Case Presentation: A 19-year-old male with recent diagnosis of human immunodeficiency virus (HIV) (CD4 580/mm³ and viral load 1.2 million/mm³) and Kaposi sarcoma (KS) was transferred to our hospital for refractory thrombocytopenia. Prior to his presentation, he experienced a month of fatigue, night sweats, weight loss, and facial and neck swelling. His outside hospital workup revealed KS with lymph node involvement and a platelet count of 12,000/mm³; Biktarvy was initiated for HIV treatment. His thrombocytopenia was refractory to platelet infusions, steroids, intravenous immunoglobulin, and romiplostim. He was fatigued without acute distress. He had multiple 1-3 centimeter pre- and post-auricular, submental, cervical, supraclavicular, axillary, epitrochlear, inguinal, and popliteal lymph nodes with diffuse petechiae and ecchymoses on both upper extremities. He had no abnormal heart or lung sounds, hepatosplenomegaly, or jaundice. He had no other mucocutaneous lesions. Laboratory workup found white blood cell count of 11,000/mm³, hemoglobin of 9 g/dL, and platelet count of 8,000/mm³. Updated CD4 count was 1,001/mm³. LDH was 233 units/L. HHV-8 viral load was 54,200/mL. The patient’s work-up was consistent with idiopathic thrombocytopenic purpura (ITP). During his stay, he developed a fever to 38.4°C and a pleural effusion in the context of HHV-8 viremia. He was diagnosed with Kaposi sarcoma inflammatory cytokine syndrome (KICS). Although there was concern for bone marrow toxicity with initiating doxorubicin for KICS, his prognosis without treatment was poor. Doxorubicin was initiated and tolerated without complication. His platelets responded to romiplostim and rituximab to 53,000/mm³.
Discussion: Historically, acquired immunodeficiency syndrome associated KS was usually diagnosed in patients with CD4 counts below 200 cells/mm³. However, over the years, reports have emerged describing KS in patients with CD4 counts exceeding 300 cells/mm³ in both antiretroviral compliant and naïve patients. Our case presents a similar patient whose CD4 count was normal with close proximity of HIV and KS diagnoses. Additionally, though KS or ITP alone are known complications of HIV, simultaneous diagnosis is rare. This created a challenge for treatment planning. Rituximab monotherapy for ITP could have worsened the patient’s KS, and doxorubicin could exacerbate his thrombocytopenia. The closest analogous condition was KSHV-associated Multicentric Castleman Disease which can also cause thrombocytopenia; to treat it, both medications are used. These combined with romiplostim finally caused our patient’s platelets to rise.
Conclusions: This case demonstrates that KS can be an early manifestation in those with HIV. As such, it is imperative to monitor for manifestations of KS in patients with new HIV diagnoses and in those with CD4 counts above 200 cells/mm³. Additionally, we describe the concurrent diagnoses of KS and ITP in a patient with HIV with a successful treatment plan which may be used for patients similarly faced with both diagnoses in the future.