The authors recently reported the antigenic phenotypes of three cases of so-called "malignant angioendotheliomatosis" and suggested that angiotropic large cell
lymphoma (ALCL) is a more appropriate designation for this disease. The authors now report an additional seven cases of ALCL with unique clinical presentations. One patient presented with prostate enlargement, the second with lytic bone lesions and thickened nasal sinus mucosa, the third had diffuse
myalgia, the fourth had
dyspnea and pulmonary infiltrates, the fifth had
gangrene of the lower extremities, total-body skin involvement, and
pancytopenia, the sixth had a lesion of the foreskin mimicking
squamous cell carcinoma, and the seventh had a mediastinal mass. In all cases histologic features were characteristic of ALCL with, in two cases, extravascular spread into soft tissue. Immunohistologic studies showed a B-cell phenotype in five cases and a T-cell phenotype in one case. Two patients received
combination chemotherapy using established treatment protocol for large cell
lymphoma, and remain in complete clinical remission and two patients are responding clinically to
combination chemotherapy. Two patients died shortly after receiving
combination chemotherapy. One patient has only recently been diagnosed as having ALCL and no long-term follow-up is available. These data indicate that, although ALCL affects predominantly the central nervous system and skin, unusual clinical presentations may occur, and patients with ALCL may respond to
combination chemotherapy for large cell
lymphoma.