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The Heart of the Matter: Secondary Hypogammaglobulinemia and Constrictive Pericarditis

Erica G Schmitt1, Aarti S Dalal2, Alok Kothari3

  • 1Divisions of Rheumatology and Immunology.

Pediatrics
|May 29, 2021
PubMed

Insights

Constrictive pericarditis can stem from mediastinal tumors. This case reveals a rare complication: protein-losing enteropathy, emphasizing diagnostic vigilance for general practitioners.

Area of Science:

  • Cardiology
  • Gastroenterology
  • Oncology

Background:

  • Constrictive pericarditis (CP) is a significant condition often presenting diagnostic challenges.
  • Identifying the etiology of CP is crucial for effective management.
  • Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms that can affect various organs.

Observation:

  • A patient presented with edema, ascites, and weight gain, initially masking cardiac or respiratory distress.
  • Diagnostic workup revealed constrictive pericarditis secondary to a mediastinal IMT.
  • The patient also exhibited hypogammaglobulinemia and lymphocytopenia during the evaluation.

Findings:

  • Elevated stool alpha-1-antitrypsin confirmed protein-losing enteropathy (PLE), a rare complication of CP.
  • The IMT was identified as the underlying cause of CP and subsequent PLE.
  • Clinical presentation lacked typical cardiorespiratory symptoms, complicating initial diagnosis.

Implications:

  • This case underscores the importance of considering rare etiologies and complications in CP diagnosis.
  • General practitioners should be aware of the association between CP, IMTs, and PLE.
  • Prompt diagnosis and management of these interconnected conditions are vital for patient outcomes.

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