Aplastic anemia at children

Description:

Aplastic anemia — the disease of the hemopoietic system which is belonging to the category of myelodisplasias and expressed in sharp oppression or the termination of growth and maturing of all three cellular lines in marrow, or a so-called panmyelophthisis.
The expressed pancytopenia — anemia, a leukopenia, thrombocytopenia and a lymphopenia is characteristic of aplastic anemias.
Can be the reasons of aplastic anemia:
1. Chemicals (arsenic, salts of heavy metals).
2. Ionizing radiation.
3. Medicines (NPVS, cytostatics, mercazolil, analginum).
4. Infectious agents (viruses, m / about).
5. Autoimmune processes (hard currency, Shiren's syndrome).

Symptoms of Aplastic anemia:

Aplastic anemias are divided by weight on heavy at which quantity of granulocytes< 0,5 × 109/л, тромбоцитов < 10 × 109/л, а ретикулоцитов < 1 %; в костномозговом пунктате мало клеток; негемопоэтические клетки составляют > of 65%. Forms with the best indicators belong to not heavy. On number of neutrophils distinguish very severe form when quantity of neutrophils < 0,2 х 109/л, и умеренно тяжелую, когда количество нейтрофилов варьирует в пределах 0,2-0,5 х 109/л.
Fankoni's Anemia. Children have defects of development of a bone tissue (lack of a beam bone, the I finger of a hand, talipomanus, etc.); microphthalmias, inborn heart diseases, anomaly of kidneys, etc. are observed. The disease is shown usually from 4-10 years or earlier. Complaints to the general weakness, headache, tendency to catarrhal diseases. The liver, a spleen, lymph nodes are not increased. In blood test: anemia of normokhromny type, an anisocytosis with tendency to a macrocytosis; sometimes - шизоцитоз, basphilic stippling of erythrocytes; the quantity of reticulocytes at first is increased to 2,5%, however there are inadequate weights of anemia; further the reticulocytopenia up to their total absence develops. The moderate hemolysis, preferential hidden, without hyperbilirubinemia and a splenomegaly, but with existence of a reticulocytosis is possible; the expressed leukopenia, thrombocytopenia; SOE is increased to 65-80 mm/h. Marrow punctate low-cellular that is confirmed also at a trepanobiopsiya. In a miyelogramma the maintenance of plasmocytes, lipophages, pigmentophages is increased. The quantity of undifferentiated cells remains normal. The maintenance of cells of a granulotsitarny sprout is considerably reduced, and percentage of lymphocytes, on the contrary, is increased. The quantity of eritrokariotsit is increased with existence of megaloblastoid elements in the beginning, and then is gradually reduced. Disease is chronic; life expectancy from 1 to 18 years; death comes from bleeding, accidental infections.

Anemia of Estrena-Damesheka proceeds with the general defeat of a hemogenesis, but without congenital anomalies of development. Meets extremely seldom.

Daymonda-Blekfen's anemia is characterized by selective defeat of an erythrogenesis; sometimes also an eye is followed by damage of a skeleton. In clinic lack of bleeding is characteristic. At survey the grayish shade of skin, and also gepato-and a splenomegaly owing to a hemosiderosis attracts attention. In the general blood test reveal heavy plastic or hypoplastic anemia. The hemoglobin content decreases to 14 g/l; the quantity of leukocytes and thrombocytes remains normal, however at a hypersplenism these indicators decrease too. In a miyelogramma already at the beginning of a disease the maintenance of eritrokariotsit is reduced; a ratio of leukocytes/erythrocytes at Daymonda-Blekfen's anemia sharply increases - to 100:1 (normal 3÷4:1). The quantity of cells of granulotsitarny and megakariotsitarny sprouts remains normal; the cellular degeneration is expressed.
Development of aplastic anemia at children's age gives the grounds to suspect an inborn form of a disease; at simultaneous detection of defects of a bone tissue, malformations of internals the diagnosis of an inborn form of aplastic anemia becomes reliable. At a pancytopenia, decrease in cellularity of marrow at the expense of all sprouts of a hemopoiesis it is possible to diagnose Fankoni's anemia; if in the presence of symptoms of aplastic anemia there are no defects of development of a bone tissue and internals, then the diagnosis of aplastic anemia of Estrena-Damesheka is more probable. Aplastic anemia of Daymonda-Blekfen distinguishes the partial nature of defeat of a hemopoiesis from other inborn forms of aplastic anemias: mainly the krasnokletochny aplasia at safety of other sprouts of a hemopoiesis is observed.

