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Acute leukoses - a preleukosis - 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 recent years in literature there was a term "preleukosis" which is considered from two positions. On the one hand, the preleukosis is considered as an independent symptom complex what follows from that at influence of certain factors the preleukosis can be transformed to a leukosis. With another — most of authors hold the opinion that the preleukosis is an early stage of a leukosis. During this period there is a reorganization of a hemopoiesis without involvement in process of internals. It is possible to believe that in a preleukosis stage in marrow the clone of tumor cells without their innidiation only forms. Certain clinical symptoms, manifest for a preleukosis stage, no. Describe change of behavior of the child, slackness, refusal of participation in active games, unmotivated rises in temperature are possible. The most characteristic is disturbance of a marrowy hemopoiesis which is expressed in a hypoplasia of one, two or three sprouts of a hemopoiesis. However it is obviously not enough these signs to predict probability of development of leukemic process. Carry to approximate most reliable signs non-constant a blastemiya and blastoz marrow, even slightly expressed, within 10%. Changes of a hemopoiesis can be combined with moderate increase in a liver, spleen, lymph nodes, unmotivated fever, symptoms of immunodeficiency that facilitates establishment of the diagnosis (F. E. Feinstein and soavt., 1984; E. B. Vladimirskaya, 1984).
In an initial stage of an acute leukosis there are no pathognomonic syndromes. This period is characterized by so various symptomatology that even at oncological vigilance it is difficult to make the diagnosis. Lack of symptoms, specific to an acute leukosis, often leads to wrong diagnosis of more widespread and often found at children's age such diseases as acute respiratory viral infections, quinsies, anemias, rheumatism etc. Behind a mask of these diseases the first displays of an acute leukosis at children can begin. As a rule, the analysis of symptoms of an initial stage is carried out retrospectively. The acute leukosis at most of children develops gradually, for 1 — 3 month though cases of fast dynamics of clinical symptoms from the moment of the first signs to the developed illness picture are noted.
Existence of so-called small symptoms is characteristic of an initial stage of an acute leukosis. Lively and playful children without the visible reasons become sluggish, adynamic. Parents notice that at the child appetite worsens. There are complaints to fast fatigue. The attention on occupations decreases. The child aims to lie down, have a rest. Search of the objective reasons of such behavior, as a rule, does not yield results and all consider as feature of mentality, the constitution, an overload, forgetting to conduct elementary researches, in particular, of blood.
Initial symptoms of an acute leukosis can pass also behind a mask of an acute respiratory viral infection. The general indisposition, muscular pain are possible rise in temperature, sometimes to high figures. Existence of the catarral phenomena in the form of a hyperemia of a pharynx, slight rhinitis, sometimes conjunctivitis conduct thought of the doctor on the blazed way, prompting the diagnosis of a respiratory infection. Dynamic supervision in most cases is absent. At the same time such elements as the developing astenisation, it is long lasting "tail" of temperature reaction, have to guard the doctor. The blood analysis at such patients is, as a rule, limited to two-three indicators and the leukopenia which is found often in an initial stage is besides attributed to a respiratory infection. At such children it is necessary to study the developed blood tests with calculation of a formula and quantity of thrombocytes.
In an initial stage of an acute leukosis fever can periodically be noted. Lack of the visible reasons of feverish reaction demands the careful clinical analysis and differential diagnosis of a number of diseases which have to include also an acute leukosis.
At certain patients the initial symptomatology of a leukosis is similar to that at quinsy. Along with the general symptoms (slackness, loss of appetite), there is fever and children complain of pain when swallowing. During objective survey it is possible to note a picture, typical for quinsy. An important point in differential diagnosis is that quinsy, as a rule, has necrotic character. Also the refrakternost to an antibioticotherapia and recurrence of quinsies has to guard.
At children of younger age it is possible to note ulcer and necrotic damages of a mucous membrane of an oral cavity. Children become uneasy, whimsical, completely refuse meal, they have temperature reactions. At objective survey on mucous membranes of cheeks, language, lips, on a hard palate define ulcers with necrosis sites. A widespread mistake is diagnosis at such children of stomatitis, use without effect of various rinsings, astringents etc., long supervision (treatment) at the stomatologist.
Singularity of a course of stomatitis, absence of therapeutic effect by comparison to data of a gemogramma have to orient correctly the doctor and promote early diagnosis of an acute leukosis.
Children of younger age option of the beginning of an acute leukosis can have an isolated damage of a digestive tract. Vomiting which is not connected with meal is observed. Dispatching frustration — a frequent liquid chair, deterioration in the general state — pallor, slackness, dehydration symptoms. At children of advanced age the abdominal syndrome — the sharp colicy pain in a stomach passing spontaneously can be noted.
