IDIOPATHIC HEMOSIDEROSIS OF LUNGS (haemosiderosis idiopathica pulmonum; Greek idios own, special + pathos suffering, disease; hemosiderosis; synonym: idiopathic brown induration of lungs, an essential pulmonary hemosiderosis, pneumohemorrhagic remittiruyushchy hypochromia anemia, Tselen's syndrome — Gellerstedta) — the disease which is characterized by repeated hemorrhages in lungs, development of a hemosiderosis and sclerosis of lungs, anemia.
For the first time And. of l. R. Virkhov in 1864 described. The disease meets seldom, children at the age of 3 — 8 years are ill preferential; cases of a family disease are noted.
The etiology and a pathogeny
the Aetiology and a pathogeny are studied insufficiently. Assume that primary underdevelopment of an elastic framework of pulmonary vessels therefore there are aneurisms, stagnation of blood and diapedetic hemorrhages in lungs is a cause of illness. The role of infections and intoxications is not excluded (cases are frequent And. of l. after whooping cough, measles, acute respiratory diseases, pneumonia) though the role of microbes and viruses in genesis of a disease is not proved. In development of a disease allergies began to attach great value and autoallergies (see), bonds with a frequent combination And. of h.p. Shenleyn's disease — Genokh, a glomerulonephritis, myocarditis, and also proceeding from features of disease in the form of crises with increase in level of immunoglobulins of blood, effect of an early extirpation of a spleen and need of steroid therapy.
Changes in pulmonary fabric at And. of l. are similar to changes at secondary brown consolidation of lungs (see), however hemosiderosis (see) it is expressed more sharply and it is more often combined with multiple hemorrhages. In air cells, interalveolar partitions, limf, vessels and limf, nodes of lungs there are accumulations of the macrophages loaded with hemosiderin and ferritin. More sharply and peculiar the elastic framework of a lung and pulmonary vessels changes. The hyperplasia of elastic fabric is combined with its destruction, gaps and twisting of fibers, elastolizy and giant-cell macrophagic reaction to scraps of elastic structures; quite often there is their ozhelezneniye and calcification.
The clinical picture
the Disease is shown by a triad of symptoms: hypochromia anemia, a periodic pneumorrhagia and characteristic symmetric damage of lungs (diffusion, dense, spotty, setevidny blackouts in lungs, hl. obr. in average and lower parts — a picture of «bee cells»), a cut comes to light radiological.
Periodically repeating attacks of fatigue, tachycardia, cyanosis after which in 12 — 24 hours an asthma, pallor, the painful cough which is quite often followed by vomiting develops are characteristic. In a phlegm and emetic masses at the same time blood, in a phlegm is found, besides, (siderophages) find so-called cells of heart diseases.
Bad attacks can be followed by an abdominal pain, fever, jaundice.
In blood significant increase in content of iron. Anemia is sharply expressed (number of erythrocytes at some patients to 600 — 800 thousand in 1 mkl). The hypochromia, anizo-and a poikilocytosis of erythrocytes, existence of normoblasts, a large number of reticulocytes, a leukocytosis, shift of a formula of white blood to the left, an eosinophilia is noted. Miyelogramma points to strengthening of an erythroidal number of a hemopoiesis — a leucio-erythroblastic ratio 1: 2 (3:1 is normal) at normal process of a differentiation and maturing of cells of a myeloid row. Bilirubin of blood in the period of crisis is a little raised. The disproteinemia due to increase in amount of globulins (a beta and scale) is noted. Increase in a liver and spleen is sometimes observed. There is a hamaturia, a cylindruria, an albuminuria. Hypertensia is very often noted.
At a bronkhoskopiya the endobronchitis testimonial of inflammatory changes in bronchial tubes quite often comes to light (see. Bronchitis ). Disturbance of bronchial passability is noted.
the Diagnosis is made on the basis a wedge, displays of a disease, indicators of blood, data rentgenol. researches, bronkhoskopiya, detection of the macrophages loaded with hemosiderin in a phlegm and rinsing waters of a stomach (especially at children of early age who swallow a phlegm).
Changes in lungs at And. of l. it is necessary to differentiate with changes at miliary tuberculosis (see. Tuberculosis of a respiratory organs ), histoplasmosis (see), pneumomycoses (see), Benye's diseases — Beck — Shaumanna (see. Sarcoidosis ).
The hypertensia observed at And. of l., it is necessary to distinguish from hypertensia at inborn heart diseases — a patent ductus arteriosus, not fusion of interatrial and interventricular partitions with Eyzenmenger's complex or without it and acquired — a stenosis of a mitral orifice (see. Heart diseases inborn , acquired ).
Apply desferal in a dose of 50 — 70 mg on 1 kg of weight; its action is based on binding of iron and its removal from an organism by kidneys. The criterion determining duration of treatment a desfer scarlet is release of iron with urine. Apply also steroid hormones, sometimes delete a spleen. According to vital indications appoint hemotransfusion (the donor shall be picked up individually since quite often slightest incompatibility involves an aggravation of symptoms of the patient).
Sometimes under the influence of treatment or spontaneously a condition of patients can improve; remission continues from several weeks to 5 — 6 years, then crisis develops again. At long disease (2 — 3 years) and often repeating crises at children lag in the weight and growth is noted, deformation of a thorax, fingers («drum sticks»), develops right ventricular insufficiency, borders of heart extend, pulmonary insufficiency appears hron. Most of patients perishes at children's age.
See also Hemosiderosis .
Bibliography: Hamburg R. L., etc. To a question of a pathogeny and treatment of an idiopathic hemosiderosis of lungs at children, Vopr, okhr. mat. also it is put., t. 8, No. I, page 81, 1963; Dolgoplosk N. A. and Skaldi-n and A. S. Case of an essentsialnogogemosideroz of lungs, Vestn, rentgenol, and radio-gramophones., Mb 1, page 88, 1971, bibliogr.; Kassirsky I. A. ialekseev G. A. Clinical hematology, M., 1970; Turbine H. C. Use of desferal in treatment of sideroses of various origin, Probl, gematol, and a modulation, blood, t. 13, No. 7, page 47, 1968, bibliogr.; Fadeyeva M. A., etc. An idiopathic hemosiderosis of lungs at children, Vopr. okhr. mat. also it is put., t. 21, No. 4, page 37, 1976, bibliogr.; Hutsi-sh in and l and G. E. Desferal test in diagnosis of a hemosiderosis at patients with hemoglobinopathies, Laborat, business, No. 11, page 660, 1971, bibliogr.; it, Treatment by desferal of a hemosiderosis at hemoglobinopathies, Rubbed. arkh., t. 43, century 10, page 98, 1971, bibliogr.; Blood and its disorders, ed. by R. M of Hardisty a. D. J. Weatherall, Oxford, 1974; Mac Donald R.A. Hemochromatosis and hemosiderosis, Springfield, 1964, bibliogr.; Roberts L. N., Montes-sori G. Patterson J. G. Idiopathic pulmonary hemosiderosis, Amer. Rev. resp. Dis., v. 106, p. 904, 1972.
A. V. Mazurin; V. V. Serov (etiol., patog., stalemate. An.).