Histologically, diffuse infiltration of cordarone pills reticular cells is noted in the lesions. The prognosis is bad. Sepsis, eosinophilic bone granuloma, acute leukemia develop. Treatment. There are reports of cure with antibiotics (erythromycin, tetracycline), corticosteroid drugs. Treatment with B vitamins, blood transfusions is indicated. Patients are subject to systematic dispensary observation. Abt-Letterer-Siwe disease is called acute systemic progressive histiocytosis, which has a hereditary cause. Most often, the disease begins to develop in early childhood, but occasionally its first symptoms appear only during puberty. It is believed that Abt-Letterer-Siwe disease is more common in boys than girls.
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Other diseases from the group Diseases of the blood, hematopoietic organs and individual disorders involving the immune mechanism: Synonyms: reticulo-endotheliose aigue hemorrhagique des nourrissons. Why Abt-Letterer-Siwe disease occurs The disease is primarily due to a genetic mutational factor, however, the onset of the disease is usually associated with the presence of a viral or bacterial infection (tonsillitis, influenza, otitis media, etc.). An unusual course of the disease indicates its neoplastic nature.
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How Abt-Letterer-Siwa disease proceeds Most often, Abt-Letterer-Siwe disease begins in the first year of a child's life, sometimes it happens even during amiodarone pills period. Suddenly, the baby's body temperature rises sharply (up to 40 degrees), after which the temperature can either rise or fall. Sometimes for a long time the temperature remains subfebrile. Hemorrhagic papules can be seen on the scalp and skin of the shoulder blades, abdomen and chest. In their center, necrosis develops, turning into the formation of small dotted scars. Often, small erosions and hemorrhages are noted on the mucous membranes of the mouth and genital organs.
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Patients often develop severe polyadenopathy and hepatosplenomegaly. Quite often, otitis media and mastoiditis are diagnosed, their distinguishing feature is resistance to antibiotic treatment. Many children suffering from Abt-Letterer-Siwe disease have damage to the bone tissue of the skull, ribs, collarbones, shoulder blades, and pelvis. Often this is accompanied by pain, swelling in the surrounding tissues. A study using fluoroscopy can reveal foci of Cordarone pills, osteolysis, periostitis, decalcification.
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In patients with Abt-Letterer-Siwe disease, changes are also observed in the respiratory organs: emphysematous, small-focal, infiltrative.
How Abt-Letterer-Siva disease is treated One of the main methods of treatment is the appointment of glucocorticoids, which are designed to inhibit the function of the reticulohistiocytic system. Such treatment stimulates hematopoiesis and normalizes metabolism. Usually, courses of treatment with glucocorticoids are quite long, the dose of the drug must be gradually reduced before the end of treatment, sometimes it takes several months, but it can often be years. It is important that drug withdrawal be completed when the body enters remission, not earlier.
Sometimes the doctor may prescribe cytotoxic drugs, anabolic drugs, gamma globulin, vitamin therapy. According to the indications, X-ray therapy is prescribed for the affected bone tissue.
With the development of Abt-Letterer-Siwe disease, the general condition of the baby deteriorates sharply. Low weight gain, pale skin, weakness are especially characteristic of this disease. A blood test also shows a pathological picture: a combination of cordarone, leukopenia or leukocytosis, monocytosis. The myelogram shows polymorphism of bone marrow cells.
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Children suffering from Abt-Letterer-Siwe disease are prescribed increased doses of glucocoricoids, and antibiotics are also prescribed in case of viral and bacterial diseases, such as measles, scarlet fever, chicken pox, SARS. This feature of treatment is dictated by the fact that Abt-Letterer-Siwe disease tends to worsen during such periods.
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You can either write your own. Abt-Letterer-Siwe disease - Acute systemic progressive hereditary histiocytosis. Usually occurs in early childhood, less often adolescence, mostly in males. The possibility of a viral or bacterial infection is assumed (in the anamnesis of children, infections are often noted - tonsillitis, otitis media, sepsis, etc.). The steadily progressive course of the disease may indicate the neoplastic nature of the process.
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Symptoms of Abt-Letterer-Siwa disease. The disease usually begins in infancy, sometimes even in the neonatal period, with a sudden rise in temperature to 40 ° C. Then the temperature curve acquires a hectic character, but sometimes it can be subfebrile. On the scalp, in the area of the shoulder blades, abdomen and chest, a lot of hemorrhagic scaly lichenoid papules and petechial elements appear. In the center of the papules, necrosis occurs, followed by the formation of dotted scars; sometimes infiltratetraditional tumor formations, leaving larger scars. Some patients with Abt-Letterer-Siwe disease may have onychia and paronychia. Erosions and hemorrhages are often noted on the mucous membranes of the mouth and vulva.
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There may be changes in the lungs of an infiltrative, small-focal and emphysematous nature, which are less often detected physically, but mainly radiologically (increased basal pattern, formation of cystic cavities, thickening of the walls of amiodarone online, the presence of emphysema vesicles).
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The genetic mutation factor plays an important role in the pathogenesis of the disease. Characteristic features are pronounced polyadenopathy and hepatosplenomegaly. Otitis and mastoiditis are often noted, which are resistant to antibiotic therapy and surgical intervention. In most patients, the bones of the skull, ribs, collarbones, shoulder blades, limbs, and pelvis are affected, which is accompanied by pain and swelling of the soft tissues. On radiographs, foci of amiodarone, osteoporosis, osteolysis, decalcification zones of a cystic nature, periostitis are determined.
The general condition of the child deteriorates rather quickly, appetite disappears, the skin becomes pale, poor weight gain, weakness are noted. In the peripheral blood: hypochromic anemia with aniso- and poikilocytosis, leukopenia or leukocytosis with neutrophilia and a left shift in the leukocyte formula to myelocytes; some patients have monocytosis. The myelogram is characterized by polymorphism of bone marrow cells, sometimes the content of reticulohistiocytic elements is increased.
Treatment of Abt-Letterer-Siwa disease. The appointment of glucocorticoids is pathogenetically justified, since they inhibit the function of the reticulohistiocytic system, stimulate hematopoiesis, normalize metabolism, etc. They are prescribed for a long time - until the clinical signs of the disease disappear. The dose of the drug (in terms of prednisolone) should be from 1 to 2 mg per 1 kg of body weight per day. A gradual decrease in the dose of the drug continues for several months, and sometimes years, until a more or less prolonged remission.
In cases of accession of intercurrent diseases (OVRI, measles, etc.), as a rule, an exacerbation of the disease occurs, which dictates the need to increase the dose of glucocorticoid for this period and prescribe antibiotics for 3-4 weeks. Courses of antibiotics are periodically repeated. In combination with glucocorticoids, a number of patients are prescribed cytostatic agents (6-mercaptopurine, leukeran, degranol, etc.) for health reasons.