The histological diagnosis of HD is based on an excisional or incisional biopsy of a lymph node or more rarely of an extranodal site. Needle aspiration of lymph nodes is inadequate for initial diagnosis because lymph node architecture is essential for histological classification. Accurate staging of HD distinguishes patients requiring radiation therapy alone from those requiring chemotherapy or multimodality therapy.
The Ann Arbor system (Table 4.1), established in 1970, defines the basic parameters for patient staging. Contiguous involvement of adjacent organs is not considered dissemination. These cases are staged based on the extent of lymph node involvement (stages I, II, or III) followed by the subscript E, for direct extension. Splenic involvement is denoted by the subscript S. Patients are also classified as A or B based on constitutional symptoms. The B classification includes temperature > 38°C for three consecutive days, night sweats and 10% loss of body weight during the previous six months.
The majority of patients diagnosed with HD have radiographic evidence of intrathoracic involvement. Therefore, anterior-posterior and lateral chest x-rays should be ordered on all patients. Mediastinal or hilar adenopathy should be evaluated with computed tomography (CT) of the chest.
Intra-abdominal staging of HD is more difficult. CT scanning, ultrasound, lymphography, magnetic resonance imaging (MRI) and gallium scanning have all been utilized for abdominal staging. Bipedal lymphangiography (LAG) provides information about lymph node architecture and may be superior to CT scanning when evaluating patients with HD with inguinal or femoral adenopathy. On the
Table 4.1. Ann Arbor staging system
Stage I Involvement of a single lymph node region (I) or a single extralymphatic organ or site (IE)
Stage II Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of an extralymphatic organ or site (IIE)
■■ Stage III Involvement of lymph node regions on both sides of the diaphragm (III) or B localized involvement of an extralymphatic organ or site (IIIE) or spleen
® Stage IV Diffuse or disseminated involvement of one or more extralymphatic organs with or without associated lymph node involvement other hand, CT scanning is superior when evaluating celiac, splenic, porta hepatic and splenic foci. LAG and CT scanning are complementary techniques for staging, but few institutions perform LAG. Most centers rely on CT scanning for in-tra-abdominal staging since it is faster and technically easier.
Bone marrow biopsy is also a part of the initial staging procedure. However, the marrow is rarely involved (less than 1%) in stages 1A or 2A. The bone marrow biopsy is particularly important when evaluating patients with bone lesions, bone pain, elevated serum alkaline phosphatase, and clinical documentation of stages 3A or 4A.
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