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A 13-month-old child with chronic diarrhea, weight loss, and tachypnea

A P Knutsen1, G M Goodman

  • 1Cardinal Glennon Children's Hospital, St. Louis, MO 63104.

Annals of Allergy
|October 1, 1993
PubMed

Insights

Common variable immune deficiency can mimic Letterer-Siwe syndrome (disseminated Langerhans cell histiocytosis). This rare association highlights the need to screen immunodeficient patients for Langerhans cell histiocytosis.

Area of Science:

  • Immunology
  • Pediatric Pathology

Background:

  • Common variable immune deficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia and impaired T-cell function.
  • Letterer-Siwe syndrome, a form of disseminated Langerhans cell histiocytosis (LCH), is a rare neoplastic proliferation of Langerhans cells.

Observation:

  • A patient with CVID presented with symptoms mimicking LCH, including chronic diarrhea, weight loss, recurrent infections, hepatosplenomegaly, and interstitial pneumonitis.
  • Laboratory findings confirmed immunodeficiency with agammaglobulinemia and reduced T-cell number and function.
  • Diagnosis of LCH was histopathologically confirmed by electron microscopy, identifying Birbeck granules in lung biopsy specimens.

Findings:

  • This case represents a unique association between CVID and LCH.
  • Previously, only combined immunodeficiency syndrome was known to present as disseminated LCH.
  • The findings suggest that CVID can also manifest with features of disseminated LCH.

Implications:

  • Patients newly diagnosed with LCH should be evaluated for underlying immunodeficiency.
  • Clinicians should consider LCH in the differential diagnosis of patients presenting with CVID or other primary immunodeficiencies.
  • Early recognition of these associations is crucial for appropriate patient management and treatment.

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