Clinical picture of lung histiocytosis, its diagnosis and treatment

Lung histiocytosis is a systemic disease characterized by increased proliferation of histiocytes with the formation of histiocytic infiltrates in the lungs.

This disease affects 5% of the population aged 20 to 30 years.

Etiology and pathogenesis of

The etiological factors and pathogenetic mechanisms of this disease are not fully understood. So some scientists attribute a histiocytosis of the lungs to oncological pathology, and some - to disease accumulation.

This disease is characterized by increased proliferation of histiocytes, their accumulation in various tissues with the formation of granulomas. Histiocytic infiltrates also contain lymphocytes, eosinophils, plasmocytes, proliferated reticular fibers, which leads to pneumofibrosis. In addition to lung tissue damage, the histiocytes infiltrate blood vessels with subsequent development of fibrosis and the formation of cystic cavities.

Basic forms of the histiocytosis of the lung

Depending on the clinical and radiologic pattern, the following forms of histiocytosis are distinguished:

• Abta-Letterer-Sieve disease
• Heta-Schuller-Krystchen disease
• disease Taratynova

Clinical picture

Histiocytosis of the lung more often has an asymptomatic beginning and is revealed during preventive examinations. As the process progresses, dyspnea, a dry cough, chest pains occur due to the development of spontaneous pneumothorax. Violated the general condition of the patient, for example, there is weakness, fatigue, a strong decrease in body weight, fever.

When percussion of the lungs, a clear pulmonary sound is heard in the early stages, and if there is emphysema in later stages - boxed, tympanic sound is heard when the pneumothorax is attached. During auscultation, weakened vesicular breathing is heard, dry rales.

In especially severe cases, when histiocytic infiltration extends beyond the lung, the clinical picture will be characterized by the appearance of anemia, hepatomegaly, splenomegaly, mediastinitis, pneumonia, signs of diabetes insipidus, exophthalmos, skin lesions like petechial rash, seborrhea, bone pain.

Diagnosis of pulmonary histiocytosis

The diagnosis of "lung histiocytosis" is made on the basis of the following studies:

1. of a general blood test, which is characterized by signs of anemia, leukocytosis, thrombocytopenia;
2. general urine analysis-proteinuria, microhematuria, cylinduria can be observed;
3. in the biochemical analysis of blood there is an increase in sialic acids, seromucoid, globulins, bilirubin, creatinine, urea;
4. during immunological examination there is an increase in the number of immunoglobulins, CEC, decrease in the level of T-suppressors;
5. X-ray study, the results of which are characterized by the presence of focal blackouts, increased pulmonary pattern, signs of interstitial fibrosis, in severe cases, the "honeycomb" pattern develops due to cystic-bullous tissue damage.
6. biopsies: in the biopsy specimens obtained, granulomas are determined, which consist of histiocytic cells.
7. Computed tomography: the presence of cystic-bullous formations in lung tissue.

Treatment and prognosis of the pulmonary form of histiocytosis

In the treatment of lung histiocytosis, 2 directions are distinguished:

• medication;
• surgical.

Conservative treatment consists in the appointment of corticosteroids up to 12 months in the amount of 0.5- 1 mg / kg of body weight, followed by a gradual decrease in dosage. With the progression of the process and the absence of the effect of corticosteroids, cytostatics are used, for example, methotrexate, vinblastine, cyclophosphamide.

Surgical methods are used for localized forms of histiocytosis in combination with radiation therapy. They consist in the removal of histiocytic infiltrates, lobectomy, pneumonectomy, pleurrectomy, and in particularly severe cases with the development of respiratory failure, a lung transplant is performed.

In generalized forms of histiocytosis, an unfavorable outcome occurs in 70% of cases. With localized forms, the prognosis is very favorable.