HIV-associated multicentric Castleman disease: a report of 19 cases at an oncology institution

Abstract

The aim of this study is to describe the clinical characteristics and outcome of multicentric Castleman disease (MCD) in HIV-infected patients at an oncological referral center in Mexico. Clinical records at the HIV-AIDS clinic of all patients diagnosed with MCD from 1994 to 2018 were reviewed. There were 19 patients, mean age was 31.3 ± 8.4 years, and 17 (89.5%) were males. Fifteen patients (79%) had also Kaposi sarcoma (KS). Main clinical characteristics were multiple lymphadenopathy (95%), systemic symptoms (63%), and hepatosplenomegaly (50%). Computed tomography scan and 2-[18F]-fluoro-2-deoxy-d-glucose positron emission tomography showed multiple lymphadenopathy, inversion of the liver:spleen uptake ratio, with an increase in SUVmax (5.7). The histopathology report described plasma cells in 58%, mixed type in 26%, and hyaline vascular in 16%. Eleven patients (57.9%) received different chemotherapy regimens. Seven patients died (36.8%): four related to MCD progression or chemotherapy complications, median survival was eight months. For those patients who survived, median, follow-up was 28 months (p < 0.001). The incidence of MCD in people living with HIV is probably underestimated. In patients with lymphadenopathy, B symptoms, deranged inflammatory markers, and/or disseminated KS, a biopsy of an enlarged lymph node is warranted, and the histology should be reviewed by an experienced pathologist.

HHV-8-associated multicentric Castleman disease with hemophagocytic syndrome in an HIV-infected patient: A case report

“…Available therapy includes steroids, single-agent, and combination chemotherapy, antiviral strategies, and monoclonal antibody therapies targeting CD20 or IL6. [1] But only rituximab-based therapy has proven to be effective either in association or alone with more than 90% of remission [3]. We treated our patient with four cycles of the combination of rituximab and etoposide, the clinical presentation and laboratory tests improved rapidly, and the follow-up chest CT scan at four months of treatment showed almost complete improvement of the lesions (figure 1 and table 1).…”

“…It represents a heterogeneous group of lymphoproliferative disorders, that share some characteristic histopathological features but have various etiologies, presentations, and treatments. [1,2] Human herpes virus-8-associated multicentric Castleman disease (HHV8-MCD) is a rare entity belonging to this group of disorders. It affects multiple lymph nodes regions and mostly occurs in immunocompromised patients including human immunodeficiency virus (HIV) infected patients as a result of HHV8 uncontrolled infection.…”

“…It affects multiple lymph nodes regions and mostly occurs in immunocompromised patients including human immunodeficiency virus (HIV) infected patients as a result of HHV8 uncontrolled infection. [1,3] Besides being rare, the clinical presentation of this disease is non-specific, making the diagnosis difficult with a rich and complex differential diagnosis. We report the case of a 58-year-old HIV-infected patient who presented with chronic fever, lymphadenopathies, pulmonary involvement, and pancytopenia.…”