Withчины развития лимфогранулематоза, симптомыdiseases, treatment methods, prognosis

Update: October 2018 Lymphogranulomatosis is hard
cancer characterized by pathological
the growth of lymphoid tissue and the appearance of specific
cells of Berezovsky-Sternberg (structural elements of granulomas).
This article will tell you about lymphogranulomatosis, symptoms, treatment,
causes of this pathology. Lymphogranulomatosis, or
Hodgkin’s disease affects people all equally
age groups. In the structure of morbidity among young people
(15 – 30 years) the proportion of the male and female population is
50% of confirmed cases. Second peak incidence
is observed among older persons (over 45), among
which about 70% of patients are men.

According to researchers of the American Cancer
Community, in 2014 in the world will be diagnosed 9190 new
cases of Hodgkin’s disease (4120 in women and 5070 in men). AT
this year, 1,180 people will die from this disease (510 women
and 670 men).

Withчины возникновения

The etiological factors of the disease are not reliably identified, but
there is evidence of increased incidence among residents
certain regions, which allows to draw conclusions about the impact
viruses and environmental factors on the incidence rate.

Among the predisposing factors emit:

  • Hereditary predisposition (the presence of relatives with
    lymphoid tissue disease);
  • ATрожденный и приобретенный иммунодефицит;
  • The presence of autoimmune diseases (see lupus erythematosus:
    symptoms, treatment, rheumatoid arthritis: symptoms and treatment).

There is a close relationship of infectious mononucleosis caused by
Epstein-Barr virus, with the development of Hodgkin’s disease. None
However, this disease does not occur in every carrier.
virus.

According to research, the isolated effect of this factor
determines the likelihood of lymphogranulomatosis just
in 0.1%. With этом вирус Эпштейна-Барра выявляется у 90% населения
Of the earth. AT группе повышенного риска находятся также люди с различными
immunodeficiency states.

Classification

Study of foci of pathological growth in granulomatosis
allowed to identify 4 histological types of the disease:

  1. Granulomatosis with a predominance of lymphoid tissue (5-10%)
    manifested as limited or common
    uncontrolled growth of lymphocytes (in some cases along with
    histiocytes). This type determines the most favorable course and
    develops without hardening and necrosis of the tumor focus;
  2. Nodular sclerosis is the most common form.
    diseases (30-60%) and is manifested by the growth of connective
    fabric in the form of knots. The phase of fibrosis is preceded by the growth of immature
    and formed lymphoid cells, while the course of the disease
    characterized by gradual slow progression;
  3. Mixed cell granulomatosis type (20-40%) is different
    unfavorable course and manifests multiple foci
    fibrosis, often undergoing necrosis. Pathological growth
    lymphoid tissue manifests polymorphic cell proliferation,
    including lymphocytes, neutrophils, eosinophils, and specific cells
    Berezovsky-Sternberg. Due to mass cell death in foci
    fibrosis, this type has a very poor prognosis;
  4. With резком сокращении количества лимфоцитов или их полном
    Disappearance type of Hodgkin’s disease is characterized as lymphoid
    depletion (less than 5% of cases). This type has two subtypes: diffuse
    sclerosis, in which in the structure of pathological foci of fibrosis
    there may be patches of Hodgkin cells or
    Berezovsky-Sternberg and reticular type, characterized by
    pathological giant single-and multi-core cells. Any
    of the two subtypes suggests severe disease and has
    extremely unfavorable prognosis.

The clinical picture of the disease

The first symptoms that the patient notices are
enlarged lymph nodes. The onset of the disease is characterized by
the appearance of increased dense formations under the skin. They
painless to the touch and can occasionally decrease in size, but
subsequently increase again. Significant increase and
soreness in lymph nodes occurs after consuming
of alcohol. AT некоторых случаях возможно увеличение нескольких групп
regional lymph nodes: Лимфогранулематоз у детей симптомы

  • Cervical and supraclavicular – 60-80% of cases;
  • Mediastinal lymph nodes – 50%.

Along with the local symptoms of the patient, the general
manifestations (symptoms of group B):

  • Fever persisting for more than one week;
  • Excessive sweating at night (see the causes of increased
    sweating in women and men);
  • Uncontrolled weight loss (more than 10% of body weight for 6
    months)

Symptoms “B” characterize a more severe course of the disease and
allow you to determine the need for the appointment of intensive
therapy.

