Various
infectious diseases can hyper-stimulate the
immune system, causing
hemophagocytic syndrome (HPS). Little is known regarding the accuracy of diagnostic criteria and epidemiological triggering factors in the
acquired immunodeficiency syndrome (
AIDS) setting. We investigated the major
infectious disease triggers of HPS in
patients living with
human immunodeficiency virus (
HIV)/
AIDS and determined the accuracy of
bone marrow aspiration (BMA). The inclusion criteria were (i) confirmed
HIV diagnosis, (ii)
bone marrow aspiration, and (iii) a minimum of four HPS criteria.
Patients were further classified into those with four presumed HPS criteria, or ≥ 5 confirmed criteria. The
disease triggers, accuracy of
bone marrow aspiration, and
prognosis markers were examined. Presumed HPS was observed in 15/36
patients (41%), and confirmed HPS in 58% (n = 21). The major etiological triggers were
infection with
Mycobacterium (34%),
Cytomegalovirus (14%),
Cryptococcus neoformans (11%), and hematological or tumoral
disease (11%). BMA demonstrated 93%
specificity on
screening diagnosis (
odds ratio [OR] 12.7, 95%
confidence interval [CI] 1.4-115.1, P = 0.01).
Ferritin > 5000 ng/mL correlated with
probability of
death in univariate
analysis (OR 6.00, 95% CI 1.33-27.05, P = 0.02).
Ferritin performance as test of
death probability presented area under the curve as 0.74 (95% CI 0.56-0.91, P = 0.016). However, neither cluster of differentiation for
lymphocyte count nor
HIV viral load correlated with
patient deaths.
Mycobacterium spp. and
Cytomegalovirus were the main factors triggering HPS, followed by
Cryptococcus neoformans, and hematological and tumoral
diseases. High
ferritin levels were associated with increased
death probability. High
specificity was noted with BMA