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Hypercalcemia and T-Cell Lymphoma

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Hypercalcemia and T-Cell Lymphoma
We describe the case of a patient with acquired immunodeficiency syndrome (AIDS) who had a CD4 cell count of 60/mL, bilateral hilar adenopathy, and hypercalcemia. Transbronchial biopsy showed T-cell anaplastic large cell lymphoma. Serology was negative for human T-cell leukemia virus-I (HTLV-I). This appears to be the first case of T-cell anaplastic large cell lymphoma occurring in an AIDS patient with hypercalcemia who was seronegative for HTLV-I.

Non-hodgkin's lymphoma (NHL) is the second most common malignancy associated with AIDS. A 2.9% incidence of NHL in AIDS patients was reported in the United States during 1981 to 1989. The AIDS-associated NHLs may be broadly classified into systemic, primary central nervous system (CNS), and body cavity-based disease. A CD4 count less than 100/mL, a Karnofsky score less than 70%, history of opportunistic infections, and extranodal involvement are associated with an unfavorable outcome.

Anaplastic large cell lymphoma (ALCL) is a distinct pathologic category of NHL that has been previously described. A pleomorphic neoplastic cell population characterizes these lymphomas. They have a predilection for sinusoids of lymph nodes and extranodal sites simulating the appearance of carcinoma. They consistently react with monoclonal antibodies Ki-1 and BerH-2, both recognizing the CD30 antigen. The majority of ALCLs are of T-cell origin in individuals without human immunodeficiency virus (HIV), while the B-cell immunophenotype is more common in patients with HIV. The development of ALCL has been associated with infections by HTLV-I, Epstein-Barr virus (EBV), and human herpesvirus 8 (HHV-8).

Anaplastic large cell lymphoma accounts for 1% to 8% of all NHL. In HIV-seropositive individuals, ALCL represents 13% to 14% of all systemic NHLs, and the T-cell immunophenotype is rare. Viruses like EBV and HTLV-I are considered to be potent stimulators of CD30 antigen in vitro, implying that they may play an important role in the development of NHL. However, HTLV-I is known to cause adult T-cell leukemia/lymphoma, characterized by a multilobulated nuclear morphology, hypercalcemia, and, frequently, a leukemic phase. Although no direct association between HIV and T-cell ALCL is known, loss of immunocompetence may contribute to oncogenesis, as with other lymphomas.

We describe the case of a patient with AIDS who tested negatively for HTLV-I and had T-cell ALCL and hypercalcemia.

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