Lymphogranulomatosis

Manifestations of Hodgkin’s disease

Diagnostics

Treatment of Hodgkin’s disease

Forecast

Lymphogranulomatosis is a disease of the lymphatic system. Lymphogranulomatosis occurs 3 times more often in families where such patients have already been registered, compared with families where they were not.

The causes of Hodgkin’s disease are not fully understood. Some experts believe that Hodgkin’s disease is associated with Epstein-Barr virus.  

Manifestations of Hodgkin’s disease

Symptoms of Hodgkin’s disease are very diverse. Beginning in the lymph nodes, the painful process can spread to almost all organs, accompanied by variously pronounced manifestations of intoxication (weakness, lethargy, drowsiness, headaches).

The predominant defeat of a particular organ or system determines the picture of the disease.

The first manifestation of Hodgkin’s lymphoma usually is an increase in lymph nodes; in 60-75% of cases, the process begins in the cervico-supraclavicular lymph nodes, somewhat more often on the right. As a rule, an increase in the lymph nodes is not accompanied by a violation of the patient’s state of health. Enlarged lymph nodes are mobile, not soldered to the skin, in rare cases painful. Gradually, sometimes rapidly increasing, they merge into large formations. Some patients have pain in enlarged lymph nodes after drinking.

In some patients, the disease begins with an increase in the lymph nodes of the mediastinum. This increase can be accidentally detected with fluorography or occur later, when the size of the formation is significant, cough, shortness of breath, less often – pain behind the sternum.

In rare cases, the disease begins with an isolated lesion of the near-aortic lymph nodes. The patient complains of pain in the lumbar region, occurring mainly at night.

Sometimes the disease begins acutely with fever, night sweats, rapid weight loss. Usually in these cases a slight enlargement of the lymph nodes appears later.

The most frequent localization of Hodgkin’s disease is lung tissue. Lesions of the lungs are usually not accompanied by external manifestations. Quite often, when lymphogranulomatosis is detected accumulation of fluid in the pleural cavities. As a rule, this is a sign of a specific lesion of the pleura, sometimes visible during X-ray examination.

The defeat of the pleura occurs usually in patients with lymphogranulomatosis with enlarged lymph nodes of the mediastinum or with foci in the lung tissue. A tumor in the lymph nodes of the mediastinum can germinate in the heart, esophagus, trachea.

The bone system is as frequent as the lung tissue, the localization of the disease in all variants of the disease. Vertebrae are more often affected, then the sternum, pelvic bones, ribs, less often – tubular bones. Involvement of bone in the process is manifested by pain, radiological diagnosis is usually delayed. In isolated cases, damage to the bone (sternum) may become the first visible sign of Hodgkin’s disease.

Liver damage due to the large compensatory capacity of this organ is found late. There are no characteristic signs of specific liver damage.

The gastrointestinal tract, as a rule, suffers for the second time due to compression or germination of the tumor from the affected lymph nodes. However, in some cases, lymphogranulomatous lesions of the stomach and small intestine. The process usually affects the submucosal layer, an ulcer does not form.

Sometimes there are lesions of the central nervous system, mainly the spinal cord, they give serious neurological disorders.

Very often with Hodgkin’s disease, various skin changes: scratching, allergic manifestations, dryness.

More or less sweating is noted by almost all patients. Heavy night sweats, forcing me to change my underwear, often accompany periods of fever and indicate a serious illness.

The skin itch happens approximately at a third of patients. Its severity is very different: from mild itching in areas of enlarged lymph nodes to widespread dermatitis with scratching all over the body. Such an itch is very painful for the patient, deprives him of sleep, appetite, leads to mental disorders. Finally, weight loss accompanies severe exacerbations and terminal stages of the disease.

Diagnostics

Even with a fairly convincing clinical picture, only a histological examination detecting lymphogranuloma allows you to finally confirm the diagnosis. The morphological diagnosis can be considered reliable only if there are Berezovsky-Sternberg cells in the histological variant.