Among other symptoms characteristic of Hodgkin’s disease,
allocate:

  • Weakness, loss of strength, loss of appetite
  • Pruritus
  • Cough, pain in chest breathing
  • Abdominal pain, indigestion
  • Ascites
  • Bone pain

AT ряде случаев единственным симптомом лимфогранулематоза в
for a long time is only a constant feeling
fatigue.

Breathing problems occur with increasing intrathoracic
lymph nodes. As the nodes grow, they gradually squeeze
trachea and cause persistent coughing and other breathing problems.
These symptoms are aggravated in the supine position. AT ряде случаев
Patients report pain in the sternum.

Clinical manifestations of granulomatosis are increasing gradually and
take place in 4 stages (depending on the prevalence of the process and
severity of symptoms).

  1. The first stage is characterized by limited growth.
    lymphoid tissue (within a regional node, thymus, or
    spleen). Clinical manifestations at this stage are very rare and
    may be characterized by swollen lymph nodes without developing
    pain or discomfort;
  2. The criterion for the transition of the disease to the second stage is the involvement
    two or more lymph nodes or a non-lymphoid organ. AT данном
    case, the clinical manifestations are general in form
    gradually increasing weakness, loss of strength, loss of appetite and
    losing weight;
  3. In the third stage of Hodgkin’s disease, pathological changes
    affect the distant lymph nodes and are accompanied
    compression of surrounding tissues with the appearance of pain. For
    This stage is characterized by an increase in the size of the glandular organs.
    (spleen, endocrine glands). Third stage of the disease
    characterized by severe clinical manifestations in the form
    persistent prolonged fever, abdominal pain. Decreased immunity and
    the spread of inflammatory changes in the digestive tract leads to
    indigestion, depletion of the body;
  4. The fourth stage is terminal. AT этот период изменения
    acquire a diffuse character. Pathological foci for this
    time develops in the liver, causing ascites and itching, bone
    brain (pain in the bones), lungs (difficulty breathing, pain behind
    sternum) and cerebrospinal fluid (pain in the lower back, limbs,
    онемение, чрезмерно высокая by thatпература).

Diagnostics – analyzes, biopsy, tomography

Diagnosis of the disease is based on general clinical
analyzes and biochemical studies to assess liver function and
spleen, as well as instrumental methods of diagnosis, including
x-ray of the chest, computer and
magnetic resonance imaging.

What changes can be with lymphogranulomatosis in the analysis
blood? Only with severe symptoms and manifestations of the disease.
observed:

  • Thrombocytopenia
  • Anemia
  • ESR is increased and pancytopenia is detected in the later stages.
    Also monocytosis, neutrophilic leukocytosis,
    eosinophilia.
  • Sometimes cells are found in leucoconcentrate.
    Berezovsky-Sternberg
  • Immunological analysis – a violation of immune reactions
    замедленного типа, угнетение выработки AT-лимфоцитов на поздних
    stages.

As for the urine analysis, it is usually normal, in the case of
renal damage can be detected by red blood cells and protein.

AT биохимическом анализе крови:

  • With поражении печени — увеличивается билирубин, уровень
    enzymes
  • May increase seromukoidy, haptoglobin, globulins

X-ray examination allows to determine the presence of
enlarged cervical and hilar lymph nodes. Considering that when
lymphogranulomatosis these nodes are affected primarily by x-rays
may be informative for early diagnosis of the disease.

A more accurate picture allows you to get a tomographic
study. Computed tomography – affordable and reliable
tool for accurate diagnosis of pathology and establishment
stage of the process. CT scan can detect lymphomas in any department or
body (thoracic, abdominal and small
pelvis, brain).

MRI has similar results as CT, but more accurately
determine the size and location of the tumor. Due to the absence
harmful effects, this study may be useful when
determining the effectiveness of treatment in dynamics.

The final diagnostic method is a tissue biopsy from
lymph nodes for histological examination. In addition, for
establish the stage of Hodgkin’s disease and severity
diseases may need a bone marrow biopsy.

Analysis of bone marrow punctate – unchanged at an early stage
diseases. With генерализованной форме — задержка созревания
cells of an erythroid row, lymphogranulomatous foci, small
количество клеток Berezovsky-Sternbergна, склонность к
hardening.

Lymph node biopsy – detection of specific cells
polymorphic cell granuloma.

How is lymphogranulomatosis treated today?

Therapeutic measures for lymphogranulomatosis are planned in
зависимости от стадии diseases.