In some cases the acute leukosis demonstrates signs of a kostnosustavny syndrome. Against the general feeling sick and already mentioned general symptoms caused by intoxication the ostealgia and (or) in joints appears. Pain can be cutting, amplify at night and dominate in all clinical picture. Due to certain difficulties of poll of children sometimes it is not possible to differentiate bone or joint character of pain: children complain of hand or legs pain. At objective inspection reveal morbidity in long tubular bones, morbidity and restriction of mobility in joints, quite often their swelling. Such clinical signs are the basis for statement of the wrong diagnosis of rheumatism or a pseudorheumatism. The careful kliniko-hematologic analysis (existence of a leukopenia, anemia, thrombocytopenia, uncharacteristic for these types of kollagenoz, detection in peripheral blood of blast cells), a torpidnost to therapy have to help the correct differential diagnosis. It should be noted that the wrong diagnosis of rheumatism and purpose in this regard of steroid hormones smoothes initial symptoms of an acute leukosis and considerably complicates establishment of the correct diagnosis.
In a clinical picture of bone pathology at an acute leukosis it should be noted defeat of a rachis — a brevispondiliya. As a result of structural disturbances of a body of vertebrae are flattened. The X-ray pattern is characterized by existence of diffusion osteoporosis. At a progression of a leukosis osteolytic disturbances in bone substance accrue. Existence of a system brevispondiliya can conduct to diagnostic mistakes when such children are hospitalized in neurologic hospitals.
In an initial stage of an acute leukosis preferential damage of heart also meets. At patients appear the asthma amplifying at an exercise stress and in horizontal position, heartbeat, pain in heart. Define expansion of borders of heart, tachycardia, muting of tones. On the roentgenogram of bodies of a thorax it is possible to see a median shadow in the form of a pipe. At ECG research — decrease in a voltage. It gives the grounds for diagnosis of cardiovascular pathology, a lymphogranulomatosis of mediastinal lymph nodes.
Early symptom of an acute leukosis at children to which parents pay attention is the increasing pallor at deterioration in the general condition of the child. At a blood analysis find anemia in such children, in most cases normokhromny or hypochromia. The unilateral and superficial assessment clinical and datas of laboratory, identification in the anamnesis of elements of improper feeding that occurs at most of healthy children, leads to wrong diagnosis of scarce anemias. At the same time quite often analyze only data of red blood (quantity of erythrocytes, hemoglobin, a color indicator), without counting leukocytes, a formula of white blood, thrombocytes.
If in peripheral blood find a pancytopenia (a leukopenia, anemia, thrombocytopenia) quite often mistakenly diagnose hypoplastic anemia which clinical picture is similar to a leykopenichesky phase of an acute leukosis. The increasing pallor, temperature increase, insignificant hemorrhagic manifestations is observed. In a miyelogramma — signs of oppression of all sprouts of a hemopoiesis. Only careful and purposeful search of blast forms can clear up establishment of the correct diagnosis.
In an initial stage the acute leukosis can be shown by the isolated hemorrhagic syndrome caused by thrombocytopenia. On skin at patients find single, multiple hemorrhages of various form and size are more rare. Observe nasal and gingival bleedings, emergence of blood in Calais less often. These symptoms lead to wrong diagnosis of hemorrhagic diathesis, in particular thrombocytopenic states.
Quite often initial symptoms of an acute leukosis is the hepatolienal syndrome, the isolated increase in a liver or spleen. It is necessary to remember that at children, especially younger age, the spleen often increases at various infections and intoxications. It is also necessary to remember that children till 2 — 3 have a palpation of a liver and spleen below a costal arch it is considered the physiological phenomenon. Therefore increase in a liver and spleen should be considered proceeding from age of the child, the previous factors, the exact sizes of the studied bodies. Experience shows that the splenomegaly serves one of the few reliable symptoms of an acute leukosis helping early and correct diagnosis.
The defeat of respiratory system dramatizing pneumonia can be seldom found option of the beginning of an acute leukosis. It is caused by an infiltration of pulmonary fabric blast cells — a leukemic pneumonitis. A condition of patients heavy, fever, cyanosis of a nasolabial triangle, short wind, dry cough are noted. Objectively listen rigid, sometimes to bronchial breath, dry rattles, is more rare — crepitant and wet. Involvement in process of a pleura with a clinical picture of exudative pleurisy is possible. The percussion sound is changed over places of an infiltration. On the roentgenogram pulmonary drawing is strengthened, sometimes reveal focal shadows. In such cases usually appoint antibiotics. However absence of effect of an antibioticotherapia, feature of a clinical picture — discrepancy of weight of a state to the available pulmonary defeats — have to raise doubts in the diagnosis of bacterial pneumonia.
