title: Progressive histoplasmosis with hemophagocytic lymphohistiocytosis and epithelioid cell granulomatosis: A case report and review of the literature.
abstract: Histoplasmosis in central Europe is a rare fungal disease with diverse clinical presentations. Apart from acute pulmonary histoplasmosis and involvement of the central nervous system, the most serious clinical presentation is progressive disseminated histoplasmosis which is generally associated with severe immunodeficiency and, in particular, advanced human immunodeficiency virus infection. Here, we report on an immunocompetent female residing in a non-endemic area, presenting with progressive disseminated histoplasmosis after a remote travel history to Thailand and Costa Rica. Diagnosis was delayed by several months due to misinterpretation of epithelioid cell granulomatosis of the intestine as Crohn's disease and of similar lung lesions as acute sarcoidosis. Prompted by clinical deterioration with signs and symptoms consistent with hemophagocytic lymphohistiocytosis, a bone marrow aspiration was performed that documented hemophagocytosis and intracellular organisms interpreted as Leishmania sp., but later identified by molecular methods as Histoplasma capsulatum. Treatment with liposomal amphotericin B followed by posaconazole led to prompt clinical improvement and ultimately cure.
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Arik Bernard
authors.identifier: http://orcid.org/0000-0002-1644-3573
authors.initials: AB
authors.lastname: Schulze
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Britta
authors.initials: B
authors.lastname: Heptner
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Torsten
authors.initials: T
authors.lastname: Kessler
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Birgit
authors.initials: B
authors.lastname: Baumgarten
authors.affiliation: Department of Medicine B, University Hospital Muenster, Muenster, Germany.
authors.forename: Viorelia
authors.initials: V
authors.lastname: Stoica
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Mohr
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Rainer
authors.initials: R
authors.lastname: Wiewrodt
authors.affiliation: Gerhard Domagk Institute of Pathology, University Hospital Muenster, Muenster, Germany.
authors.forename: Viktoria Susanne
authors.initials: VS
authors.lastname: Warneke
authors.affiliation: Gerhard Domagk Institute of Pathology, University Hospital Muenster, Muenster, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Hartmann
authors.affiliation: Institute of Medical Microbiology, University Hospital Muenster, Muenster, Germany.
authors.forename: Jörg
authors.initials: J
authors.lastname: Wüllenweber
authors.affiliation: Department of Clinical Radiology, University Hospital Muenster, Muenster, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Schülke
authors.affiliation: Department of Nuclear Medicine, University Hospital Muenster, Muenster, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Schäfers
authors.affiliation: National Reference Center for Cryptococcosis, Scedosporiosis, and Endemic Mycoses, Robert Koch Institute, Berlin, Germany.
authors.forename: Dunja
authors.initials: D
authors.lastname: Wilmes
authors.affiliation: Institute of Medical Microbiology, University Hospital Muenster, Muenster, Germany.
authors.forename: Karsten
authors.initials: K
authors.lastname: Becker
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Lars Henning
authors.initials: LH
authors.lastname: Schmidt
authors.affiliation: Infectious Disease Research Program, Department of Pediatric Hematology/Oncology, Center for Bone Marrow Transplantation, University Hospital Muenster, Muenster, Germany.
authors.forename: Andreas H
authors.initials: AH
authors.lastname: Groll
authors.affiliation: Department of Medicine A, University Hospital Muenster, Muenster, Germany.
authors.forename: Wolfgang E
authors.initials: WE
authors.lastname: Berdel
mesh_terms: D015415:Biomarkers; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D004724:Endoscopy; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006660:Histoplasmosis / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D008875:Middle Aged; D000072078:Positron Emission Tomography Computed Tomography; D014057:Tomography, X-Ray Computed
chemical_list: D015415:Biomarkers
keywords: 
  Histoplasma capsulatum
  ; epithelioid cell granulomatosis; hemophagocytic lymphohistiocytosis