radiation therapy is used much less often, generally at inefficiency of treatment by cytostatic drugs. A neuroleukosis usually treat endolumbar introduction of a methotrexate in a dose of 12 mg/sq.m, at the same time it is possible to enter Cytosarum and Prednisolonum into the spinal canal. Injections carry out 2 times a week. The dose of Prednisolonum makes 12 mg/sq.m. Cytosarum enter 5 mg in an initial dose. Then Cytosarum dose at good tolerance can be raised on 5 mg on introduction — 10 — 15 — 20 — 25 mg. Efficiency of Cyclophosphanum in a dose of 100 — 150 mg/sq.m at subnormal indicators of a leukemic cytosis of liquor is proved (V. I. Kurmashov, 1985).
At treatment of a neuroleukosis at children the by-effects connected with neurotoxic effect of drug can be noted. The general state worsens, meningeal signs amplify, vomiting, a headache, confusion and a black-out are noted. For decrease in a neurotoxicity drugs need to be dissolved only in the bidistilled water. When carrying out a spinal puncture it is desirable that the amount of remote liquor corresponded to amount of the entered solution. In treatment of a neytroleykoz also according to indications appoint dehydrational, anticonvulsant therapy and other symptomatic means.
At treatment of a neuroleukosis against marrowy remission by the patient continue a course of a maintenance therapy. However the probability of development of a tsitopenichesky syndrome allows to cancel for 1 month the general polikhimioteration (N. A. Alekseev, I. M. Vorontsov, 1979). If the neuroleukosis develops against marrowy recurrence, it is necessary to carry out in parallel therapy of one and other state according to the standard programs. Usually active treatment of a neuroleukosis for 3 — 4 weeks leads to normalization of the kliniko-neurologic status. Endolumbar administration of drugs is cancelled when obtaining triple normal analyses of cerebrospinal fluid.
Further tactics of maintaining patients is defined depending on a complex of events which were held during the period of prevention of a neuroleukosis. So, if radiation of the head was offered, then repeated radiation therapy is unsafe and it is not appointed, as a rule. If before radiation was not, then it can be seen off according to the scheme stated above. In the subsequent during courses of a reinduktion of remission do



 
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