Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Nov 28, 2015; 21(44): 12713-12721
Published online Nov 28, 2015. doi: 10.3748/wjg.v21.i44.12713
Characteristic clinical features of Aspergillus appendicitis: Case report and literature review
Mihajlo Gjeorgjievski, Mitual B Amin, Mitchell S Cappell
Mihajlo Gjeorgjievski, Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States
Mitual B Amin, Anatomic Pathology, William Beaumont Hospital, Royal Oak, MI 48073, United States
Mitual B Amin, Mitchell S Cappell, Oakland University William Beaumont School of Medicine, Royal Oak, MI 48073, United States
Author contributions: Gjeorgjievski M and Cappell MS contributed equally to the paper; Gjeorgjievski M and Cappell MS are responsible for all the clinical aspects of the paper; Amin MB contributed solely to the pathologic aspects of this paper and is responsible for this section of the paper.
Institutional review board statement: Case report exempted/approved by William Beaumont Hospital IRB on 12/01/2014.
Conflict-of-interest statement: None for all authors. This paper does not discuss any confidential pharmaceutical industry data reviewed by Dr. Cappell as a consultant for the United States Food and Drug Administration Advisory Committee on Gastrointestinal Drugs. Dr. Cappell is a member of the speaker’s bureau for AstraZeneca. This paper does not discuss any drug manufactured or marketed by AstraZeneca.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Mitchell S Cappell, MD, PhD, Chief, Division of Gastroenterology and Hepatology, William Beaumont Hospital, MOB #602, 3535 West Thirteen Mile Road, Royal Oak, MI 48073, United States. mscappell@yahoo.com
Telephone: +1-248-5511227 Fax: +1-248-5517581
Received: June 1, 2015
Peer-review started: June 2, 2015
First decision: June 19, 2015
Revised: July 13, 2015
Accepted: August 30, 2015
Article in press: August 31, 2015
Published online: November 28, 2015
Abstract

This work aims to facilitate diagnosing Aspergillus appendicitis, which can be missed clinically due to its rarity, by proposing a clinical pentad for Aspergillus appendicitis based on literature review and one new case. The currently reported case of pathologically-proven Aspergillus appendicitis was identified by computerized search of pathology database at William Beaumont Hospital, 1999-2014. Prior cases were identified by computerized literature search. Among 10980 pathology reports of pathologically-proven appendicitis, one case of Aspergillus appendicitis was identified (rate = 0.01%). A young boy with profound neutropenia, recent chemotherapy, and acute myelogenous leukemia presented with right lower quadrant pain, pyrexia, and generalized malaise. Abdominal computed tomography scan showed a thickened appendiceal wall and periappendiceal inflammation, suggesting appendicitis. Emergent laparotomy showed an inflamed, thickened appendix, which was resected. The patient did poorly postoperatively with low-grade-fevers while receiving antibacterial therapy, but rapidly improved after initiating amphotericin therapy. Microscopic examination of a silver stain of the appendectomy specimen revealed fungi with characteristic Aspergillus morphology, findings confirmed by immunohistochemistry. Primary Aspergillus appendicitis is exceptionally rare, with only 3 previously reported cases. All three cases presented with (1)-neutropenia, (2)-recent chemotherapy, (3)-acute leukemia, and (4)-suspected appendicitis; (5)-the two prior cases initially treated with antibacterial therapy, fared poorly before instituting anti-Aspergillus therapy. The current patient satisfied all these five criteria. Based on these four cases, a clinical pentad is proposed for Aspergillus appendicitis: clinically-suspected appendicitis, neutropenia, recent chemotherapy, acute leukemia, and poor clinical response if treated solely by antibacterial/anti-candidial therapy. Patients presenting with this proposed pentad may benefit from testing for Aspergillus infection by silver-stains/immunohistochemistry and considering empirical anti-Aspergillus therapy pending a tissue diagnosis.

Keywords: Aspergillosis, Aspergillus appendicitis, Fungal appendicitis, Appendicitis, Neutropenia, Chemotherapy, Acute myelocytic leukemia

Core tip: This work reports the fourth reported case of isolated Aspergillus appendicitis, further characterizes this syndrome, and identifies a clinical pentad associated with this syndrome: clinically-suspected appendicitis, neutropenia, recent chemotherapy, acute leukemia, and poor clinical course if treated solely with antibacterial or anti-Candidial antibiotics. These risk factors are biologically reasonable. Immunosuppression from neutropenia and acute leukemia may promote Aspergillus appendicitis. Local gastrointestinal ulcers from recent chemotherapy provides a nidus for fungal colonization. In patients presenting with this proposed pentad, Aspergillus appendicitis should be considered in the differential diagnosis, special silver stains should be performed to evaluate for this infection, and empiric anti-Aspergillus therapy may be considered pending tissue diagnosis.