[Clinicopathologic characteristics and prognosis of neoplastic cell-rich mixed cellularity classic Hodgkin lymphoma].

Objective

To investigate the clinicopathologic characteristics of neoplastic cell-rich mixed cellularity classical Hodgkin lymphoma(MCCHL-R) and to compare the prognosis with typical mixed cellularity classic Hodgkin lymphoma(MCCHL).

Methods

Fifty-four patients with MCCHL-R(the tumor cells >10%) and 65 patients with typical MCCHL identified from 1 721 Hodgkin lymphomas were reviewed to compare the clinicopathological characteristics including morphologic and immunophenotypic features, EBV infection status, clinical therapy and overall survival.

Results

The median age of the patients of MCCHL-R was 28.5 years(range: 9-76 years, male∶female=1.6∶1.0). Twenty-seven patients(50.0%) had B symptoms. Most patients had cervical lymph node involvement(81.5%, 44/54). Mediastinum and spleen involvement were seen in 69.2%(36/54) and 24.1%(13/54), respectively. Extranodal non-lymphoid organ involvement was seen in 41.3%(19/46) cases. Morphologically, lymph node architectures were effaced at various degree with large neoplastic cells of variable morphology, including Hodgkin/Reed-Sternberg(H/RS) cells and anaplastic large cells. There were abundant background heterogeneous admixtures of non-neoplastic inflammatory and accessory cells that were predominant mature small lymphocytes. All tumors were positive for CD30 and weakly positive for PAX5. Epstein-Barr encoded RNA(EBER)detectable by in situ hybridization was seen in 39.0% cases. Forty-six patients had a median follow-up time of 32.5 months(range: 5-128 months) and the 5-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients were 91.7% and 50.1%, respectively(P<0.05). The 5-year survival rate for MCCHL-R was lower than typical MCCHL patients. Single factor analysis showed that age of >45 years, extranodal involvement and stage Ⅲ-Ⅳ were correlated with poorer 5-year survival rate(P<0.05). Multiple factors Cox proportional hazards regression showed that extranodal involvement was the independent prognostic factor(RR: 4.352, 95%CI: 1.122-16.879, P<0.05).

Conclusions

MCCHL-R is more common in young people. The tumor has pathological features of classic Hodgkin lymphoma enriched with the tumor cells(>10%) and similar immunophenotype to classical Hodgkin lymphoma. Compared with typical MCCHL, extranodal disease is an independent prognostic factor of MCCHL-R.