Primary Lymph Node Kaposis Sarcoma in Two HIV Positive Patients Presenting with Generalized Lymphadenopathy and Pancytopenia in a Third Level Hospital in Guatemala

Abstract

Kaposi’s Sarcoma (KS) is a malignant systemic disease that originates from vascular endothelium with a variable clinical course. Once considered a rare disease, with the advent of the HIV epidemic, it has become one of the most common malignancies associated with the infection. It has different presentations: the classic Kaposi’s sarcoma presenting with proliferative cutaneous lesions in lower extremities of elderly men of Mediterranean and Jewish origin. The endemic African Kaposi’s sarcoma, affecting both children and adults, more aggressive and with frequent dissemination to bone, skin and lymph nodes, the epidemic, HIV-associated Kaposi’s sarcoma 20,000 times more common in persons with AIDS than in the general population, and 300 times more common in AIDS than in other immunosuppressed host. Finally the organ transplanted associated KS caused by the immunosuppression therapy [1]. The Human Herpes Virus-8 infection (HHV8) characterizes all forms and probably represents the same pathogenic process as the AIDS-associated Kaposi’s sarcoma. Lesions of epidemic KS may arise on the skin and the mouth and may affect the lymph nodes and other organs, usually the gastrointestinal tract, lung, liver, and spleen. In contrast, classic KS usually involves only one or a few areas of skin, most often the lower legs. At the time of diagnosis of KS some people experience no symptoms, especially if their only lesions are on the skin. However, many of those with epidemic KS, even those without skin lesions, will have enlarged lymph nodes, fever or weight loss. Eventually, in almost all cases, epidemic KS spreads throughout the body. Extensive KS lung involvement can be fatal. Primary KS of lymph nodes is a rare presentation of the disease. We present two consecutive HIV patients without skin lesions and with generalized lymphadenopathy and pancytopenia diagnosed with Kaposi’s sarcoma of lymph nodes with excisional biopsy.