Reasons of Aplastic anemia:

Aplastic anemia — the disease of the hemopoietic system which is belonging to the category of myelodisplasias and expressed in sharp oppression or the termination of growth and maturing of all three cellular lines in marrow, or a so-called panmyelophthisis.
The expressed pancytopenia — anemia, a leukopenia, thrombocytopenia and a lymphopenia is characteristic of aplastic anemias.
Can be the reasons of aplastic anemia:
1. Chemicals (arsenic, salts of heavy metals).
2. Ionizing radiation. (see Maria Sklodovskaya-Curie)
3. Medicines (NPVS, cytostatics, mercazolil, analginum).
4. Infectious agents (viruses, m / about).
5. Autoimmune processes (hard currency, Shiren's syndrome).

Treatment of Aplastic anemia:

At Fankoni's anemia - transplantation of marrow from a singenny sibling (treatment in 70-75% of cases). At its impossibility appoint androgens (methandrostenolone at the rate of 0,2 mg/kg/days, testosterone-propionate - on 1-2 mg/kg/days) and other anabolic means within 3-6 months; improvement occurs in 6-8 weeks from an initiation of treatment. Replacement therapy - transfusions of eritrotsitny weight and a trombotsitny suspension at decrease in level of hemoglobin < 80 г/л, нейтрофилов < 1,0 х 109/л, тромбоцитов < 20 х 109/л. Активации гемопоэза способствует также применение эритропоэтина и ростовых факторов.
At Daymonda-Blekfen's anemia the main method of treatment is use of glucocorticoid hormones in a dose of 2 mg/kg/days during 4 weeks; at positive effect in 4 weeks the dose is gradually reduced to supporting. The effect is shown already in 2 weeks from an initiation of treatment; duration of therapy depends on clinical effect, expressiveness of side effects and complications of hormonal therapy. At steroidorezistentny forms transplantation of marrow is necessary. Pulse therapy by Methylprednisolonum is carried out at the heavy course of anemia.
Therapy of the acquired forms of aplastic anemia includes: high doses of Methylprednisolonum, immunosuppressive therapy by anti-lymphocytic globulin, cyclosporine A, cytostatics (Cyclophosphanum); hemopoietic growth factors, transplantation of marrow.
Methylprednisolonum is entered intravenously at the rate of 1 mg/kg/days in the first 9 days with a gradual dose decline to 0,08 mg/kg/days by 25th day of treatment. Cyclophosphanum in the first days is appointed on 45 mg/kg/days with a gradual dose decline to 7,5 mg/kg/days. Anti-lymphocytic globulin is entered at the rate of 15 mg/kg/days intravenously within 10 days; cyclosporine A - on 5 mg/kg/days accept inside within 14 days under control of arterial pressure, level of activity of ACT and ALT. Growth hemopoietic factors - the granulotsitarno-macrophagic colony stimulating factor is recommended to accept 5 mkg/kg/days in a dose; a granulotsitarny colony stimulating factor - on 5 mkg/kg/days. These drugs are appointed along with a course of cytostatic and immunosuppressive therapy. At therapy androgens can be used by anti-lymphocytic globulin. At the expressed anemia, leukopenias, thrombocytopenia carry out haemo component therapy. During long remission of aplastic anemia morphological parameters of a hemogenesis and immunity remain normal.
Antibacterial therapy is appointed at infectious diseases, preventively - at a neutropenia < 0,5 х 109/л, протекающей с лихорадкой до 38 °С. Если очаг инфекции не установлен, назначают антибиотики широкого спектра действия, комбинируя аминогликозиды III поколения с цефалоспоринами III поколения: амикацин, тобрамицин и другие с цефтриаксоном, клафораном и т.п. При их неэффективности используют карбопенемы: тиенам, имипенем, меропенем.
At a hemorrhagic syndrome haemo static therapy is carried out: e-aminocapronic acid, Dicynonum, etc. At a hemosiderosis of internals use chelate therapy: desferal in a dose of 20 mg/kg/days daily within 30 days; a repeated course - in 4 weeks. Indication: level of serumal iron> 1000 mg/ml. Splenktomiya is effective at the acquired form of the aplastic anemia proceeding with deep refractory thrombocytopenia, the expressed hypersplenism, a hemorrhagic syndrome.
Improvement, partial and full kliniko-hematologic remission, lack of effect can be results of treatment of aplastic anemia.