Progressive histoplasmosis with hemophagocytic lymphohistiocytosis and epithelioid cell granulomatosis: A case report and review of the literature |
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| Authors: | Arik Bernard Schulze Britta Heptner Torsten Kessler Birgit Baumgarten Viorelia Stoica Michael Mohr Rainer Wiewrodt Viktoria Susanne Warneke Wolfgang Hartmann Jörg Wüllenweber Christoph Schülke Michael Schäfers Dunja Wilmes Karsten Becker Lars Henning Schmidt Andreas H. Groll Wolfgang E. Berdel |
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| Affiliation: | 1. Department of Medicine A, University Hospital Muenster, Muenster, Germany;2. Department of Medicine B, University Hospital Muenster, Muenster, Germany;3. Gerhard Domagk Institute of Pathology, University Hospital Muenster, Muenster, Germany;4. Institute of Medical Microbiology, University Hospital Muenster, Muenster, Germany;5. Department of Clinical Radiology, University Hospital Muenster, Muenster, Germany;6. Department of Nuclear Medicine, University Hospital Muenster, Muenster, Germany;7. National Reference Center for Cryptococcosis, Scedosporiosis, and Endemic Mycoses, Robert Koch Institute, Berlin, Germany;8. Infectious Disease Research Program, Department of Pediatric Hematology/Oncology, Center for Bone Marrow Transplantation, University Hospital Muenster, Muenster, Germany |
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| 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. |
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| Keywords: | epithelioid cell granulomatosis hemophagocytic lymphohistiocytosis
Histoplasma capsulatum
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