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The Ann Arbor staging classification is shown in Table 66-5. Disease is staged by performing physical examination, chest x-ray, thoracoabdominal CT, bone marrow biopsy, ultrasound examinations, and lymphangiogram. Staging laparotomy should be used, especially to evaluate the spleen, if pt has early-stage disease on clinical grounds and radiation therapy is being contemplated. Pathologic staging is unnecessary if the pt is treated with chemotherapy.

Treatment: Hodgkin's Disease

About 85% of pts are curable. Therapy should be performed by experienced clinicians in centers with appropriate facilities. Most pts are clinically staged and treated with chemotherapy alone or combined-modality therapy. Those with stage II disease often receive either two or four cycles of doxorubicin, bleomycin, vinblastine, dacarbazine (ABVD) with or without involved-field radiation therapy or Stanford V, a combined-modality program using lower doses of chemotherapy. Those with stage III or IV disease receive six cycles of combination chemotherapy, usually ABVD. Pts with any stage disease accompanied by a large mediastinal mass (greater than one-third the greatest chest diameter) should receive combined-modality therapy with MOPP/ABVD or mechlorethamine, vincristine, procarbazine, prednisone (MOPP)-ABV hybrid followed by mantle field radiation therapy. (Radiation plus ABVD is too toxic to the lung.) A persistently positive midtreatment positron emission tomography scan may be an index of risk of relapse and need for additional therapy. About one-half of pts (or more) not cured by their initial chemotherapy regimen may be rescued by high-dose therapy and autologous stem cell transplant. Brentuximab vedotin, an anti-CD30 drug conjugate, has activity in pts relapsing after transplant.

With long-term follow-up, it has become clear that more pts are dying of late fatal toxicities related to radiation therapy (myocardial infarction, stroke, second cancers) than from HD. It may be possible to avoid radiation exposure by using combination chemotherapy alone in early-stage disease as well as in advanced-stage disease.

For a more detailed discussion, see Longo DL: Malignancies of Lymphoid Cells, Chap. 134, p. 695; Tefferi A, Longo DL: Less Common Hematologic Malignancies, Chap. 135e; Munshi NC, Longo DL, Anderson KC: Plasma Cell Disorders, Chap. 136, p. 710 in HPIM-19.

Outline

Section 6. Hematology and Oncology