Angioimmunoblastic lymphadenopathy evolution to a Burkitt-like lymphoma

Abstract

In a 64 year old white woman with angioimmunoblastic lymphadenopathy of one and one half years' duration, a primitive lymphoid neoplasm developed characterized by generalized lymphadenopathy, hepatosplenomegaly, and bone marrow and peripheral blood infiltration by lymphoid blast cells. The blast cells demonstrated a high nuclear-cytoplasmic ratio, deeply basophilic cytoplasm and prominent cytoplasmic vacuoles, features similar to those described in both immunoblastic and Burkitt cell leukemias. The patient's clinical course resembled that of Burkitt's lymphoma with markedly elevated serum lactate dehydrogenase levels that paralleled disease activity, hyperuricemia before therapy, and marked tumor sensitivity to the administration of cyclophosphamide, vincristine, cytosine arabinoside and prednisone resulting in acute hyperkalemia, hyperphosphatemia and hypocalcemia. The patient died three and one half months after malignant transformation with leukemic meningitis. Cytogenetic studies of circulating blast cells revealed a distinctive translocation from the long arm of chromosome 8 to the long arm of chromosome 14 (46XX, t(8q −: 14q +)). This karyotype has previously been correlated with Burkitt's lymphoma. The blast cells possessed no demonstrable B- or T-cell surface markers, nor did they transform to a continuous cell line in tissue culture. Antibody to Epstein-Barr viral capsid antigen was minimally increased at a titer of 1:80. No antibody to Epstein-Barr early antigen was present. This patient demonstrates the clinical, pathologic and cytogenetic overlap among Burkitt cell and immunoblastic leukemias and lymphomas. These disease processes appear to be part of a continuum in which this patient represents an intermediate form.