A 49-year-old man diagnosed with human immunodeficiency virus (HIV) two years earlier and started on antiretroviral therapy two months prior presented to a clinic with fever, diffuse palpable lymphadenopathy, and shortness of breath. His CD4 count and viral load were 480 cells/mm3and 127,000 copies/mL, respectively. He was admitted to the hospital and treated for methicillin-resistant staphylococcus aureus (MRSA) pneumonia. Shortly after treatment was initiated, he developed a diffuse, maculopapular rash consistent with a drug eruption, which improved when his medications were changed. During the hospital stay, the patient also underwent a cervical lymph node biopsy with instructions to follow-up on an outpatient basis to receive the results. The patient was discharged home.
Five days later, the patient was readmitted after presenting with a worsening pruritic, papular rash and progressive, diffuse edema including angioedema of his upper lip. He also had continued lymphadenopathy and new violaceous skin lesions on his chest and feet. Computed tomography scan showed interlobular septal and peribronchovascular thickening, pleural effusions, mediastinal and axillary adenopathy, and splenomegaly. Laboratory testing showed normocytic anemia, thrombocytopenia, and leukocytosis. His CD4 count increased to 544 cells/mm3, and his viral load decreased to 19,000 copies/mL. Biopsy of the new skin lesions revealed Kaposi’s sarcoma (KS). The lymph node biopsy from the previous stay revealed a plasma cell variant Kaposi’s sarcoma herpesvirus (KSHV)-associated multicentric Castleman’s disease (MCD).
He received rituximab and was later placed on cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). The patient’s symptoms improved over the next two weeks, and he was discharged home with an appointment for additional CHOP therapy. Prior to the next scheduled appointment, the patient presented to the emergency room (ER) with progressive dyspnea. He coded in the ER and never recovered. Sputum cultures were positive for MRSA. He was placed on comfort care approximately 10 days after admission and passed away several days later.
Most life threatening opportunistic infections in HIV patients occur when their CD4 counts drop below 200 cells/mm3. However, KSHV-associated MCD, a rare lymphoproliferative disorder, tends to strike HIV patients with relatively intact CD4 counts. Patients typically present with nonspecific symptoms often including fever, generalized peripheral lymphadenopathy, weakness, and night sweats. Although diagnosis is typically made by histopathologic examination, no single set of criteria exists for active MCD requiring treatment.
Rapid diagnosis of active MCD is essential. Untreated active MCD can result in hemophagocytic syndrome, multiple organ failure, infections, or transformation to lymphoma, all of which can be fatal. Among therapies currently used are the therapies used in this patient, rituximab and CHOP, as well as anti-herpesvirus therapy and targeted therapy aimed at interleukin-6 production, dysregulation of which has been found to be associated with the symptoms of MCD.
This case illustrates the potential to identify MCD early in the course of the disease, particularly where an HIV patient with a relatively normal CD4 presents with nonspecific symptoms including fever and diffuse lymphadenopathy. Recognition of this disease early in its development is critical to prevent or slow progression of disease.
To cite this abstract:Rodriguez M, Shiao J, Bowers K. Culprit in Camo: Kshv-Associated Multicentric Castleman’s Disease. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 765. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/culprit-in-camo-kshv-associated-multicentric-castlemans-disease/. Accessed March 29, 2020.