Very seldom in an initial stage of an acute leukosis defeat of the central nervous system meets. Main clinical signs: loss of appetite, easy fatigue, weakness, drowsiness, block. Then there are mental and neurologic symptoms. Paresis of a facial nerve, a nystagmus, an anizorefleksiya, positive meningeal symptoms are possible. Children become whimsical, irritable, complain of a headache, they reduce memory and attention. The diagnosis becomes clear at research of cerebrospinal fluid in which blast cells are defined.
In an initial stage of an acute leukosis allergic rashes and shingles are sometimes observed.
Children of chest age with inborn leukoses the first manifestations can have leukemids on skin which at other forms of an acute leukosis meet seldom and, as a rule, in a closing stage of pathological process.
One of early symptoms of an acute leukosis, first of all a lymphoblastoid form is a hyperadenosis. More often cervical and submaxillary lymph nodes are surprised though increase in several groups is possible at once (usually symmetrically). Lymph nodes of an elastic consistence, at a palpation are painless, are not soldered to surrounding cellulose, are mobile, skin over them is not changed Reaction of lymphatic system at children's age often is observed at viral infections, chronic nasopharyngeal processes, kariyesny teeth etc. It complicates the correct diagnosis. However the wavy current, return to initial symptomatology have to guard after a while doctors. The widespread mistake is a purpose of thermal and physiotherapeutic procedures (UVCh) which stimulate tumoral growth. Therefore in all cases which are followed by a hyperplasia of lymph nodes it is necessary to avoid thermal procedures (A. I. Vorobyov, 1979). Now quite seldom tubercular defeat of lymph nodes which differs from an acute leukosis in suppuration and formation of fistulas meets.
Rare form of the beginning of an acute leukosis — defeat of sialadens (Mikulich's syndrome). At children the swelling of a parotid gland, as a rule, on both sides is noted. The person becomes bloated. At a palpation the picture is similar to epidemic parotitis. However at epidemic parotitis defeat usually unilateral, at a palpation a tumor dense and painful in the center. In process of removal from the center density and morbidity decrease. The epidemiological anamnesis, fast return dynamics of clinical symptoms, uncharacteristic for leukemic process matter. At Mikulich's syndrome at the same time with sialadens the lacrimal glands can be also involved in process.
The initial stage of an acute leukosis is characterized by gradual development of clinical symptomatology and waviness of a current when after an aggravation of symptoms there comes relative wellbeing again. Less often the illness develops violently, reminding heavy septic process. The condition of the child suddenly worsens, appears high fever, anemia and thrombocytopenia quickly progress. On mucous membranes of an oral cavity necroses appear.
The variety and not specificity of clinical symptoms of an acute leukosis in an initial stage considerably complicate the correct and early diagnosis of a disease. Therefore in the situations which are listed above there shall be a comprehensive investigation of blood in dynamics. During this period there are already hematologic shifts specific to an acute leukosis.
According to many authors, in an initial stage indicators of red blood change slightly. So, the amount of hemoglobin and erythrocytes remains at the high level. Anemia is, as a rule, observed at the height of an illness.
In an initial stage of an acute leukosis changes of white blood are more typical. The leukopenia is most often noted, is more rare — a leukocytosis. Existence at children of a long and resistant leukopenia of not clear genesis or accompanying the pathological syndromes which are listed above is the direct indication for research of marrowy punctate. This situation belongs also to cases of not clear and resistant leukocytosis. The most characteristic and precursory symptom of an acute leukosis at children is an emergence in peripheral blood of blast cells. It is worth to remember that at children of younger age in connection with lability of a hemopoiesis at various toksiko-septic states it is possible to observe emission of young cells in peripheral blood. However such reactions as neutrophylic shift are characterized by existence of all intermediate forms to the left — from blast to mature elements of a haemo cytopoiesis. For differential diagnosis the return hematologic dynamics at reduction of toksiko-septic process matters. Repeated research of composition of peripheral blood (in dynamics) and marrowy punctate is necessary.
Important value has definition of quantity of thrombocytes. This indicator at most of children, as a rule, decreases. In early terms increase in SOE is also noted. For the correct statement of the diagnosis it is important to investigate marrow punctate: at an acute leukosis find the increased maintenance of blast cells (tens of percent).
Only the careful and sequential analysis of set of clinical and hematologic data allows to establish the diagnosis of an acute leukosis in early terms and, therefore, in many respects promotes receiving positive therapeutic effect.



 
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