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Lymphogranulomatosis: causes, symptoms and treatment, stages of the disease

Malignant granuloma or lymphogranulomatosis (LGM) is included in an extensive group of diseases affecting human lymphatic tissue. It is characterized by malignant lymphoid hyperplasia, which causes the formation of lymphorganulam in the internal organs and lymph nodes.

What it is?

At its core, lymphogranulomatosis disease is the primary lesion of the immune system of the malignant tumor process.

The disease was first described at the beginning of the eighteenth century (1832) by a British physician, Thomas Hodgkin. More recently, until the beginning of the 20th century (2001), it was called Hodgkin's disease or lymphoma. The disease affects men of young, middle age (between 15-35 years old) and those who have crossed the fifty-year limit. Children and women rarely get sick.

A distinctive feature of the malignant granuloma is a specific morphological picture with the presence of huge dual-core cells in the site (Berezovsky-Reed-Sternberg).

Lymphogranulomatosis is able to spread in the body in various ways - adjacent to the lymph and metastasizing with blood, to places with extensive capillary development (bone and lung tissue, liver). Dual-core cells are unique - they are able to move both inside and outside the vessel, forming new daughter tumor nodes in places susceptible to them.

Content

Causes of Hodgkin's disease - a theory of development

Lymphogranulomatosis

There are several theories of the development of LGM. Conditioned:

  1. Viral nature - nearly a quarter of the dual-core tumor cells have detected the genetic components of the Epstein-Barr virus, which has the ability to limit the proliferation (division) of immunological lymphoid cells.
  2. Genetic predisposition - in the presence of a family history of lymphogranuloma, or congenital or acquired immunodeficiencies (autoimmune diseases).
  3. Immunological reason based on the transfer of key cells of the immune system from the mother to the fetus.

Predisposing factors that give rise to the development of Hodgkin's disease can be:

  • radiological and ionizing radiation;
  • chemicals that enter the body through inhalation and with food;
  • drugs used to treat joint pathologies, some antibiotics, etc .;
  • viral and infectious diseases;
  • stress caused by childbirth or abortion;
  • surgery.

The onset of the tumor process is characterized by the formation of individual small nodules within the lymph node. Gradually expanding, they displace normal lymphoid nodular tissue, erasing it "to no." The development of a polymorphoncellular tumor (granuloma) begins, which may have a different cellular composition. With substrate formed by:

  • lymphocytes and prolymphocytes;
  • small lymphoid multi-chain reticular cells;
  • granulocyte leukocytes (neutrophils and eosinophils);
  • plasma cells or fibrous connective tissue.

Stages and forms of LGM

The stages and forms of a malignant granuloma are determined by the extent of the process. Which are expressed:

  • local form of destruction - stage one. The lymph nodes are affected on one side of the pectoral obstruction (diaphragmatic area), or in its adjacent areas;
  • limited regional - stage two. Several groups of nodes on one side of the diaphragm are affected;
  • common generalized - the third stage with the processes of nodal lesions on both sides of the pectoral obstruction;
  • the dissimiated form is the fourth stage. It is characterized by a lesion of any organ, without affecting the spleen and lymph nodes.

Each of the stages is divided into subgroups.

  1. (A) - due to the absence of intoxication from toxic products of tumor metabolism.
  2. (B) - intoxication with tumor decay products is present.
  3. (E) - metastasis of a tumor mass from a lymph node to adjacent organs and tissues.

Symptoms of intoxication are expressed - febrile temperature (38 ° C) with short periods of decline, sweating at night, unexplained weight loss (10% or more for the half year).

Symptoms of Hodgkin's disease, photo

Symptoms of Hodgkin's disease

Symptoms of Hodgkin's disease

Manifesting symptoms of Hodgkin's disease, depending on the predominant local lesion, reflecting the most diverse clinical picture.

До 70% случаев заболевание начинается с поражения шейных узлов. 1) Up to 70% of cases, the disease begins with a lesion of the cervical nodes. The process develops mainly on the right side. The affected lymph nodes increase in size (from the pea to the size of a large apple). You can easily notice the deformation of the neck.

At the early stage of Hodgkin's disease, the affected node is not painful, elastic, well mobile, the skin over it does not change. This condition can last more than six months. The development of the disease contributes to the spread of the process to other individual lymph nodes or a whole group of them.

Второе место по частоте заболеваемости занимают патологические процессы в узлах средостения и паховой зоны. 2) The second place in the incidence rate is occupied by pathological processes in the nodes of the mediastinum and inguinal zone. Lymphogranulomatosis in the nodes of the mediastinum is manifested by acute onset:

  • with coughing spells;
  • chest pain and shortness of breath;
  • increased sweating at night;
  • leukocyte decline in blood and the development of anemia;
  • quick weight loss.

