Work

331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination

Year: 2019

Type: conference-abstract

Abstract: Background Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder that can be either primary (genetic) or secondary (reactive) in etiology. Diagnosis can be elusive, especially in patients with HIV infection. Methods Medical records were reviewed for 5 patients with HIV infection and the diagnosis of HLH. Standard and alternative criteria were utilized to establish the diagnosis. Results Five patients with HIV infection had clinical criteria for the diagnosis of HLH. Ages ranged from 33–70 years and 4 were males. All five presented with fevers, cytopenias, and markedly elevated ferritin levels (table). All of the patients had CD4 levels of < 200 cells/µL. Evidence of hemophagocytosis was found on bone marrow examination in 3 patients. Inciting conditions included Pneumocystis jiroveci infection, EBV infection, lymphoma, and multiple myeloma. All patients received broad-spectrum antimicrobial as well as immunosuppressive therapy. Despite aggressive treatments, all patients died within one month of presentation. Conclusion In patients with underlying HIV infection, HLH can be a difficult diagnosis to establish. Mortality rates can be high, even with prompt recognition and therapy. The finding of fever and cytopenia in a patient with HIV infection should prompt the clinician to determine a ferritin level. If markedly elevated, the diagnosis of HLH should be aggressively pursued. Disclosures All authors: No reported disclosures.

Source: Open Forum Infectious Diseases

DOI: 10.1093/ofid/ofz360.404

Authors: Isha Bhatt, Zeb Khan, Paul Lam, John M Quale

Institution: SUNY Downstate Health Sciences University

Language: English

Topic: Autoimmune and Inflammatory Disorders Research

Subfield: Hematology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $4,053