Clinical Features

In a retrospective review of 219 LP HL cases, the majority of affected patients were male (74%) and most patients presented with stage I-II disease (81%).5,44 These patients typically present with isolated cervical, axillary, or inguinal node involvement, while mediastinal, splenic, or bone marrow involvement is uncommon.45 Likewise, bulky disease and B symptoms are infrequent. In the same series, only 7% of patients presented with mediastinal involvement, and only 10-13% of patients had bulky disease or bone marrow involvement.5,44

Patients with LP HL often experience multiple relapses, although the disease progresses slowly and is usually quite responsive to therapy at the time of relapse, leading to little impact on overall survival.44,45 The European Task Force on Lymphoma reported a 10-year overall survival exceeding 90% for patients with LP HL, compared to a 10-year progression-free survival of approximately 75%.5,44 In a second retrospective review of 50 cases of LP HL, 78% of patients presented with early-stage disease, and overall survival at 4 years reached 92%.8 Similar results were reported in a Stanford series of 59 patients with LP HL.9 Interestingly, some case series have reported a high rate of second malignancies, ranging from 12 to 14%,5 8 in patients with LP-HL, possibly attributable to either extended radiation fields or chemotherapy historically used in the treatment of this disease. In addition, transformation to diffuse large B-cell lymphoma has also been described.46-48

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