SHM Converge Scientific Abstract ePoster Gallery-What the HLH Is Going On?

Pdf Summary

This case describes an 18-year-old undocumented Spanish-speaking man who presented with 2 weeks of fever, nonproductive cough, sore throat, nausea/vomiting, weight loss, and severe illness. Exam showed high fever, tachycardia, tachypnea, oropharyngeal petechiae, crusted lip lesions, cervical lymphadenopathy, hepatosplenomegaly, and delayed capillary refill.<br /><br />Initial labs revealed pancytopenia with lymphopenia, mild transaminitis, and an HIV-1/2 reactive test with a CD4 count of 10, indicating advanced AIDS. A broad infectious workup was negative for hepatitis, RMSF, Ehrlichia, blood cultures, respiratory viral panel, GC/CT, RPR, and Bartonella. Bone marrow biopsy showed no blasts or malignancy. Additional testing supported hemophagocytic lymphohistiocytosis (HLH), with fever, ferritin of 10,000 ng/mL, elevated soluble IL-2 receptor, splenomegaly, and cytopenias meeting HLH criteria.<br /><br />Imaging showed diffuse bilateral miliary-appearing pulmonary nodules. Further evaluation identified multiple opportunistic infections in the setting of advanced HIV, including HSV-1, CMV and EBV viremia, Pneumocystis jirovecii pneumonia, and positive Histoplasma and Coccidioides testing. He also had severe protein-calorie malnutrition.<br /><br />Treatment focused on the underlying infections rather than HLH-specific chemotherapy. He received acyclovir for HSV, trimethoprim-sulfamethoxazole for Pneumocystis, and amphotericin B followed by prolonged itraconazole for fungal infection. Antiretroviral therapy was started later with bictegravir/emtricitabine/tenofovir alafenamide. No separate HLH-directed immunochemotherapy was needed.<br /><br />The key teaching point is that newly diagnosed advanced HIV should prompt a broad search for opportunistic infections, because HLH may reflect severe inflammatory burden secondary to infection. Early recognition can prevent unnecessary HLH chemotherapy and guide treatment of the underlying causes first. Social barriers, including undocumented status, low health literacy, and language discordance, likely contributed to delayed presentation and required intensive support.

Asset Subtitle

Elizabeth M. O'Neil

Meta Tag

Author List Elizabeth M. O'Neil, Gretter Benitez, Matthew Howlett, Natalie Schmidt, Patrick M. O'Shea, Ria Dancel, Sahal Thahir

Category Clinical Vignettes

Concept HIV/AIDS

Concept Hemophagocytic Lymphohistiocytosis

Concept Opportunistic Infection

Concept Infectious Workup

Concept HIV Therapy

Distinguished Non-Finalist

Presenter Organization UNC Hospitals

Presenting Author Elizabeth M. O'Neil

Track Pediatric

Keywords

advanced HIV

hemophagocytic lymphohistiocytosis

opportunistic infections

AIDS

pancytopenia

Pneumocystis jirovecii pneumonia

Histoplasma

Coccidioides

CD4 count 10

antiretroviral therapy

HIV/AIDS

Hemophagocytic Lymphohistiocytosis

Opportunistic Infection

Infectious Workup

HIV Therapy