doi: 10.1097/01.HS9.0000547893.88491.92
Survivorship and Patients Perspective

Jun Zhu, Mingzi Yang, Meng Wu, Lingyan Ping, Aliya, Yanfei Liu, Reyizha Nuersulitan, Yuqin Song

Peking University Cancer Hospital & Institute

Objective: Primary extranodal Hodgkin lymphoma is rare. We conducted a retrospective study to analyze clinical characteristics and prognostic factors of primary extranodal classical Hodgkin lymphoma (cHL).

Methods: From January 2008 to January 2018, 27 cases with cHL treated in Lymphoma Department of Peking University Cancer Hospital were identified as primary extranodal lymphoma. We retrospectively reviewed their clinical features and prognosis, and compared their outcome with 366 cases of non-primary extranodal cHL treated in the same period. To minimize the effects of confounding factors, primary extranodal cHL patients were matched with non-primary extranodal cHL patients at a ratio of 1:1 by age, gender and histological types using propensity score matching of SPSS software.

Results: Among 27 patients, 15 (56%) were male. The median age was 30 years (15–69). The most common extranodal lesion was lung (n = 16, 59%), followed by bone, thoracic wall, nasopharynx, parotid gland, brain and adrenal gland. Comparing with 366 cases of non-primary extranodal cHL, with a median follow-up time of 41 months, the overall response rate (ORR) was 81.5% vs. 91.3%, (P = 0.093), and complete remission (CR) rate was 51.9% vs. 66.9% (P = 0.11), showed no significant differences. But primary extranodal cHL patients did have a higher recurrence rate (44% vs. 14.8%, P < 0.01) and poorer survival (5-year OS 63.5% vs. 91.9%, P < 0.01; 5-year PFS 35.1% vs. 79.6%, P < 0.01). Comparing with 27 matched non-primary extranodal cHL cases, the OS rates showed no significant differences (5-year OS 63.5% vs. 94.4%, P = 0.14); but primary extranodal cHL was still associated with poor PFS (5-year PFS 35.1% vs. 80.5%, P = 0.025). As to 27 cases with primary extranodal cHL, univariate analysis showed malignancy family history, originating from bone, elevated serum lactate dehydrogenase (LDH) and elevated alkaline phosphatase (ALP) were associated with poor OS (P < 0.05); elevated LDH, ALP and platelet (PLT) were associated with poor PFS (P < 0.05). Multivariate analysis showed that LDH level was an independent prognostic factor of PFS (p = 0.015).

Conclusion: Comparing with non-primary extranodal cHL patients, primary extranodal cHL showed a considerable shorter duration of remission, higher recurrence tendency and poorer survival. Malignancy family history, originating from bone, elevated LDH, ALP and PLT were poor prognostic factors.

Copyright © 2018 The Authors. Published by Wolters Kluwer Health Inc., on behalf of the European Hematology Association.