So-called “Lennert's Lymphoma”. Is it a clinicopathologic entity?

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Abstract
In order to investigate the natural history of Lennert''s lymphoma and to reevaluate whether non-Hodgkin''s lymphoma with a high content of epithelioid histiocytes represents a clinicopathologic entity, the histopathologic and clinical features of 60 patients were reviewed. Pretreatment diagnostic tissues in these patients had shown a diffuse and florid epithelioid histiocytic reaction identical to that originally described by Lennert and Mestdagh. Lennert''s lymphoma is a heterogeneous group of disorders, which, in this series, included Hodgkin''s disease (27 patients), non-Hodgkin''s lymphoma (24 patients), angioimmunoblastic lymphadenopathy (1 patient) and atypical lymphoepithelioid cell proliferations of uncertain etiology and pathogenesis (8 patients). Most of the patients with Hodgkin''s disease had stage I or II disease without B symptoms, whereas patients with non-Hodgkin''s lymphoma usually had stage III or IV disease, commonly with B symptoms. The median survival was 79 mo. in the Hodgkin''s disease group, compared with 12 mo. in patients with non-Hodgkin''s lymphoma (P < 0.0001). In patients with atypical lymphoepithelioid cell proliferations, the survival pattern was unpredictable, and the number of patients was too small for a meaningful statistical comparison. Progression to malignant lymphoma in 1 of the 8 patients with atypical lymphoepithelioid cell proliferations, however, underscores the malignant potential of this disorder. One patient with angioimmunoblastic lymphadenopathy had generalized disease and constitutional symptoms. In Hodgkin''s disease with a prominent epithelioid histiocytic reaction, the gross and microscopic features were similar to those observed in Hodgkin''s disease in which this reaction was lacking. In non-Hodgkin''s lymphoma, however, the macroscopic and microscopic features differed from those of the usual non-Hodgkin''s lymphomas. Moreover, subdivision into poorly differentiated lymphocytic, mixed and histiocytic types did not reveal any differences in median survival among these subtypes. Non-Hodgkin''s lymphoma with a multifocal epithelioid histiocytic reaction, previously included in the heterogeneous group called Lennert''s lymphoma, appears to be a distinct clinicopathologic entity.