Abstract
ABSTRACTObjectivesTo analyze the clinical characteristics and prognosis of primary extranodal classical Hodgkin lymphoma (PE-cHL).MethodsClinical features and outcomes of 22 PE-cHL patients who received initial chemotherapy January 2008 to January 2018 were analyzed retrospectively, and compared with 274 primary nodal Hodgkin lymphoma (PN-cHL) patients treated in the same period.ResultsWith a median follow-up period of 42 months, compared with 274 PN-cHL patients, no significant difference of overall response rate (ORR) or complete remission (CR) rate was found, but the PE-cHL patients showed a higher recurrence rate (36.4% vs. 13.1%, p = .003) and poorer survival [(5-year overall survival (OS) rate: 64.6% vs. 97.7%, p = .001; 5-year progression-free survival (PFS) rate: 42.4% vs. 82.2%, p < .001)]. To minimize the effects of confounding factors, PE-cHL patients were matched with PN-cHL patients at a ratio of 1:1 according to age, gender, histological types and stage. Compared with 22 matched PN-cHL cases, PE-cHL was still associated with poor PFS (5-year PFS: 42.4% vs. 79.9%, p = .004). As to 22 PE-cHL patients, univariate analysis showed elevated serum lactate dehydrogenase (LDH) and elevated platelet (PLT) were associated with poor PFS (p < .05).DiscussionCompared with PN-cHLs, PE-cHLs showed a considerable shorter duration of remission, higher recurrence tendency and poorer survival, indicating that more intensive therapy may be needed.ConclusionThe prognosis of PE-cHL is unfavorable. Elevated LDH and PLT are poor prognostic factors for PE-cHL.
Highlights
Hodgkin lymphoma (HL) is a sort of malignancy originating from B-cell abnormal clonal expansion
Clinical features and outcomes of 22 primary extranodal classical Hodgkin lymphoma (PE-classical Hodgkin lymphoma (cHL)) patients who received initial chemotherapy January 2008 to January 2018 were analyzed retrospectively, and compared with 274 primary nodal Hodgkin lymphoma (PN-cHL) patients treated in the same period
According to the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms [1], HL is subclassified into classical Hodgkin lymphoma and nodular lymphocyte-predominant HL (NLPHL). cHL is further divided into four histological subtypes, including nodular sclerosis (NS), mixed cellularity (MC), lymphocyte-predominant (LP) and lymphocyte-depletion (LD)
Summary
Hodgkin lymphoma (HL) is a sort of malignancy originating from B-cell abnormal clonal expansion. According to the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms [1], HL is subclassified into classical Hodgkin lymphoma (cHL) and nodular lymphocyte-predominant HL (NLPHL). CHL is further divided into four histological subtypes, including nodular sclerosis (NS), mixed cellularity (MC), lymphocyte-predominant (LP) and lymphocyte-depletion (LD). The lesions of cHL originate from organs and structures belonging to lymphoid system with favorable prognosis. CHL developing from extranodal organs may occur in some cases. Previous reports of primary extranodal cHL (PE-cHL) are rare and mostly are case analysis, and little is known about this special population. We conducted a retrospective study to analyze clinical characteristics and prognostic factors of PE-cHL
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