Hodgkin’s Disease: Etiology, Clinical Manifestations, Diagnosis & Management

Hodgkin’s disease is a malignant disorder of lymph nodes first described by Thomas Hodgkin in 1832. It is characterized by proliferation of abnormal giant, multinucleate cells called Reed-Sternberg cells which are located in lymph nodes. It is a relatively rare malignancy that has an impressive cure rate. Unlike other lymphomas, Hodgkin’s disease is unicentric in origin in that it initiates in a single node. The disease spreads by contagions extension along the lymphatic system.

Etiology of Hodgkin’s Disease

The etiology of the Hodgkin’s disease is unknown, but there may be several factors that are thought to play a role in its development. The main interactive factors include infection with Epstein Barr Virus (EBV), genetic predisposition and exposure to occupational toxins. The 1st peak incidence in 30-40 years. The second peak incidence occurs at 55 to 77 years.

Clinical manifestations of Hodgkin’s Disease

Hodgkin’s disease usually begins as a painless enlargement of one or more lymph nodes on the side of the neck. Enlarged lymph nodes most commonly are found in the supraclavicular, cervical and mediastinal regions. Local manifestations produced by lymphadenopathy usually are caused by pressure or obstruction. Involvement of the extremities can be manifested by pain, nerve irritation, and obliteration of the pulse. Patient’s may experience a nonproductive cough, with the chest radiograph revealing a mediastinal mass, which is present in about 50% of clients.

Pericardial involvement can occur by direct invasion from mediastinal lymph nodes. The involvement can cause pericardial friction rub, pericardial effusion, and engorgement of neck veins. Other manifestations arise when lymph nodes obstruct or compress an adjacent structure (e.g. edema of the face, neck and right arm secondary to superior vena cava compression or renal failure according to urethral obstruction).

If the tumor infiltrates the spine and presses on the spinal cord, manifestations of spinal cord compression can develop. Manifestations range from early back pain with motor weakness and sensory loss to loss of motor function, urinary retention, constipation, and other manifestations of compression of the cord late in the disease.

Associate clinical manifestations of unexplained weight loss of more than 10% of body weight in 6 months, frequent drenching night sweats, and fever above 38 degree Celsius also may be present. Pruritus is a systemic manifestation that can be significant if it is recurrent. These additional manifestations are known as B symptoms for staging purposes, they occur in greater frequency in older clients and are negatively related to the prognosis.

The patient may notice weight loss, fatigue weakness, fever, chills, tachycardia, or night sweats. After the ingestion of small amount of alcohol individuals with Hodgkin’s disease may complain of a rapid onset of pain at the site of disease. The cause for the alcohol-induced pain is not known.

In more advanced disease, there is hepatomegaly, and splenomegaly and decreased the production of erythrocytes. Other physical signs vary depending on where the disease has spread for e.g. intrathoracic involvement leads to superior vena cava syndrome enlarged peritoneal nodes may cause palpable abdominal masses or interfere with renal function jaundice may occurs from liver involvement and spinal and compresses lead to paraplegia may occur with extradural involvement. Bone pain occurs as a result of bone involvement.

Diagnosis of Hodgkin’s Disease

  • The diagnosis is confirmed by lymph nodes and bone marrow biopsy.
  • The diagnosis is confirmed by a chest radiograph to evaluated complaints of a persistent cough or dyspnea may identify mediastinal involvement.
  • The extent of disease is determined by CT-Scan of the thoracic, abdominal, and pelvic areas as well as gallion scan of mediastinal or hilar lymph nodes and lymphangiography of the lower extremities. If the extent of the disease cannot be determined by these diagnostic tests and confirmation of abdominal disease is necessary for determining treatment choice, a staging laparotomy may be performed.

Staging of Hodgkin’s Disease

Hodgkin’s disease is divided into categories, or stages according to the microscopic appearance of the involved lymph nodes, the extent and severity of the disorder and the prognosis. Accurate staging of Hodgkin’s disease is important for determining treatment option.

  • Stage I: involvement of a single lymph node or a single extranodal site.
  • Stage II: involvement of two or more lymph nodes regions on the same site of the diaphragm or localized lymph node regions of the same side of the diaphragm.
  • Stage III: involvement of lymph nodes regions on both sides of the diaphragm may include a single extranodal site, the spleen, or other, now sub-divided into the lymphatic involvement of the upper abdomen in the spleen (ophiceliac and portal nodes).
  • Stage IV: a diffuse or disseminated disease of one or more extra lymphatic organs or tissues with or without associated lymph node involvement; the extranodal site is identified as H- hepatic, L-lung, P-pleura, M-marrow, D-dermal and O-osseous.

Management of Hodgkin’s Disease

Peripheral blood analysis, lymph nodes biopsy, bone marrow examination and radiological evaluation are important measures of evaluating Hodgkin’s disease using  all the information form the various diagnostic studies, treatment decisions made on the basis of stages of disease as follows:

Stage Recommended therapy
I, II (A or B) Radiation
I, II (A or B with mediastinal mass 7-1/3 diameter of a chest) Combination chemotherapy followed by radiation to involved field
III A (minimal abdominal disease) Combination of chemotherapy with radiation to involved sites
IIIB Combination therapy
IV (A or B) Combination therapy

Eradication of tumor cells

  • Radiation: radiation treatment for Hodgkin’ disease involves three location; the mantle, the para-aortic region, and the pelvis
  • Chemotherapy: chemotherapy has become the primary treatment strategy with or without radiation therapy in stage I or II as with poor prognostic indicators and  in clients with advanced Hodgkin’s disease

Complication

The complications related to Hodgkin’s disease are numerous because they are a result of the disease itself or of radiation therapy, chemotherapy, or a combination of several of these factors.

  • Thyroid dysfunction
  • Thymic hyperplasia
  • Hypothyroidism
  • Thyroid cancer
  • Pericarditis
  • Dental caries
  • Cardiac dysfunction
  • Cardiomegaly
  • Pulmonary dysfunction
  • Pneumonia
  • Non-Hodgkin’s disease
  • Solid Tumor

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