Histoplasmosis-induced hemophagocytic syndrome: A case series and review of the literature

Jennifer Leigh Townsend, Satish Shanbhag, John Hancock, Kathryn Bowman, Ank E. Nijhawan

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Background. Histoplasmosis-associated hemophagocytic lymphohistiocytosis (HLH) is a relatively rare disorder for which data are limited regarding optimal treatment and clinical outcomes in adults. We describe the clinical features, treatment, and outcomes of patients with histoplasmosis-associated HLH at our institution. Methods. We performed a retrospective chart review of all inpatients at Parkland Hospital diagnosed with HLH associated with Histoplasma capsulatum from 2003 to 2013. Results. Eleven cases of histoplasmosis-associated HLH over this time period were identified. Nine of eleven cases were males (82%). Nine of these patients had human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS), 1 was a renal transplant patient on immunosuppressants, and the other had no documented immunocompromise. The most common HLH criteria were splenomegaly (n = 10), fever (n = 10), and ferritin >500 ng/dL (n = 9). Urine Histoplasma antigen was positive in every patient tested (n = 9 of 9), and most antibodies for Histoplasma were positive if checked (n = 4 of 5). A majority of patients received liposomal amphotericin B (n = 9) with an average treatment duration of 11 days, and 5 patients also received prednisone, intravenous immunoglobulin (IVIG), or both. Overall, 5 patients died within 30 days (45.5%), and 7 patients died 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), 2 died (40%). Conclusions. Histoplasmosis-associated HLH among adults is a lethal disease of highly immunocompromised patients, especially patients with HIV/AIDS. Clinical features such as splenomegaly, elevated ferritin, and cytopenias should prompt evaluation for HLH in this population. Further data are needed to define the role of immunosuppression, IVIG, and highly active antiretroviral therapy in treating this condition.

Original languageEnglish (US)
Article numberofv055
JournalOpen Forum Infectious Diseases
Volume2
Issue number2
DOIs
StatePublished - 2015

Fingerprint

Hemophagocytic Lymphohistiocytosis
Histoplasmosis
Histoplasma
Immunosuppression
Intravenous Immunoglobulins
Splenomegaly
Ferritins
Acquired Immunodeficiency Syndrome
HIV
Highly Active Antiretroviral Therapy
Immunocompromised Host
Immunosuppressive Agents
Prednisone
Inpatients
Fever
Urine
Transplants

Keywords

  • Disseminated histoplasmosis
  • Hemophagocytic syndrome
  • HIV

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology

Cite this

Histoplasmosis-induced hemophagocytic syndrome : A case series and review of the literature. / Townsend, Jennifer Leigh; Shanbhag, Satish; Hancock, John; Bowman, Kathryn; Nijhawan, Ank E.

In: Open Forum Infectious Diseases, Vol. 2, No. 2, ofv055, 2015.

Research output: Contribution to journalArticle

Townsend, Jennifer Leigh ; Shanbhag, Satish ; Hancock, John ; Bowman, Kathryn ; Nijhawan, Ank E. / Histoplasmosis-induced hemophagocytic syndrome : A case series and review of the literature. In: Open Forum Infectious Diseases. 2015 ; Vol. 2, No. 2.
@article{cb137b5a9dde438db98003966caebda6,
title = "Histoplasmosis-induced hemophagocytic syndrome: A case series and review of the literature",
abstract = "Background. Histoplasmosis-associated hemophagocytic lymphohistiocytosis (HLH) is a relatively rare disorder for which data are limited regarding optimal treatment and clinical outcomes in adults. We describe the clinical features, treatment, and outcomes of patients with histoplasmosis-associated HLH at our institution. Methods. We performed a retrospective chart review of all inpatients at Parkland Hospital diagnosed with HLH associated with Histoplasma capsulatum from 2003 to 2013. Results. Eleven cases of histoplasmosis-associated HLH over this time period were identified. Nine of eleven cases were males (82{\%}). Nine of these patients had human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS), 1 was a renal transplant patient on immunosuppressants, and the other had no documented immunocompromise. The most common HLH criteria were splenomegaly (n = 10), fever (n = 10), and ferritin >500 ng/dL (n = 9). Urine Histoplasma antigen was positive in every patient tested (n = 9 of 9), and most antibodies for Histoplasma were positive if checked (n = 4 of 5). A majority of patients received liposomal amphotericin B (n = 9) with an average treatment duration of 11 days, and 5 patients also received prednisone, intravenous immunoglobulin (IVIG), or both. Overall, 5 patients died within 30 days (45.5{\%}), and 7 patients died 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), 2 died (40{\%}). Conclusions. Histoplasmosis-associated HLH among adults is a lethal disease of highly immunocompromised patients, especially patients with HIV/AIDS. Clinical features such as splenomegaly, elevated ferritin, and cytopenias should prompt evaluation for HLH in this population. Further data are needed to define the role of immunosuppression, IVIG, and highly active antiretroviral therapy in treating this condition.",
keywords = "Disseminated histoplasmosis, Hemophagocytic syndrome, HIV",
author = "Townsend, {Jennifer Leigh} and Satish Shanbhag and John Hancock and Kathryn Bowman and Nijhawan, {Ank E.}",
year = "2015",
doi = "10.1093/ofid/ofv055",
language = "English (US)",
volume = "2",
journal = "Open Forum Infectious Diseases",
issn = "2328-8957",
publisher = "Oxford University Press",
number = "2",

