Histoplasmosis-induced Hemophagocytic Syndrome: A Case Series
Methods: We performed a retrospective chart review study of all inpatients at Parkland Memorial Hospital diagnosed with HLH associated with Histoplasma capsulatum from 2003-2013.
Results: Eleven cases of histoplasmosis-associated HLH over this time period were available for review. 9 of 11 cases were males (82%) with a mean age of 46.8 years. 9 had AIDS with a CD4 <100 cells/mm3, one was a renal transplant, and the last had no immune compromise. The most frequently seen HLH criteria were splenomegaly (n=10), fever (n=10), and a ferritin >500 ng/dL (n=8). The urine Histoplasma antigen was positive in every patient examined (n=9/11), and most antibodies for Histoplasma were positive if checked (n=4/5). Blood culture and bone marrow yielded the highest positive cultures (64% and 54% respectively). Four of the HIV patients were on HAART at diagnosis. A majority of patients received liposomal amphotericin B during their hospital stay (n=9) with average duration of treatment being 11 days. Five patients received immunosuppressive treatments during admission, including prednisone and IVIG. Five patients died within 30 days (45.5%), and seven within 90 days (63.6%). Of the 5 patients that received immunosuppression, 4 died (80%), whereas in the group not given additional immunosuppression (n=5), only 2 died (20%).
Conclusion: We describe the largest case series to date of HLH related to histoplasmosis. Clinicians should consider this diagnosis in HIV+ and other immunocompromised patients in endemic areas with the clinical features described. Histoplasmosis-associated HLH among adults remains a lethal disease of highly immune compromised patients.
K. Bowman, None
A. Nijhawan, None