With this variant of the disease, the prognosis of life is disappointing, and is determined by a short interval. The development of the process is complicated by damage to the lymph nodes throughout the body.

Symptoms of inguinal lymphogranulomatosis are manifested by small erosive single or group formations (within one, one and a half weeks). The temperature rises, muscle and joint pain, nausea and vomiting appear. Erosion is not accompanied by pain and often disappears on its own, which is the main danger of the initial stage of the lesion, postponing the timely start of treatment.

Therefore, patients seek medical help when the disease has entered the second stage involving the femoral and iliac nodes in the process. Soreness of the nodes is accompanied by temperature, they can open up on their own and exude a purulent substrate. Marked by:

  • enlargement of some internal organs;
  • at the peak of development (in a few years) trophic ulcers appear;
  • papillomatous neoplasms;
  • inflammatory reactions in the rectum and intestines;
  • possible hyperemia of the genitals;
  • narrowing of the urethra and end segment of the colon.

In metastasis of the granuloma, the lymph system of the spleen may be affected, forming nodes of various sizes or without any special changes. The regeneration process is characterized by damage to the liver, gastrointestinal tract and pulmonary nodes, with the formation of diffuse infiltrates and cavities in the lungs, resulting in tissue breakdown. Not infrequently, the tumor is localized in the pleura, provoking exudative pleurisy with the presence of dual-core cells in the fluid.

In a bone variant of Hodgkin's lymphoma, the granuloma is localized in the vertebrae, sternum, ribs, pelvic bones, and sometimes in tubular bones. As for the central nervous system - marked spinal lesion.

The skin variant of the pathology is manifested by excruciating itching on the skin in the area of ​​the enlarged site, scratching and dermatitis. Nodes acquire a dense-elastic structure, are soldered to one another and difficultly mobile, but not prone to suppuration.

The biological activity of the tumor process is reflected by the characteristic symptom of a blood test for lymphogranulomatosis — an increase in ESR, neutrophilic leukocytosis, absolute lymphocytopenia, high plasma fibrinogen values.

Lymphogranulomatosis in children

Almost 15% of all patients with lymphogranulomatosis occur in children. In children one year old the disease does not occur at all. Out of 100,000 patients, one pathology can occur in children under 6 years of age.

Raising the peak of Hodgkin's disease in children is noted at puberty (in adolescents). Boys are ill, mainly. The clinical course of LGM in children is no different from the manifestation of the disease in adults.

Diagnostic Examination Program

To identify a malignant granuloma today are used the most modern methods of laboratory and instrumental examination. Established:

  • on developed blood counts;
  • highly specific monitoring tests of the level of tumor markers;
  • PET research;
  • MRI of the peritoneal organs, chest and neck;
  • radiography;
  • Ultrasound of the lymph nodes of the peritoneum and pelvic region.

The morphological status of the tumor is detected by the method of punctuation of the lymph nodes, or by the method of complete removal of the node to identify dual-core large cells (Reed-Berezovsky-Sternberg). With the help of a bone marrow study (after a biopsy), differentiated diagnostics are performed, excluding other malignant neoplasms.

Perhaps the appointment of cytogenetic and molecular genetic tests.

Treatment of Hodgkin's disease, methods

Treatment of Hodgkin's disease

Based on the diagnostic results, an individual treatment plan is drawn up. Take into account the age characteristics of the patient and his condition at the moment.

The basis of the treatment of Hodgkin's disease are therapeutic techniques in combination with HLT (chemo and radiation therapy). Exact dosages of chemotherapeutic agents are calculated - intravenous or oral administration.

Chemotherapy sessions consist of 7-9 procedures (1 / three weeks), according to the chosen treatment plan.

Recurrent lymphogranulomatosis process is treated with high-dose chemotherapy methods. When the processes in the bone marrow, need a transplant of new immature cells. The most optimal treatment tactics of early stage Hodgkin's disease is radiation therapy.

Radiotherapy sessions for several weeks are held daily. In severe processes, intravenous steroid therapy is prescribed.

The result of treatment determines the control diagnosis. To prevent recurrence, the doctor prescribes a regular diagnosis.

LGM Forecast

The prognosis of Hodgkin's disease depends on the time of treatment, which provides patients with 85% to 95% five-year survival. In most patients, there comes a complete cure or steady state without relapse for over twenty years.

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