}

TY - JOUR

T1 - Histoplasmosis-induced hemophagocytic syndrome

T2 - A case series and review of the literature

AU - Townsend, Jennifer Leigh

AU - Shanbhag, Satish

AU - Hancock, John

AU - Bowman, Kathryn

AU - Nijhawan, Ank E.

PY - 2015

Y1 - 2015

N2 - Background. Histoplasmosis-associated hemophagocytic lymphohistiocytosis (HLH) is a relatively rare disorder for which data are limited regarding optimal treatment and clinical outcomes in adults. We describe the clinical features, treatment, and outcomes of patients with histoplasmosis-associated HLH at our institution. Methods. We performed a retrospective chart review of all inpatients at Parkland Hospital diagnosed with HLH associated with Histoplasma capsulatum from 2003 to 2013. Results. Eleven cases of histoplasmosis-associated HLH over this time period were identified. Nine of eleven cases were males (82%). Nine of these patients had human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS), 1 was a renal transplant patient on immunosuppressants, and the other had no documented immunocompromise. The most common HLH criteria were splenomegaly (n = 10), fever (n = 10), and ferritin >500 ng/dL (n = 9). Urine Histoplasma antigen was positive in every patient tested (n = 9 of 9), and most antibodies for Histoplasma were positive if checked (n = 4 of 5). A majority of patients received liposomal amphotericin B (n = 9) with an average treatment duration of 11 days, and 5 patients also received prednisone, intravenous immunoglobulin (IVIG), or both. Overall, 5 patients died within 30 days (45.5%), and 7 patients died 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), 2 died (40%). Conclusions. Histoplasmosis-associated HLH among adults is a lethal disease of highly immunocompromised patients, especially patients with HIV/AIDS. Clinical features such as splenomegaly, elevated ferritin, and cytopenias should prompt evaluation for HLH in this population. Further data are needed to define the role of immunosuppression, IVIG, and highly active antiretroviral therapy in treating this condition.

AB - Background. Histoplasmosis-associated hemophagocytic lymphohistiocytosis (HLH) is a relatively rare disorder for which data are limited regarding optimal treatment and clinical outcomes in adults. We describe the clinical features, treatment, and outcomes of patients with histoplasmosis-associated HLH at our institution. Methods. We performed a retrospective chart review of all inpatients at Parkland Hospital diagnosed with HLH associated with Histoplasma capsulatum from 2003 to 2013. Results. Eleven cases of histoplasmosis-associated HLH over this time period were identified. Nine of eleven cases were males (82%). Nine of these patients had human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS), 1 was a renal transplant patient on immunosuppressants, and the other had no documented immunocompromise. The most common HLH criteria were splenomegaly (n = 10), fever (n = 10), and ferritin >500 ng/dL (n = 9). Urine Histoplasma antigen was positive in every patient tested (n = 9 of 9), and most antibodies for Histoplasma were positive if checked (n = 4 of 5). A majority of patients received liposomal amphotericin B (n = 9) with an average treatment duration of 11 days, and 5 patients also received prednisone, intravenous immunoglobulin (IVIG), or both. Overall, 5 patients died within 30 days (45.5%), and 7 patients died 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), 2 died (40%). Conclusions. Histoplasmosis-associated HLH among adults is a lethal disease of highly immunocompromised patients, especially patients with HIV/AIDS. Clinical features such as splenomegaly, elevated ferritin, and cytopenias should prompt evaluation for HLH in this population. Further data are needed to define the role of immunosuppression, IVIG, and highly active antiretroviral therapy in treating this condition.

KW - Disseminated histoplasmosis

KW - Hemophagocytic syndrome

KW - HIV

UR - http://www.scopus.com/inward/record.url?scp=84978328471&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84978328471&partnerID=8YFLogxK

U2 - 10.1093/ofid/ofv055

DO - 10.1093/ofid/ofv055

M3 - Article

C2 - 26380347

AN - SCOPUS:84978328471

VL - 2

JO - Open Forum Infectious Diseases

JF - Open Forum Infectious Diseases

SN - 2328-8957

IS - 2

M1 - ofv055

ER -