It is a disease characterized by the gradual enlargement of the lymph nodes. It starts from a single focus and grows by spreading to neighboring glands around it. The reason is unknown. Its frequency varies between 1-10/100,000. The most common ages are between 15-35 and over 50. It is 3 times more common in boys than girls. It occurs more frequently in consanguineous marriages and in the children of people with HH. It is more common with systemic lupus erythematosus, rheumatoid arthritis, ataxia-telengiectasia and Swiss type agammaglobulinemia. It is known to be related to Ebstein-Barr virus.
Pathology
HH spreads from one lymph node chain to another through neighborhood. When the lymph node on the left collarbone is affected, enlargement occurs in the paraaortic lymph nodes in the abdomen, and when the lymph node on the right collarbone is affected, enlargement occurs in the mediastinal lymph nodes. When the paraaortic lymph node is involved, spleen, liver and bone marrow involvement also occurs. While nodular sclerosing type HH most commonly spreads through the neighborhood, mixed cell and lymphocyte-poor type HH spreads through non-neighborhood ways.
According to the Real Classification, HH is divided into the following types;
- in which lymphocytes are at the forefront. , nodular (diffuse or non-diffuse)
- classic HH
- lymphocyte-rich classic disease
- nodular sclerosis
- mixed cell
- lymphocyte-poor
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With the application of modern treatment protocols, the prognostic difference between HH types is gradually losing its importance.
Familial HH
The concentration of HH cases in certain families or certain races is a genetic predisposition or the same increases the likelihood of exposure to the causative agent. As a result of the examination of HH families, no mutation was found in the sex chromosomes. However, as a result of examination of affected families, some common HLA antigens were found. Many publications have reported co-occurrence of the disease in first-degree relatives (including children) and parent-child pairs. In families with identical twins, the risk of having HH in first-degree relatives has been found to be three-fold increased and in children it is seven-fold increased.
Clinical features
1-Lymphadenopathy (in 90% of cases)
Painless swelling of one or more superficial lymph nodes (rarely, pain may occur). In 60-80% of the cases, the lymph nodes in the neck enlarge, and in 60% of the cases, the lymph nodes in the chest cavity (mediastinal) also enlarge. Lymph nodes in the armpit, groin and behind the peritoneum may also be frequently involved. The retained glands generally have a well-distinguishable elastic and rubbery consistency. Sensitivity is rare. When mediastinal lymph nodes are involved, persistent dry cough and superior vena cava syndrome (enlargement of neck veins, hoarseness, difficulty in breathing and swallowing) occur.
2-Splenomegaly (enlargement of the spleen)
An enlarged spleen is often detected during physical examination, but an enlarged spleen does not necessarily mean that the spleen is involved. It is very important to understand whether the spleen is involved or not, and for this, the most sensitive FDG-PET examination should be performed. The spleen is most commonly kept in the lymphocyte-poor, and less frequently in the mixed-cell type.
3-Systemic findings (in 30% of cases)
Intermittent fever, loss of appetite, fatigue and weakness. , nausea, night sweats and weight loss. Mild itching is observed in 15-25% of cases. Itching is more common in advanced disease and is accompanied by other systemic symptoms. Sometimes it can be the first symptom of HD. As the disease heals, the itching disappears. Its recurrence should bring to mind the possibility that the disease has recurred. In less than 5% of cases, when alcohol is consumed, pain occurs starting from the chest and radiating to the arms, back and legs. The reason is unknown. It can be seen before the symptoms of the disease appear. It decreases and disappears with treatment. When the disease recurs, the pain may return before other symptoms appear.
4- Lung disease (in 17% of cases)
The disease may spread to the lungs from the mediastinal and hilar lymph nodes. In Hodgkin's disease, there is involvement of the chest cavity in 2/3 of the cases. Therefore, patients with mediastinal or hilar lymphadenopathy should be examined with lung tomography. Lung involvement is more common in nodular sclerosing type HH.
5-Neurological symptoms
Neurological findings are usually It occurs in the late stages of the disease. The disease metastasizes to the nervous system through nerves, blood vessels or lymphatic pathway from the lymph nodes around the spine or by hematological spread. In the midline nervous system, the disease is located on the dural membrane and causes compression findings related to the area it affects. When the disease spreads to the brain, signs of increased intracranial pressure, paralysis or sensory disturbances in half the body, focal convulsions, or sometimes edema at the bottom of the eye are observed. Neurological findings may sometimes occur without intracranial spread;
- metabolic disorders
- infections (herpez zoster, meningitis, and brain abscess)
- neurotoxicity due to chemotherapy
- radiotherapy.
6-Bone disease (in 2% of cases)
Typically manifests itself with bone pain. Radiological findings of bone involvement are variable, but its presence indicates an aggressive course, but the prognosis of patients with bone involvement has improved in the last 10 years. The most important complication seen in patients with spinal involvement is spinal cord compression as a result of compression fracture or tumor extension to the epidural region.
