Angiotropic (intravascular) large cell lymphoma. A clinicopathologic study of seven cases with unique clinical presentations.

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.

Pulmonary Kaposi's sarcoma. Premortem histologic diagnosis.

Nine open lung biopsies and nine transbronchial biopsies from 10 patients with pulmonary Kaposi's sarcoma were reviewed to define the pattern of involvement in the lung by Kaposi's sarcoma and to determine the usefulness of transbronchial biopsy in making the diagnosis. There were nine patients with acquired immune deficiency syndrome (AIDS) and one patient with sporadic pulmonary Kaposi's sarcoma. A lymphatic distribution was seen in all cases. A spectrum ranging from distinctive polymorphous cellular infiltrates ultimately interpreted as Kaposi's sarcoma to "classic" Kaposi's sarcoma was found. Recognition of the former enabled retrospective recognition of Kaposi's sarcoma in four of eight transbronchial bronchial biopsies. The diagnosis of pulmonary Kaposi's sarcoma in one other patient was made solely on the basis of transbronchial biopsy. Eight patients died from pulmonary Kaposi's sarcoma; two patients are alive with extensive pulmonary Kaposi's at last follow-up. We believe that transbronchial biopsy may be useful in establishing a diagnosis of pulmonary Kaposi's sarcoma in many more patients than is generally appreciated.

Infantile digital fibromatosis occurring outside the digit.

This report illustrates an extradigital fibromatosis located in the right upper arm. The clinical, light-, and electron-microscopic features were identical to those found in the recurrent digital fibromatosis of childhood. As is typical for infantile digital fibromatosis, this reported lesion recurred once. Light microscopy showed eosinophilic, perinuclear inclusions which by electron microscopy were nonmembrane-bound, compact fibrillary structures. Whereas major reviews state that infantile digital fibromas occur exclusively on the digits, this unique case describes the rare occurrence outside the digit.

Fatal myocardial infarction following therapy with prothrombin complex concentrates in a young man with hemophilia A.

A fatal myocardial infarction in a 22-year-old man with hemophilia A and a factor VIII inhibitor is described. The catastrophic event occurred while the patient was receiving high doses of unactivated prothrombin complex concentrates. Autopsy examination revealed myocardial hemorrhage with no evidence of coronary artery disease or thrombosis. There also was postmortem evidence of previous myocardial infarctions. This is the fourth documented case of myocardial infarction occurring in a young hemophiliac patient using unactivated prothrombin complex concentrates. It is concluded that utilization of prothrombin complex concentrates in hemophiliac patients must be limited and closely monitored. Therapeutic guidelines are recommended.