Hospital de Clinicas de Porto Alegre (HCPA), Federal University of Rio Grande do Sul (UFRGS), Rua Ramiro Barcelos 2350/7, Porto Alegre, CP 90.030-003, Brazil
*E-mail: edsprinz@via-rs.net
The use of highly active antiretroviral therapy (HAART) was associated with a dramatic improvement in the outcome of patients with human immunodeficiency virus (HIV) infection [1, 2]. In this report, we would like to describe the case of a patient with AIDS-related multicentric Castlemans disease who has also enjoyed a long-lasting complete clinical and pathological remission following the use of HAART alone.
Castlemans disease belongs to the group of atypical lymphoid proliferations, which are usually confused with the diagnosis of malignant lymphoma. First described in 1956, the typical patient presents with either localized mediastinal lymphadenopathy or a more aggressive form of the disease characterized by diffuse lymphadenopathy and systemic symptoms [3]. The differential diagnosis of Castlemans disease should include malignant lymphoma, as well as other causes of lymphoma-like syndromes, such as adverse reactions to drugs, autoimmune disorders and viral or bacterial infections.
Until recently, Castlemans disease was not considered an AIDS-related event, being managed with local surgery, irradiation and cortisteroids. Cytotoxic therapy was reserved only for highly symptomatic patients with refractory disease. Over the past years, however, a newly described AIDS-related multicentric form of Castlemans disease (MCD) has been recognized. In this aggressive form of the disease, patients develop progressive lymphadenopathy, B symptoms and usually a fatal course [4].
Our patient was a 46 year-old HIV-positive black man who presented with dyspnea, cough, hemoptysis and severe weight loss. He had fever, bibasilar rales and widespread lymphadenopathy. The blood tests were normal, except for a hemoglobin level of 10.7 g/dl. The chest X-ray showed bilateral reticulonodular infiltrates and the computed tomography (CT) scan revealed multiple mediastinal lymph nodes (Figure 1a). The CD4 count was 54 cells/mm3 and the CD4/CD8 ratio was 0.06. A supraclavicular and submandibular lymph node biopsy confirmed MCD. The additional medical work-up ruled out malignant lymphoma, catch-scratch fever, autoimmune or infectious diseases.
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Our report comes as the first in which a complete histologically-proven response for a prolonged period of follow-up is clearly documented in AIDS-related MCD with the use of HAART alone. There are other reports on the clinical outcome of AIDS-related MCD, using a variety of combined therapeutic approaches including antiviral drugs, interferon-, anti-interleukin-6 and chemotherapeutic agents along with HAART. In general, tumor responses are partial and short-lasting, sometimes at the cost of significant clinical toxicity [58]. It should be noted that our patient did not develop a clear neoplastic transformation of the disease into a malignant lymphoma after such a long-term follow-up period. Considering the poor treatment results and high risk of infectious complications following cytotoxic therapy in the AIDS population, we postulate that patients with AIDS-related MCD should be first managed with HAART, leaving more aggressive therapeutic approaches for patients at relapse.
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