Histiocytosis is a group of rare pathological conditions based on multiplication by dividing the cellular structures belonging to the monocyte system( this is a special kind of leukocytes).
What is it?
In general, histiocytosis X( X) is referred to as special blood pathology. If lymphoid, mucous or skin tissues are affected, pathological processes develop mainly from Langerhans cells, if the nervous system is affected, then from microglia, with bone localization from osteoclasts.
Causes of pathology
Although modern diagnostic and technological science is constantly developing, it has not yet been possible to determine exactly the pathogenetic origin of histiocytosis.
For example, with histiocytosis based on the cell structures of Langerhans, phagocytosis cells form scar tissue. So usually develops pulmonary or bony histiocytosis, which is more likely to affect patients older and younger children, although not immune from pathology and adult population.
The development mechanism is based on the direct participation of cells, which for some reason are damaged at the initial stage of the formation of their specific specialization. Then these damaged cellular structures begin to accumulate in a specific place. Here they produce prostaglandin and cytokine substances, which leads to the destruction of nearby tissues.
The immune system sends plasma, lymphocytic and phagocytosis cells, as well as eosinophils and neutrophils, to the zone of congestion of langergant cellular structures, which, having reached the goal, are transformed into an infiltrative compaction that passes into scar tissue.
Forms of the disease
Specialists identify several specific histiocytosis forms:
- Granuloma of the eosinophilic form - is considered the most common type of pathology and is found in approximately 65-80% of clinical cases of histiocytosis based on the cell structures of Langerhans. Primary schoolchildren and young patients are subject to pathology before the age of 30;
- Abt-Letterer-Sieve Syndrome - a similar form is found less often - only in 20-35% of patients with histiocytosis of Langerganskletnochnogo origin. The predominant age of patients is children under 2 years;
- The following histiocytosis form is typical for small children 2-5 years of age - this Hend-Schüller-Krischen pathology .It is diagnosed in 20-35% of histiocytosis cases.
Symptoms of
For each specific form, there is individual symptomatology.
Pathological processes are localized on cranial, humeral and femoral, vertebral and pelvic bone structures. In the place of the lesion:
- An intense pain is felt, especially worse with a heavy load;
- Swelling observed;
- A tumor-shaped nodular seal is formed;
- Chronic fatigue;
- Unexplained weight loss;
- Skin pimple;
- The motor possibilities for localization in the bones of the shoulders, hips, pelvis or spine are substantially limited.
With the progression of histiocytosis pathology, the pain symptom acquires a permanent character, exhausting patients even in a calm state. There is a hyperthermia, at which temperature values are kept at 39 ° C.With vertebral localization, patients easily identify scoliosis of the spine, stooped posture.
Speaking of the Abd-Letterer-Sive syndrome , it is characterized by eruptions of eczematoid nature, localized on the ears, bright pink seborrhea formations on the dermal surface of the head. Gradually the scales from the formations are separated layer by layer, which leads to the formation of deep ulcers, and subsequently provokes the emergence of infectious lesions and septic processes.
In the photo, a child with Abta-Letterter-Siwa syndrome with g , an astrocytosis
A similar pathological form is typical for children younger than 2 years of age, in which, before the usual disease, teeth begin to erupt, gingival tissue is seen.
Sometimes there are signs of pulmonary lesions - a strong, persistent cough, rapid breathing, pneumothorax symptoms such as chest and pulmonary pain, dyspnea, difficulty breathing.
Symptoms of central inflammation, enlargement of the spleen and / or liver, infectious and inflammatory lesions of the lymph nodes, anorexia and irritability may also occur. The signs such as thrombocytopenia and anemic disorders worsen the prognosis.
Hyunda-Schuller-Krischen Syndrome shows the following manifestations:
- Diabetes of a non-sugar form, accompanied by copious urinary excretion( up to 9 liters / day);
- An oedemas caused by infiltrative lesions of the eye muscles and optical nerve fibers;
- Bone lesions localized on flat bones like scapula, ribs, skull, pelvis.
This pathology is often complicated by otitis, tooth loss, disturbances in the fat and iron activity and the synthesis of sex hormones, pathologically high levels of prolactin hormone.
Hystiocytosis of the lungs
Pulmonary histiocytosis is often called granulomatosis, because pathology is characterized by the formation of granulomas. Hystiocytosis of the lung can occur in the primary chronic and acute form.
For the acute form is typical the formation of granulomatous and cystic lesions in the lung tissues, which leads to a noticeable increase in body volume. Acute pulmonary histiocytosis is characterized by rapid progression and spread of pathology to other systems like bones, skin, kidneys, the central nervous system, and is characterized by dry cough, hyperthermia, dyspnea.
Chronic form is characteristic of the formation of multiple emphysema-like swelling and nodular rashes on the pulmonary surface. With chronic histiocytosis of the lungs, patients have signs such as:
- Thoracic pain;
- Shortness of breath;
- Pathological weakness;
- Painful symptoms in costal, pelvic and cranial bones;
- Hormonal disorders with symptomatic diabetes diabetes insipidus;
- An exhausting, unproductive cough.
