Staging

Historically, staging of HL has followed the Ann Arbor staging system29 (Table 72.3), although updated versions of this system incorporating a variety of prognostic factors exist (Table 72.4).30 Overall, 55% of patients present with localized disease (Ann Arbor stage I-II).

The staging work-up for HD has evolved. Historically, lymphangiography, CT imaging, bone marrow biopsy, and staging laparotomy have been extensively used. Staging laparotomy evolved in an era where treatment for limited-stage (Ann Arbor stage I and II) HL consisted primarily of extended radiation therapy fields, and precise pathological staging was required to include all areas of involvement. With the detection of occult splenic or high retroperitoneal disease at the time of laparotomy in 20-30% of patients with clinical stage IA-IIA disease and 35% of patients with clinical stage IB-IIB disease,3132 clinical staging alone was not accurate enough to predict which patients with early-stage HL were likely to attain a prolonged disease-free survival

Table 72.3 Ann Arbor staging system for Hodgkin's lymphoma29

Stage I Involvement of a single lymph node region or lymphoid structure, or involvement of a single extra-lymphatic site (IE)

Stage II Involvement of two or more lymph node regions on the same side of the diaphragm, which may be accompanied by localized contiguous involvement of an extralymphatic site or organ (IIE).

Stage III Involvement of lymph node regions on both sides of the diaphragm, which may also be accompanied by involvement of the spleen (IIIS) or by localized contiguous involvement of an extralymphatic site or organ (IIIE)

Stage IV Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without lymph node involvement.

Note: The absence or presence of fever (>38°C), unexplained weight loss (>10% body weight), or night sweats should be denoted by the suffix letters A or B, respectively.

with radiation therapy alone. Furthermore, even for patients with pathologically confirmed stage I and II disease, the recurrence rate after extended-field radiotherapy was approximately 20%. This led to the gradual abandonment of staging laparotomy for early-stage HL, in favor of a combined modality therapeutic approach. The addition of chemotherapy to the radiation regimen minimized the importance of detection of microscopic splenic involvement, and allowed the adoption of smaller radiation therapy portals (involved field), with significant improvements in toxicity and similar, if not better, outcomes. The elimination of staging laparotomy as part of routine staging also minimized the risk of postsplenectomy sepsis in patients with compromised immune function, and prevented prolonged treatment days associated with the hospitalization for surgical staging.

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