With выявлении болезни на ранних стадиях (1 или 2) могут
apply the following treatment principles:

  • For взрослых рекомендуется проведение лучевой therapy. Her
    application allows to achieve stable remission, although along with by that
    there are a number of serious complications;
  • Children at an early stage are given a combination treatment.
    suggesting the use of chemotherapy and low-dose radiation
    therapy. The principle of low-dose radiation therapy was developed
    specifically to prevent infertility, leukemia and
    toxic effects exerted by medicinal drugs and
    манипуляциями на дыхательную и сердечно-сосудистую сисby thatы.

Standard treatment for all patients with third stage
Hodgkin’s disease is radiotherapy and chemotherapy. On the fourth
stage of the disease, experts recommend chemotherapy.
The latest developments in the field of chemotherapy provide
positive prognosis of Hodgkin’s disease and five-year survival
about 90% of patients.

Relapse after treatment occurs in 20-35% of patients. With
The development of relapse is repeated radiation and chemotherapy with
subsequent transplantation of bone marrow cells. Usually such treatment
leads to stable remission, although repeated relapses are all
are possible.

Radiation therapy helps stop growth and decrease
the size of the tumor. This method is mainly used for
treatment of adult patients, as it has many severe
side effects.

According to the latest recommendations, extended beam
therapies involving healthy tissue surrounding
pathological lymph node has no significant advantage over
limited exposure and unreasonable risk
тяжелых side effects. In this regard, the leading modern
specialists adhere to the principle of point effects on
выявленный патологический очаг после проведения химиоtherapy.

Withменение химиотерапии означает прием специальных лекарственных
means causing the death of cancer cells. These drugs are called
цитотоксичными и обладают сисby thatным действием, так как воздействуют
on the whole body, not just on the tumor.

The volume of chemotherapy is planned based on the stage of the disease, and
includes 4 to 8 courses. One course is 28 days.

The standard scheme of chemotherapy for lymphogranulomatosis includes
ABVD or Stanford V. ABVD is an abbreviation for the names
used drugs: Adriamycin, Bleomycin, Vinblastine and
Dacarbazine.

With назначении схемы Stanford V предполагается использование
combinations of 7 drugs: Adriamycin, Mechlorethamine, Vincristine,
Vinblastine, Bleomycin, Etoposide, Prednisolone. This principle
treatment of Hodgkin’s disease is applied to patients with high
risk of malignant disease. This is the maximum
effective late-stage method with confirmed positive
effect in 95% of cases. Наряду с by that, данный метод опасен высокой
likelihood of developing secondary cancers. So sick
long-term prophylactic surveillance is assigned to identify
отдаленных последствий химиоtherapy.

Transplantation

Patients with recurrent or progressive lymphogranulomatosis
Repeated chemotherapy may be prescribed, followed by
bone marrow transplant. This means that bone marrow cells
the patient will be replaced by healthy ones. So provided
normalization of bone marrow work after toxic exposure
therapeutic drugs. The patient’s bone marrow can be replaced by his
same own or donor cells. The second option is associated with
increased risk of transplant rejection and is only used
in case of failure of the own material transplant.

Forecast

Lymphogranulomatosis is a form of cancer that responds well to
treatment, especially in the early stages. Unlike other cancers
diseases, even in the later stages of patient survival rates
stays high enough.

Five-year survival in patients is on average 60-80%,
at the first and second stage is 90-95%, at stage 3 about
70%. According to studies for patients who have lived for more than 15 years
after treatment for Hodgkin’s disease,
the chance of dying from the complications of the underlying disease is much
less than any other impact. With химиотерапии на 3
and 4 stages of remission is achieved in 70 percent of cases.

Forecast является неблагоприятным, когда:

  • Within 1 year after the treatment occurs
    relapse;
  • There is no improvement from the treatment, and the disease
    is progressing.

The prognosis is influenced by the following factors:

  • Gender – men have a higher risk of severe prognosis;
  • Пожилой возраст – чем старше пациент, by that хуже прогноз (45 лет
    and older);
  • Stage 4 disease;
  • Low albumin and hemoglobin in the blood;
  • High white blood cell count (more than 15 x
    109/л);
  • Low lymphocyte count (less than 600).

The more facts from the above list are noted in
конкретного пациента, by that больше вероятность того, что ему
need more serious medical support.

Автор: Коробов Никита Александрович врач-анестезиолог

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