7-Bone marrow involvement (5% of all cases)
Hemoglobin, leukocyte count, Bone marrow involvement should be suspected in patients with low platelet count. Diagnosis should be made by examining bone marrow from more than one region because HH involves the bone marrow locally.
8-Liver disease
Spleen involvement is also present in almost all cases. Mild liver enlargement and deterioration in liver function tests do not usually indicate the extent of liver involvement. Therefore, a liver biopsy should be performed for a definitive diagnosis. When jaundice occurs, if it is due to HH, it is a sign that the disease is very advanced. However, HH is not the only cause of jaundice. Jaundice can also be seen in cases such as hemolytic anemia, viral hepatitis, toxic hepatitis or cholestasis.
9-Skin findings
It is characterized by specific skin lesions and paraneoplastic lesions. It is seen in 0.5-7.5% of cases. It manifests itself as red nodules and papules. When skin retention occurs, it is usually It is kept in the shadows and the prognosis is very poor. The lesions regress with treatment. Its recurrence is a sign that the disease has relapsed. Skin lesions occur with the release of substances called cytokines from tumor cells. Sometimes inflammatory nodules called erythema nodosum occur on the front surface of the legs. This finding regresses with chemotherapy. It may reoccur weeks or months before the disease relapses.
10-Kidney findings
In up to 13% of cases, HH may directly affect the kidney. A single kidney may be involved, or both kidneys may be flat and involved. Eclipse may be diffuse or focal. Renal dysfunction may also occur following ureteral obstruction or renal vein thrombosis. HH may cause glomerulonephritis. Although the minimal chenge type is the most common, all types of glomerulonephritis can be seen with HH. Nephrotic syndrome usually occurs during the course of the disease. Sometimes nephrotic syndrome may occur before the disease occurs. It responds to treatment and reoccurs during relapse.
11-Hematological findings
- bone marrow suppression occurs as a result of overactivity of the spleen (hypersplenism) or bone marrow retention.
- A positive Coombs test may occur with or without hemolysis. A positive Coombs test is seen with advanced disease and systemic symptoms.
- immune thrombocytopenia is observed in 1-2% of cases.
- Thrombotic thrombocytopenic purpura is also a described condition in HH.
- autoimmune neutropenia is a disease that can be seen in HD and may occur before the disease is diagnosed.
- An increase in cells called eosinophils in the blood may be observed in 15% of patients. It has been shown that interleukin 5, released from the cells that form HH, increases eosinophils.
12-Endocrine and metabolic findings
The most common metabolic disorder associated with HH is increased blood calcium level. This condition usually occurs in the advanced stages of the disease and is a poor prognosis indicator. The reason is the deterioration in vitamin D metabolism. It may occur during relapse and returns to normal with chemotherapy. Another metabolic disorder is lactic acidosis that occurs during HD. It disappears with chemotherapy. Inappropriate release of antidiuretic hormone is another metabolic abnormality that can be observed.
Laboratory findings
Anemia, increase in the number of neutrophils, increase in the number of eosinophils, decrease in the number of lymphocytes and bone marrow findings are hematological laboratory findings. Biochemical findings include increase in serum copper level, increase in serum ferritin level, decrease in transferrin levels, increase in erythrocyte sedimentation rate, increase in fibrinogen levels, increase in haptoglobulin levels, increase in alkaline phosphatase levels (bone disease), increase in serum soluble interleukin receptor level and beta 2 macroglobulin level.
Immunological features
A persistent suppression of T cell functions is observed in Hodgkin's patients. Defects in cellular immunity occur as a result of abnormal sensitivity to suppressive monocytes and suppressor T cells and impaired interleukin 2 production. This disorder in T cells depends on the cytokines released from Hodgkin cells. In addition to the cellular immune disorder, humoral immunity is also impaired. This is due to temporary deterioration in B lymphocyte functions following treatment. If the spleen needs to be surgically removed due to disease, patients should be vaccinated against pneumococcal and H. influenza B before this procedure. In advanced stage HH, lymphocytopenia occurs as a result of the decrease in T and B lymphocyte numbers. In order to detect these disorders, absolute lymphocyte count, T and B cell counts and T cell function studies should be performed in patients.
Staging
Abdominal and pelvic tomography are valuable methods to evaluate the spread of the disease, but lymph node They are inadequate to reveal the disease. Tomography can detect spleen involvement with only 19% sensitivity. FDG-PET tomography is the one that can best detect spleen involvement (92%). MR imaging has limited use. It is used to distinguish suspected intestinal lymph nodes in tomography. Gallium 67 scintigraphy should be evaluated carefully because it gives accurate results in 40% of patients with subdiaphragmatic disease. Laparotomic staging requires a major surgical intervention and can cause complications such as floor infection, intra-abdominal hematoma and abscess development, pancreatitis and lung problems.
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