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Internal Medicine

Cryptococcosis

Cryptococcosis, also known as cryptococcal disease, is a potentially fatal fungal disease.

Cryptococcosis, also known as cryptococcal disease, is a potentially fatal fungal disease.


Aetiology

  • Cryptococcus neoformans
  • Cryptococcus gattii

Risk factors:

  • Immunosuppression

Pathophysiology

  • Sites:
    • Commonly:
      • Central nervous system (CNS)
    • Less commonly:
      • Pulmonary
      • Disseminated forms
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General view of the brain anatomy and areas affected by C. neoformans. a. Brain sagittal section and structures associated with the CSF. b. Coronal section of skull, meninges and parenchyma. c. Transversal section of the brain capillary and structural organization of the blood-brain barrier. | COLOMBO, A. N. A. C., & RODRIGUES, M. L. (2015). Fungal colonization of the brain: anatomopathological aspects of neurological cryptococcosis . Anais Da Academia Brasileira de Ciências . scielo .

Clinical features

Clinical presentations:

  1. Wound or cutaneous cryptococcosis
  2. Pulmonary cryptococcosis
  3. Cryptococcal meningitis

Symptoms:

  • Headache
  • Vomiting
  • Altered sensorium
  • Signs of meningism
  • Less commonly: Neurologic deficits

Case study:

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Disseminated Cryptococcosis | A 60-year-old man presented with a 4-week history of progressive skin lesions. For the past 6 years, he had been treated for chronic lymphocytic leukemia (CLL) with successive administration of a combination of rituximab, fludarabine, and cyclophosphamide, a combination of alemtuzumab and dexamethasone, and a combination of rituximab, bendamustine, and methylprednisolone, to which he had had a partial response. His blood CD4+ T-cell count was 20 per cubic millimeter (1%). The physical examination revealed multiple papules of various sizes that were sometimes umbilicated and were located mainly on his face but also on his shoulders, arms, and legs (Panels A and B). The patient also had polyadenopathy related to the CLL. He was otherwise asymptomatic and, in particular, did not present with fever, a decline in general health, or neurologic signs such as headaches, altered mental status, or neck stiffness. The biopsy of one papule revealed numerous encapsulated yeast forms (Panel C, arrowheads; hematoxylin and eosin). Cryptococcus neoformans was cultured from cerebrospinal fluid, blood, urine, and skin, and a test for serum cryptococcal antigen was positive at a titer of 1:400. Amphotericin B plus flucytosine was administered for 3 weeks before he was transitioned to fluconazole. The skin lesions gradually disappeared, with a decrease in the serum cryptococcal antigen titer. | Martin-Blondel, G., & Ysebaert, L. (2014). Disseminated Cryptococcosis. New England Journal of Medicine, 370(18), 1741. https://doi.org/10.1056/NEJMicm1309435

Diagnosis

Demonstrating cryptococci in:

  • CSF by India ink
  • Cryptococcal antigen testing
  • Culture

CSF examination:

  • ↑ pressure
  • ↑ protein
  • Pleocytosis (↑ lymphocytes in CSF)

Management

Antifungal therapy:

  • Amphotericin B + Flucytosine (2 weeks)
    • Followed by,
      • Fluconazole (prolonged periods)

Serial lumbar punctures:

  • Reduction of elevated pressure

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