Histological analysis not only confirms and establishes the disease, but also determines its morphological variant. The morphological diagnosis of Hodgkin’s disease is considered unquestionable if it is confirmed by three morphologists. Sometimes obtaining material for histological examination is difficult due to the location of lesions in the lymph nodes of the mediastinum or retroperitoneal space.

To diagnose the disease that caused an increase in only the lymph nodes of the mediastinum, a diagnostic opening of the chest cavity is used.

Localization of lymphogranulomatosis only in the retroperitoneal nodes is extremely rare, but in such cases histological confirmation of the diagnosis is required, that is, a diagnostic opening of the abdominal cavity is shown.

Involvement of the lymph nodes of the mediastinum, the roots of the lungs, lung tissue, pleura, and bones into the process is detected by X-ray studies, including computed tomography. Lymphography is used to study paraaortic lymph nodes.

The method of scanning retroperitoneal lymph nodes is not accurate enough (the percentage of false-positive and false-negative responses reaches 30-35%). The best method is direct contrast lymphography (method error 17-30%). Specifying the stage of the disease is made by additional methods of research, which include:

  • medical examination
  • chest x-ray
  • bone marrow percutaneous biopsy
  • liver, spleen and radionuclide scans
  • contrast angiography

Treatment of Hodgkin’s disease

Modern methods of treatment of Hodgkin’s disease are based on the concept of the curability of this disease. While lymphogranulomatosis remains a local lesion of several groups of lymph nodes (stage 1-2), it can be cured by radiation. The results of long-term use of polychemotherapy up to the limit of tolerance of healthy tissues suggest a cure even with a common process.

Radical radiation therapy, that is, radiation therapy at the onset of the disease in doses of 35-45 Gy per focus for sufficient areas (wide fields, including all groups of lymph nodes and outflow paths), with a sufficiently high beam energy ( megavolt therapy), can completely cure 90 % of patients with limited forms of the disease. The exceptions are patients with stage 1-2, in which the lymph nodes of the mediastinum are more than 1/3 the diameter of the chest. These patients should receive additional chemotherapy.

Chemotherapy is prescribed at the time of diagnosis. Also use radiation therapy. Many hematologists believe that it is necessary to combine chemo and radiation therapy.

Proper treatment of the first stage can lead to full recovery. Chemotherapy and irradiation of all groups of lymph nodes are very toxic. Patients are difficult to tolerate treatment due to frequent adverse reactions, including nausea and vomiting,   hypothyroidism , sterility, secondary lesions of the bone marrow, including acute leukemia.

Treatment regimens for Hodgkin’s disease

  • MOPP – Mustagen , Oncovir ( Vincristine ), Procarbazine , Prednisone. Apply at least for 6 cycles plus 2 additional cycles after achieving complete remission.
  • ABC – adriamycin ( doxrubicin ), bleomycin , vinblastine , dacarbazine . This scheme is highly effective in patients with relapses. In combination chemotherapy, the ABCD regimen is often used.
  • MUPP (similar to the MOPP scheme, oncovin is replaced by vinblastine at a dose of 6 mg / m2).
  • If the therapy is not effective or within a year after achieving remission, a relapse occurs, then the patient is given a more powerful therapy – DexaBEAM : where Deha is dexamethasone , B is BCNU, E is phasic , A is aracene ( cytosar ), M is melfolan . Conduct 2 courses. If the effect is obtained, then bone marrow or blood stem cells are taken and auto-transplantation is done to such a patient. Otherwise, there is a bad outcome.

Forecast

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The greatest value in the prognosis for lymphogranulomatosis is the stage of the disease. In patients with stage 4 disease, 75% five-year survival is observed, in patients with stage 1-2, 95%. Prognostic signs of intoxication are bad. Early signs of an unfavorable course of the disease are “biological” indicators of activity.The biological indicators of activity include:  

  • increase in total blood count ESR more than 30 mm / h
  • boost   fibrinogen concentrations more than 5 g / l,
  • alpha-2-globulin more than 10g / l,
  • haptoglobin more than 1.5 mg%,
  • cerruloplasmin more than 0.4 units of extinction.

If at least 2 of these 5 indicators exceed the specified levels, then the biological activity of the process is ascertained.

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