Date: 26 November 2013
History : A 20 yr old female with cystic fibrosis complicated by CF-related Diabetes mellitus (diagnosed at age 12 years) & ABPA (diagnosed at
15 years ). She was fairly stable until last 9 months, when she started to experience frequent pulmonary exacerbations, which have prompted intensive therapies.
Her serum IgE at the time of diagnosis was 5060 IU/L, skin prick test for aspergillus was positive, and serum was positive for precipitating antibodies to Aspergillus. She was treated with oral prednisone (1 mg/kg/day) for first two weeks followed by prednisone at 0.5 mg/kg every other day for at least 6 months with some clinical and serologic improvement. Over the following 5 years, she presented with a pattern of repeated episodic exacerbations with wheezing and crackles, increases in IgE and need to increase prednisone dosage. In an attempt to control her frequent ABPA relapses, itraconazole was added at 200mg twice a day, with some clinical & serologic improvement.
2-3 weeks prior to the scans and X rays, she developed severe protracted coughing spells associated with minor hemoptysis, low grade intermittent fever, and weight loss. Her FEV1 declined in a 3 months period from 56% to 33%.
A chest-x ray (Fig 3) did not reveal any new changes when compared to the one obtained almost a year before. A CT scan of the chest Figs 1 and 2- lateral view), however shows an ovoid soft tissue density within an ectatic bronchi in the anterior basal segment of the LLL, felt to be an aspergilloma.
She was started on oral voriconazole (3 months after the above scans and X-rays) which is better absorbed in CF patients than itraconazole, at 200mg twice a day. Her prednisone was gradually reduced to 5 mgs/day and her FEV1 increased to 46% of predicted (03/31/10), her IgE level was 1167 kU/L (previously in January 2009, her IgE level was 3053 kU/L)and her weight has plateaued.
Since she was not fit enough for surgery at that time, removing the aspergilloma by flexible bronchoscopy was unsuccessful. The aspergilloma can be seen at bronchoscopy in Fig 4.
Go to follow up 12 months later
Copyright:
Kindly donated by Dr N Turcios, Director of pediatric pulmonology/cystic fibrosis in Somerville, NJ.
Notes: n/a
Images library
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BAL specimen showing hyaline, septate hyphae consistent with Aspergillus, stained with Blankophor
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Mucous plug examined by light microscopy with KOH, showing a network of hyaline branching hyphae typical of Aspergillus, from a patient with ABPA.
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Corneal scraping stained with lactophenol cotton blue showing beaded septate hyphae not typical of either Fusarium spp or Aspergillus spp, being more consistent with a dematiceous (ie brown coloured) fungus
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Corneal scrape with lactophenol cotton blue shows separate hyphae with Fusarium spp or Aspergillus spp.
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A filamentous fungus in the CSF of a patient with meningitis that grew Candida albicans in culture subsequently.
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Transmission electron micrograph of a C. neoformans cell seen in CSF in an AIDS patients with remarkably little capsule present. These cells may be mistaken for lymphocytes.
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India ink preparation of CSF showing multiple yeasts with large capsules, and narrow buds to smaller daughter cells, typical of C. neoformans