Pulmonary histiocytosis of the acute course is most typical for small children under 3 years of age, the chronic form is more typical for adults.
Sinus histiocytosis in the lymph nodes
Systic sinusis is a rare species of this pathology and has a benign character. It is characterized by the accumulation of pathogenic cells in the lymph node tissue, which makes the lymph nodes bigger.
This pathology can occur in two varieties:
- A typical disorder of lymph node cells;
- Localization in the cervical lymph nodes with a characteristic increase.
Sometimes, along with the lymph nodes, the lesion covers the skin, bone tissue, nasopharynx. Often, sinus histiocytosis is accompanied by cancerous pathologies of the blood. For a typical disease, the presence of such symptoms:
- Hyperthermia;
- Increased volume of lymph nodes;
- Arthralgia and ossaly;
- Anemia Symptoms;
- The presence of neutrophilic leukocytosis;
- High rates of ESR.
Pathological processes are constantly exacerbated, accompanied by a lack of immune protection and plasma phagocytosis( when phagocytes capture and absorb plasma cells).To diagnose sinus histiocytosis, a lymph node biopsy and other laboratory tests are indicated.
Features of the disease in adults and children
All histiocytosis varieties are manifestations of a single pathological process, only the symptomatology and prognosis for them differ. Sometimes one type of disease passes into another.
In pediatric patients, histiocytosis lesion can develop in three forms:
- Langerkosletochnaya;
- Malignant;
- Syndrome of virus-associated hemophagocytosis.
According to statistical information, in children, histiocytosis is detected by a frequency of 1 case per 1 million people, and in adults it is less frequent. Moreover, boys are more likely to develop such an anomaly.
Lengergan cell cell histiocytosis in the form of Hend-Schüller-Krischen syndrome is characterized by a systemic character and delayed development. It is typical for the manifestation of granuloma-like bone, lymph node and intraorganic lesions.
For pathology in the late stages of progression, symptomatology is typical in the form:
- ;
- Cranial-bone granulomatous formations;
- A non-malignant version of diabetes;
- 30% of patients develop skin lesions, such as infiltrative lesions in the groin and armpit.
Malignant form of child histiocytosis almost always ends fatal, it affects such pathology of children under 3 years. In short time intervals, the disease causes massive systemic disorders and disorders, spreading through bone tissues and internal organs.
Malignant histiocytosis forms are characterized by the presence of skin lesions such as the formation of brownish-brown papules, erythema-like eruptions, often degenerating into deep ulceration.
Diagnostics
Diagnostic laboratory and instrumental studies are based on the biopsy of various tissue samples to detect langergant cells. With pulmonary histiocytosis, additional chest X-ray examination, bronchoscopic analysis, and high-frequency computed tomography are necessary.
The photo CT of the thoracic cavity, which shows the granuloma of the eosinophilic form of Langerhans' histiocytosis
Histiocytosis processes often have symptoms similar to other pathologies, therefore they need to be differentiated from purulent processes in bone and bone marrow, tuberculosis lesions, blood cancer and vascular autoimmune inflammations such as granulomatosis.
Treatment
Therapeutic measures are selected on the basis of diagnostic data determining the stage and form of the pathological process. If the pathology is characterized by a severe course, then the treatment of histiocytosis should be performed in a profile hematological hospital. Although medicine knows of such cases, the histiocytosis itself was resolved without the participation of doctors.
As for the patient-children, they must be hospitalized for the purpose of carrying out therapy with the use of cytostatic and hormonal drugs like Prednisolone and Leykeran.
The bulk of specialists are inclined to think that the most optimal treatment option will be cyclic reception of these medications - the entire course is 10 two-week cycles with a break between courses of 14 days. If children's histiocytosis is less aggressive, then symptomatic drugs like Decaris and Hypothiazide are prescribed.
For bone lesions of histiocytosis, ionizing irradiation procedures are indicated, after which a course of cytostatic and hormonal treatment is necessarily prescribed to prevent subsequent relapses. If radiation treatment is contraindicated or inconclusive, then they resort to a surgical intervention in combination with the administration of the hormone preparation of the glucocorticoid group into the bone.
If there is a lesion of the lung tissue, then the reception of drugs like Theophyllin, related to bronchodilators, is indicated. In the case of severe pulmonary lesion, removal of the organ is indicated. Adult patients in order to prevent relapse are recommended to permanently abandon cigarettes.
For skin lesions, the use of mono-chemotherapy with Vepesid is recommended. In addition, a good therapeutic effect is achieved in the treatment of sinus histiocytosis with the use of interferon drugs.
Forecast
In general, the prognostic data of histiocytosis are favorable. Though pathology is characterized by systemic and organic disorders, it can be treated in a therapeutic way.
Practice shows that most cases of histiocytosis( about 80%) result in complete cure of the patient. High mortality is characteristic only in the case of multiorgan lesion.
A video about pathomorphological features of lung histiocytosis from Langerhans cells: