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Atypical Pneumocystis carinii pneumonia in a child with hyper-IgM syndrome

M L Miller1, I A Algayed, R Yogev

  • 1Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois, USA. millermd@nwu.edu

Pediatric Pathology & Laboratory Medicine : Journal of the Society for Pediatric Pathology, Affiliated with the International Paediatric Pathology Association
|May 5, 1998
PubMed

Insights

Children with hyper-immunoglobulin M syndrome face high Pneumocystis pneumonia risk. An atypical lung response with granulomas, mimicking tuberculosis, highlights the need for biopsy in diagnosing this opportunistic infection.

Area of Science:

  • Pediatric Immunology
  • Infectious Diseases
  • Pulmonology

Background:

  • Children with hyper-immunoglobulin M (hyper-IgM) syndrome exhibit increased susceptibility to opportunistic infections.
  • Pneumocystis carinii pneumonia (PCP) is a significant risk in these immunocompromised pediatric patients, presenting with respiratory symptoms.

Observation:

  • A case is presented of a child with hyper-IgM syndrome experiencing pulmonary disease.
  • Bronchoalveolar washings were negative for Pneumocystis carinii pneumonia.
  • Atypical lung histopathology revealed predominant caseating granulomas, resembling tuberculosis.

Findings:

  • The atypical granulomatous response in the lung, despite negative PCP diagnostics, underscores diagnostic challenges.
  • Histopathological findings mimicked tuberculosis, necessitating careful differential diagnosis.

Implications:

  • A high index of clinical suspicion and prompt histologic confirmation are crucial for early intervention in immunocompromised children with pulmonary disease.
  • Lung biopsy and specific stains (e.g., GMS) may be required to identify Pneumocystis carinii pneumonia in atypical presentations.
  • This case emphasizes the importance of considering diverse pathological responses in immunocompromised hosts.

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