Lymphoma of uncertain phenotype

Abstract

In February 1987, a 42-year-old Caucasian man was referred to our department with symptoms of left recurrent nerve palsy and a 12-month history of lymphadenopathy involving both sides of the neck, the axillae and the anterior mediastinum. Previous neck node biopsies taken in September 1986 had shown evidence of lymphoid hyperplasia consistent with reactive lymphadenitis, and follicular and parafollicular hyperplasia was diagnosed. His past medical history included two episodes of transient transverse myelitis, in 1973 and 1980. He had suffered from nasal sinus polyposis with chronic sinusitis since infancy. Further biopsies of neck nodes were taken and showed pleomorphic malignant cells infiltrating the interfollicular areas as well as several Reed-Sternberg (RS) cells. The diagnosis of Hodgkin’s disease (HD), interfollicular type, was made and confirmed by external expert review (Fig. la). The RS cells were CD30+ (Fig. lb), CD15+, leukocyte common antigen (LCA)-, CD3-. In the perifollicular zone, small T lymphocytes formed rosette-like arrangements around the RS cells. The Ann Arbor clinical stage was IIA. The only blood abnormality was a monocytosis at 1.4 × 109/L (N<0.8×l09). Because of the history of transverse myelitis, the patient was not considered suitable for radiotherapy and he received 3 courses of combination chemotherapy with doxorubicin, bleomycin, etoposide and prednisone, leading to partial remission, followed by 3 further courses of cyclophosphamide, vincristine, procarbazine and prednisone. A complete clinical remission was obtained in November 1987.